Pediatrics: by DR - Mohammad Z. Abu Sheikha@

PEDIATRICS
By Dr.Mohammad Z. abu sheikha@

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The Newborn And Resuscitation
**Apgar Scoring System
+Heart Rate
.0 points – absent
.1 point - <100/min
.2 points - >100/min
+Respiration
.1 point – irregular, shallow, gasps, weak cry
.2 points – crying (vigorous)
+Color
.0 points – pale, cyanotic
.1 point – cyanotic extremities
.2 points – pink
+Tone (Muscle)
.1 point – weak, slightly flexed extremities
.2 points – active
+Reflex Irritability
.1 point – facial grimace
.2 points – active cry – withdrawal and avoidance
+NOTES
.apgar scores are routinely assessed at 1 and 5mins, and every 5 mins thereafter as long as resuscitation is continuing
.the 1-min score gives an idea of what was going on during labor and delivery
.the 5-mins score gives an idea of response to therapy (resuscitation)
.infants with score 0-3 at 5 mins and longer compared to infants with score 7-10 have a worse neurologic outcome (infant with
apgar score (0-3) have worse neurological outcome)
+The perinatal mortality rate (stillbirth) (refers to fetal deaths occurring from the 20 th week of gestation until the 7th day after
birth)
+The neonatal mortality rate (infants who die between birth and 28 days of life)
+Postneonatal period (which begins after 28 days of life and extends to the end of the first year of life)
**Newborn Care
1.Vitamin K - IM
.to reduce incidence of hemorrhagic of newborn. If your baby has a deficiency of Vit K, he may spontaneously bruise or bleed
2.Prophylactic eye erythromycin
3.Umbilical cord care
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4.Hearing test
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5.Newborn screening test

.phenylketonuria (PKU) – is an amino acid that is needed for normal growth and development
+All Are Recognized Findings In Phenylketonuria (PKU) :
.seizures
.macrocephaly
.eczema
.blonde hair and blue sclera
+Galactosemia (is a rare genetic metabolic disorder that affects an individual's ability to metabolize the sugar galactose
properly)
**Birth Marks
1.Salmon Patch
.flat vascular lesion
.on eyelid / glabella
.disappears with time, except if developed in the nuchal area
2.Capillary (strawberry) Hemangioma
.starts in first few month and quickly grow in the 1 st year of life
.bright red / regress spontaneously (no treatment)
3.Nervus Sebaceous (of jadassohn)
.yellow/orange plagnes
.hairless / in scalp
.increased risk of malignancy (removed at adolescent)
4.Cafe-au-lait Spots
.bright-brown
.anywhere / flat
.well demarcated
**Birth Injuries
1.Skull Fractures
.linear M/C (no symptoms And no treatment nedeed)
.depressed (elevate to prevent cortical injury)
2.Brachial Plasy
.in large infant + traction of head
.is a loss of movement or weakness of the arm. It occurs when the collection of nerves around the shoulder (called the brachial
plexus) are damaged during birth
+Erb Palsy (C5 - C6) M.C nerve injury in brachial palsy (adducted / pronated arm – internally rotated arm) (if C 4 affected –
diaphragmatic paralysis)
+Klumpke (C7 - C8) (hand paralysis / Horner syndrome)
3.Clavicular Fracture (Clavicle Fracture)
.is a bone fracture in the clavicle, or collarbone. Is found in 2-3% if vaginal deliveries (Rt. clavicle > Lt. clavicle - to fracture)
.M.C birth injyry (M.C bone fracture during delivery)
.predisposing factors (large size, shoulder dystocia and traumatic delivery)
.on examination (swelling, fullness over the fracture site, crepitus and decreased arm movement)
.of affected neonates 80% have no symptoms
.the injury is often diagnosed when a callus is detected ar 3-6wks of age
.radiographs are not indicated, and no specific treatment is necessary
.the parents should be advised to avoid tension on the affected arm (asymmetric moro reflex)
4.Facial Nerve Palsy
.post-difficult instrumental delivey (usually after forceps)
.peripheral injury (flacid paralysis)
.improve withen weeks
5.Caput Succedaneum
.is a diffuse, edematous and often dark swelling of the soft tissue of the scalp that across the midline and/or suture lines
-involving a serosanguinous, subcutaneous, extraperiosteal fluid collection with poorly defined margins caused by the pressure
of the presenting part of the scalp against the dilating cervix (tourniquet effect of the cervix) during delivery. It involves
bleeding below the scalp and above the periosteum (is swelling of the scalp in a newborn. It is most often brought on by
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pressure from the uterus or vaginal wall during a head-first (vertex) delivery)
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.is commonly found in infants who are delivered vaginally in the customary occiput-anterior position
.is commonly seen after prolonged labor in both full-term and premature infants
.resolves in fews days
6.Cephalohematoma
.is a subperiosteal hemorrhage (usually involving the parietal bone) that does not cross suture lines (limited to bone)
.the scalp hematoma is characteristically (fluctuant without discoloration of overlying skin and may not become apparent until
hours to days after delivery)
.predisposing factors (large head size, prolonged labor, vacuum extraction and forceps delivery)
.spontaneous resolution occurs over several weeks
.can cause anemia or jaundice
7.Subcutaneous Fat Necrosis
.after difficult labor + instrumental deliveries
.resolves spontaneously
**Newborn Screening
-Every newborn before discharge or day 4 of life
-Total disease screened are determined by individual states :
.phenylketonuria
.galactosemia
.hypothyroidism
.tyrosinemia (is an error of metabolism, in which the body cannot effectively break down the amino acid tyrosine)
.Hb SS
.Hb C
.cystic fibrosis
.21-hydroxylase deficiency
.G6PD
+Comparison Of Two Newborn Screening Disease
+Phenylketonuria (PKU) +Classic Galactosemia

-Defect .phenylalanine hydroxylase; .gal-1-P uridylyltransferase deficiency;
accumulation of PHE in body accumulation of gal-1-P with injury
fluids and CNS to kidney, liver and brain
-Presentation .mental ret., vomiting, growth .jaundice (direct), hepatomegaly,

retardation, purposeless movem., vomiting, hypoglycemia, cataracts,
athetosis, seizures seizures, poor feeding, poor weight gain,
mental retardation
-Associations .fair hair, fair skin, blue eyes, tooth .predisposition to E.coli sepsis; develop.
abnormalities, microcephaly mental delay, speech disorders, learning disability
-Treatment .low PHE diet for life .no lactose, but no neuro. Developmental problems
*Phenylketonuria PKU
-Defect on hydroxylation of phenylalanine to tyrosine. is an inborn error of metabolism involving impaired metabolism of the
amino acid phenylalanine. Phenylketonuria is caused by absent or virtually absent phenylalanine hydroxylase (PAH) enzyme
activity
-Signs & Symptoms :
.mental retardation
.projectile vomiting
.eczema
.musty odor
.fair-haired, fair-skinned babies
*Galactosemia
-Is a rare genetic metabolic disorder that affects an individual's ability to metabolize the sugar galactose properly. Galactosemia
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follows an autosomal recessive mode of inheritance that confers a deficiency in an enzyme responsible for adequate galactose
degradation
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+Galactose-1-phosphate uridyl transferase deficiency (elevated galactose 1-p. – renal/liver/brain injury)

-Signs & Symptoms :
.jaundice, hypoglycemia
.cataract
.seizure
.hepatosplenomegaly
.poor weight gain
.presented as E.coli (sepsis)
-Treatment :
.low galactose diet
**Congenital Anomalies
1.Eye
.coloboma (defect in lid)
.aniridia (absent iris)
2.Neck
+Branchial cleft cyst (incomplete closure of branchial clefts-cysts)
.usually unilateral
.can be infected
+Thyroglossal duct cyst
.midline
.moves with swelling and tongue protusion
+Congenital torticollis
.mostly from injury to sternocleidomastoid
3.Chest
+Breast hypertrophy
.due to increased circulating hormones
+Polythelia (nipple)
.along mamary line
.may be associated with cardiac – renal abnormalities
+Poland syndrome
.amastia, pectoralis aphasia, rib deformities, webbed finger, radial nerve palsy
4.Abdomen
.mass (M.C is renal – hydronephrosis, cyst)
.umbilical hernia
.omphalocele
.gastroschisis
**Prematurity
+Low Birth Weight LBW
.<2.500g (VLBW <1.500g)
.causes (premature birth, IUGR) (maternal factors – previous LBW birth, low socioeconomic status, lack of prenatal care,
maternal age <16yrs or >35yrs, short time interval between pregnancies, unmarried status, low pregnancy weight, poor weight
gain during pregnancy, maternal substance use)
.medical causes of preterm birth (fetal distress, multiple gestations, erythroblastosis hydrops fetalis, congenital anomalies,
placenta previa, abruptio placenta, bicornuate uterus, incompetent cervix, preeclampsia, chronic medical illness, infection, drug
abuse, PROM, polyhydramnios, trauma, diethylstilbestrol exposure)
+Postmaturity
.infants born after 42 weeks gestation from last menstrual period
.characteristics (increased birth weight, absence of lanugo, decreased, absence vernix, desquamating-cracked-pale-loose-
peeling and wrinkled skin, abundant hair, long nails, meconium aspiration and depression at birth, PPHN, hypoglycemia,
hypocalcemia, polycythemia)
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**Gestational Age And Size At Birth

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*Large For Gestational Age (LGA) – fetal macrosomia

.birth weight >4500grams at term
.predisposing factor (obesity, diabetes)
.higher incidence of birth injuries and congenital anomalies
*Small For Gestational Age (SGA)

-IUGR, birth weight <3rd percentile for GA
+Intrauterine Growth Restriction IUGR
-Types :
1.Symmetric
.reason (early, in utero-insult that affects growth of most organs)
.etiology (genetic syndromes, chromosomal abnormalities, congenital infections, teratogens, toxins)
.complications (etiology dependent; delivery of oxygen and nutrients to vital organs usually normal)
2.Asymmetric
.reason (relatively late onset after fetal organ development; abnormal delivery of nutritional substances and oxygen to the
fetus)
.etiology (uteroplacental insufficiency secondary to maternal disease ‘malnutrition, cardiac, renal, anemia’) and/or (placental
dysfunction ‘hypertension disease, abruption’)
.Complications (neurologic ‘asphyxia’ if significant decreased delivery of oxygen to brain)
+Causes Of IUGR
.fetal (multiple gestation, congenital viral infections, chromosomal abnormalities, congenital malformation syndromes - CNS)
.placental (chorionic villitis, chronic abription placenta, twin-twin transfusion, placental tumor, placental insufficiency secondary
to maternal vascular disease)
.maternal (severe peripheral vascular disease, chronic hypertension, diabetic vasculopathy, preeclampsia,sickle cell anemia,
cardiac disease, renal disease, alcohol or drug abuse, reduced nutritional intake, smoking, uterine anomalies, uterine constraint
– noted in mothers of small stature and reduced weight gain during pregnancy)
*Infants Of Diabetic Mother IODM

-Maternal hyperglycemia (type 1-2 DM) – (fetal hyperinsulinemia)
+Insulin is the major fetal growth hormone - increase (insulin) in size of all organs except the brain
+Major metabolic effect is at birth with sudden placental separation - (hypoglycemia)
-Complications :
.decreased surfactant production (increased risk of ARDS)
.polycythemia, hyperbilirubinemia
.hypertrophic cardiomyopathy
.increased risk for congenital anomalies (cardiac defects - asymmetric septal hypertrophy M.C / caudal regression syndrome or
‘sacral agenesis’)
.increased risk of obesity + DM in children
+Metabolic Findings
.hypoglycemia
.hypocalcemia
.hypomagnesemia
.hyperinsulinemia
+Common Findings
.birth trauma (macrosomia)
.tachypnea (transient tachypnea,respiratory distress syndrome,cardiac failure,hypoglycemia)
.cardiomegaly (LEAD TO) asymmetric septal hypertrophy
.polycythemia and hyperviscosity (LEAD TO) hyperbilirubinemia (LEAD TO) jaundice
.renal vein thrombosis (flank mass, hematuria,and thrombocytopenia) from polycythemia
.congenital anomalies (cardiac-VSD, ASD, transposition) (small left colon syndrome)
.infants of diabetic mothers are more predisposed to diabetes and LGA infants are at increased risk of childhood obesity
**Respiratory Disorders
*Respiratory Distress Syndrome RDS (hyaline membrane disease)
-Is a syndrome in premature infants caused by developmental insufficiency of surfactant production (deficiency of mature
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surfactant) and structural immaturity in the lungs. It can also result from a genetic problem with the production of surfactant
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associated proteins (complex phospholipid and protein mixture that lines the alveoli, is produced by II pneumocytes)
+Is the M.C.C of respiratory failure in newborn infants
+RDS is caused by a deficiency of surfactant (The major function of surfactant is to decrease alveolar surface tension and
increase lung compliance. Surfactant prevents alveolar collapse at the end of expiration and allows for opening of the alveoli at
low intrathoracic pressures)
+Inability to maintain (deficiency) alveolar volume at end expiration (LEAD TO) decreased FRC (functional residual capacity) and
atelectasis
+The incidence of RDS increases with decreasing GA
+Primary initial pulmonary hallmark is (hypoxemia), then (hypercarbia and respiratory acidosis ensue)
-Signs & Symptoms :
.tachypnea (1st sign)
.nasal flaring
.cyanosis
.grunting
.chest wall retractions
.apnea
-Comlications :
.pulmonary interstitial emphysema
.pneumothorax
.pneumomediastinum
.pneumopericardium
-Diagnosis :
.best initial diagnostic test - Chest radiograph CXR – findings (Ground-glass or reticulonodular)
.most accurate diagnostic test - L/S ratio (lecithin-to-sphingomyelin ratio is <2) and phosphatidylglycerol is absent in the
amniotic fluid
-Treatment :
.best initial treatment-Oxygen
.most effective treatment-intubation and exogenous surfactant administration
*Transient Tachypnea Of The Newborn (TTN) ("wet lungs" or type II respiratory distress syndrome)
-Is a respiratory problem that can be seen in the newborn shortly after delivery. Amongst causes of respiratory distress in term
neonates, it is the most common
+Newborns' breathing during the first hours of life is more rapid (tachypnea at birth) and labored than normal because of a lung
condition called (TTN)
+Slow absorption of fetal lung fluid (LEAD TO) decreased pulmonary compliance
+Newborns At Higher Risk For TTN include those who are :
.delivered by cesarean section (C-section)
.born to mothers with diabetes
.born to mothers with asthma
.small for gestational age (small at birth)
+CXR (BEST TEST) - Findings (air trapping, fluid in fissures, perihilar streaking)
*Meconium Aspiration Syndrome (MAS)

-Is a medical condition affecting newborn infants. It occurs when meconium is present in their lungs during or before delivery.
Meconium is the first stool of an infant, composed of materials ingested during the time the infant spends in the uterus
-Signs & Symptoms :
.tachypnea
.hypoxia
.hypercapnia
.the infant's skin, umbilical cord, or nailbeds may be stained green if the meconium was passed a considerable amount of time
before birth
.after birth, rapid or labored breathing, cyanosis, slow heartbeat, a barrel-shaped chest or low Apgar score
.abnormal lung sounds (diffuse 'wet' crackles and rhonchi)
-Complications :
.aspiration pneumonia
.pneumothorax
.persistent pulmonary hypertension (pulmonary artery HTN)
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.air way obstruction

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-Risk factors :
.fetal distress
.post-term baby
.IUGR
.placental insufficiency
.oligohydramnios
+CXR (BEST TEST) – Findings (patchy infiltrates, increased AP diameter, flattening of diaphragm)
+Prevention (endotracheal intubation and airway suction)
-Treatment :
.positive pressure ventilation and other complex NICU therapies
*Diaphragmatic Hernia
-Failure of the diaphragm to close (LEAD TO) abdominal contents enter into chest, causing pulmonary hypoplasia
-A congenital malformation (birth defect) of the diaphragm / More at Lt. side
-Types :
.Bochdalek hernia (M.C type) – Postero-lateral diaphragmatic hernia 90%
.Morgagni hernia – Anterior defect of the diaphragm 10%
.diaphragm eventration
.central tendon defects
-Presentation :
.bowel sounds may be heard in chest
.malformation of the diaphragm allows the abdominal organs to push into the chest cavity (abdomen is scaphoid), and lead to
respiratory distress at birth
.infants born with diaphragmatic hernia experience respiratory failure due to both pulmonary hypertension and pulmonary
hypoplasia.
.pulmonary hypoplasia or decreased lung volume is directly related to the abdominal organs presence in the chest cavity which
causes the lungs to be severely undersized, especially on the side of the hernia
.work has been done to correlate survival rates to ultrasound measurements of the lung volume as compared to the baby's
head circumference. This figure known as the lung to head ratio (LHR)
-Diagnosis :
.prenatal ultrasound
.postnatal x-ray (best test)
-Treatment :
.orogastric tube placement
.intubation
.surgery
**Gastrointestinal And Hepatobiliary Disorders

*Necrotizing Enterocolitis (NEC)
-Is a medical condition primarily seen in premature infants, where portions of the bowel undergo necrosis (tissue death). It
occurs postnatally (i.e. it is not seen in stillborn infants) and is the second most common cause of morbidity in premature
infants (greatest risk factor is prematurity and rare in term infants)
-Signs & Symptoms :
.pneumatosis intestinalis (air in bowel wall) ( is a radiological sign which is highly suggestive for necrotizing enterocolitis)
.feeding intolerance
.increased gastric residuals
.abdominal distension
.bloody stools
.abdominal discoloration with intestinal perforation
.peritonitis
.systemic hypotension
+Symptoms usually related to introduction of feeds – Start after feeding (MILK) - (bloody stools, apnea, lethargy and abdominal
distention once perforation has occurred)
-Treatment :
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.cessation of feeds
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.gut decompression
.systemic antibiotics
.supportive care
.surgical (resection of necrotic bowel)
+Notes :
.M.C emergency in newborn
.90% in preterm infants
.in 1st 2wks of life, related to feeding
.bloody stool, abdominal distension, lethargy
.pneumatosis intestinalis is pathognomonic
*Meconium Ileus
-Meconium is the first stool (bowel movement) that a newborn has. This stool is very thick and sticky. Meconium ileus is a
bowel obstruction that occurs when the meconium in your child's intestine is even thicker and stickier than normal meconium,
creating a blockage in a part of the small intestine called the ileum / Intestinal obstruction from solid meconium concretions
+Most infants with meconium ileus have a cystic fibrosis / X-ray
-Signs & Symptoms :
.bilious vomiting (green)
.abdominal distension (a swollen belly)
.failure to pass meconium (no passage of meconium)
.Neuhauser`sign (refers to a soap bubble appearance seen in the distal ileum in cases of meconium ileus, related to the air
mixed with meconium. It may be seen with barium enema if contrast passes beyond the ileocaecal valve or with small-bowel)
.peritoneal calcifications
+Impaction of meconium leads to intestinal obstruction. Mostly related to cystic fibrosis
+Symptoms (mostly in lower ileum, abdominal distension, vomiting)
-Diagnosis :
.gastrografin enema (X-ray of the large intestine that includes the colon and rectum)
*Imperforate Anus
-Birth defects in which the rectum is malformed. ARMs are a spectrum of different congenital anomalies in males and females,
that varies from fairly minor lesions to complex anomalies
+The cause of ARMs is unknown
-Associated anomalies :
.VACTERL
V – Vertebral anomalies
A – Anal atresia
C – Cardiovascular anomalies
T – Tracheoesophageal fistula
E – Esophageal atresia
R – Renal (kidney) and/or radial anomalies
L – Limb defects
.trisomies 18 and 21
.the cat-eye syndrome (partial trisomy or tetrasomy of a maternally derived number 22 chromosome)
.Baller-Gerold syndrome
.Currarino syndrome
.caudal regression syndrome
.FG syndrome
.Johanson-Blizzard syndrome
.McKusick-Kaufman syndrome
.Pallister-Hall syndrome
.short rib-polydactyly syndrome type 1
.Townes-Brocks syndrome
.urorectal septum malformation sequence
.OEIS complex (omphalocele, exstrophy of the cloaca, imperforate anus, spinal defects)
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*Hirschsprung Disease
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-Also called congenital megacolon or congenital aganglionic megacolon, is a form of megacolon that occurs when part or all of
the large intestine or antecedent parts of the gastrointestinal tract have no ganglion cells and therefore cannot function
+M > F and 10% of cases are familial / M.C site is Rectum
+Hirschsprung's disease is diagnosed shortly after birth, although it may develop well into adulthood, because of the presence
of megacolon, or because the baby fails to pass the first stool (meconium) within 48 hours of delivery / Some cases are
diagnosed later, into childhood, but usually before age 10
-Signs & Symptoms :
.meconium ileus (1st symptom)
.green or brown vomit
.explosive stools after a doctor inserts a finger into the rectum
.swelling of the abdomen (meconium ileus)
.lots of gas and bloody diarrhea
.fecal retention, constipation or abdominal distention
+M.C.C of death in children with HD is (enterocolitis)
-Diagnosis :
.rectal biopsy (Best)
-Treatment :
.surgical removal (resection) of the abnormal section of the colon, followed by reanastomosis
*Duodenal Atresia
-Is the congenital absence or complete closure of a portion of the lumen of the duodenum / Complete obstruction or stenosis
of duodenum caused by an ischemic insult during development of failure of recanalization
+It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies
+During pregnancy, duodenal atresia is associated with increased amniotic fluid in the uterus, which is called polyhydramnios.
This increase in amniotic fluid is caused by the inability of the fetus to swallow the amniotic fluid and absorb it in their digestive
tract
-Anatomic Location :
.85% are distal to the ampulla of vater
.15% are proximal to the ampulla of vater (these present with nonbilious vomiting)
-Signs & Symptoms :
.bilious vomiting (green vomit) – at 1st day (if distal to the ampulla) / nonbilious vomiting (if fistal to the ampulla)
.epigastric distention
.plain abdominal film revealing (double bubble)
-Complications :
.serious congenital anomalies
+Late complications may occur in about 12% of patients with duodenal atresia, and the mortality rate for these complications is
6%
+20-40% of all infants with duodenal atresia have Down syndrome (trisomy 21) / 8% all infants with Down syndrome have
duodenal atresia
-Diagnosis :
.X-ray of the abdomen (double bubble sign)
-Treatment :
.suctioning out any fluid that is trapped in the stomach, providing fluids intravenously
.surgical repair of the intestinal closure
*Tracheoesophageal Fistula
-Is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but
when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy
+TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT)
-Signs & Symptoms :
.salivation
.choking
.coughing
.vomiting
.cyanosis coincident with the onset of feeding
+Esophageal atresia and the subsequent inability to swallow typically cause polyhydramnios in utero. Rarely it may present in
an adult
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-Associations :
.dysphagia and thoracic problems
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+Children with TEF can also be born with other abnormalities, most commonly those described in VACTERL association - a group
of anomalies which often occur together, including heart, kidney and limb deformities. 6% of babies with TEF also have a
laryngeal cleft
**Jaundice
-First appears in the face and progress down as the level increased
+Physiologic Jaundice VS Pathologic Jaundice
nd rd
.Appears on 2 to 3 DOL (term) .May appear in first 24hrs of life
th
.Disappears by 5 DOL (term)-7th .Variable
(maximum 14days)
nd rd
.Peaks at 2 to 3 DOL .Variable
.Peak bilirubin <13mg/dL (term) .Unlimited
.Rate of bilirubin rise <5mg/dL/d .Usually >5mg/dL/d
-Causes Of Pathological Jaundice :
+Direct Hyperbilirubinemia (Conjugated) – is always pathological
.sepsis
.TORCH infections
.hypothyroidism
.galactosemia
.cystic fibrosis
.biliary atresia (usually start at 7th day)
.tyrosinemia
.alpha-1 Antitrypsin Deficiency (alpha-1ATD)
.choledochal cyst (bile duct cyst)
.total parenteral nutrition (TPN)
+Indirect Hyperbilirubinemia (Unconjugated)
.ABO incompatibility - rh incompatibility (Rh disease)
.polycythemia
.twin-twin transfusion
.IUGR
.infant with diabetic mother (IODM)
.maternal fetal transfusion
.spherocytosis, elliptocytosis (ovalocytosis) - high reticulocyte count
.enclosed hematoma, dehydration, decreased calories – normal reticulocyte count
.delayed cord clamping
.G6PD deficiency
.hemorrhage
-Complications :
.increased indirect bilirubin (kernicterus - is a bilirubin-induced brain dysfunction. Bilirubin is a highly neurotoxic substance that
may become elevated in the serum, a condition known as hyperbilirubinemia) (symptoms; hypotonia, seizure, hear loosing)
.increased direct bilirubin (not neurotoxic but, it indicate serious underlying disease)
-Treatment :
.phototherapy for indirect (NOT for direct)
.double volume exchange transfusion (if bilirubin continues to rise despite intensive phototherapy and/or kernicterus is a
concern)
-Complications Of Phototherapy :
.Bronze-baby syndrome (occurs with direct hyperbilirubinemia; brown discoloration of the skin)
.loose stools
.erythematous macular rash
.overheating, leading to dehydration
+Jaundice appearing in the first day of life suggests (Hemolytic disease of the newborn)
+Increases the Risk Of Neonatal Jaundice :
.Prematurity
.Trisomy 21
.Congenital hypothyroidism
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.Cephalohematoma
st
+Breast milk jaundice (at 1 week of age / treated by stop breast feeding)
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+Breast feeding jaundice (treated by feeding)
**Crigler–Najjar syndrome (CNS) - Indirect

-Is a rare inherited disorder affecting the metabolism of bilirubin, a chemical formed from the breakdown of the heme in red
blood cells. The disorder results in a form of nonhemolytic jaundice, which results in high levels of unconjugated bilirubin and
often leads to brain damage in infants. The disorder is inherited in an autosomal recessive manner
-Types :
.type 1 (more sever Kernicterus is common)
.type 2 (mild kernicterus is uncommon)
**Gilbert Syndrome GS – Indirect
-Is a common genetic liver disorder found in 3-12% of the population. It produces elevated levels of unconjugated bilirubin in
the bloodstream (hyperbilirubinemia), may appear under conditions of exertion or stress. The cause of this hyperbilirubinemia
is the reduced activity of the enzyme glucuronyltransferase
**INFECTIONS
*Neonatal Sepsis
st
-Early (1 week) / Late (8-28 days)
-Most Common Organisms :
.group B streptococcus GBS (M.C)
.E.coli
.Listeria monocytogenes
.HSV, enterovirus
-Signs & Symptoms :
.tachypnea
.cyanosis
.crying
.poor feeding
.seizure
-Risk Factors :
.prematurity
.chorioamnionitis, UTI
.intrapartum fever
.prolonged rupture of membranes PROM
-Diagnosis :
.sepsis workup (CBC, differential and platelets, blood culture, urine analysis and culture, chest radiographic)
.lumbar puncture (not routinely performed unless there is a likelihood of meningitis, irritability, lethargy, hypothermia)
-Treatment :
.if no evidence of meningitis (ampicillin and aminoglycoside until 48-72hr culture are negative)
.if meningitis or diagnosis is possible (ampicillin and third-generation cephalosporin, NOT ceftriaxone)
+Beta Hemolytic streptococci group A is responsible for :
.Acute glomerulonephritis
.Rheumatic fever
.Scarlet fever
.Impetigo
**Transplacental Intrauterine Infections TORCH

-Most TORCH infections are acquired in first or second trimester. Most infants have TORCH
+Toxoplasmosis, Other (syphilis, varicella, HIV and parvovirus B19), Rubella, Cytomegalovirus, Herpes
*Toxoplasmosis
-Causative Agent (Toxoplasma gondii)
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-Clinical Manifestations :
.rash (maculopapular)
.lymphadenopathy
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.hepatosplenomegaly
.jaundice
.thrombocytopenia
.secondary to in utero meningoencephalitis
.hydrocephalus with generalized calcifications
.microcephaly
.chorioretinitis
.seizures
.psychomotor calcification
-Diagnosis :
.congenital infection cinfirmed by (positive IgG and IgM antibody titres) in the infant
.neurologic examinations with lumbar puncture and head CT
-Treatment :
.maternal treatment during pregnancy reduces the likelihood of transmission significantly (spiramycin) in 1 st trimester
.infants are treated with pyrimethamine + sulfadiazine and leucovorin (corticosteroids for ocular and CNS disease)
+Maternal infection worldwide primarily from (ingestion of undercooked or raw meat containing tissue cyst. Ingestion of water
or food with oocytes that have been excreted by infected cats – fecal-oral)
+Only primary infection of the mother, which is usually asymptomatic, results in congeninatl infection
+Infants with congenital infection are asymptomatic in 70% to 90% of cases
*Congenital Rubella
-Causative Agent (Rubella virus)
.ophthalmologic (cataracts, pigmentary retinopathy, microphthalmia, congenital glaucoma)
.auditory (congenital hearing loss)
.cardiac (patent ductus arteriosus PDA, peripheral pulmonary artery stenosis)
.blueberry muffin spots (extramedullary hematopoiesis)
.thrombocytopenia
.IUGR (M.C)
.hepatosplenomegaly
.growth retardation
.interstitial pneumonitis
.radiolucent bone disease
.microcephaly
.mental and motor retardation
-Diagnosis :
.presence of Rubella specific IgM or rising IgG titre
.cardiac, dermatology examination
+Congenital Rubella syndrome results from transplacental transmission or Rubella
+Vaccination should not be given during pregnancy
*Cytomegalovirus (CMV)
-Causative Agent (CMV)
.hepatosplenomegaly
.jaundice
.retinitis
.IUGR
.periventricular calcifications
.microcephaly
.thrombocytopenia
.sensorineural hearing loss (deafness)
.neuromuscular abnormalities
.mental retardation
-Diagnosis :
13
.CMV culture positive from urine, stool, respiratory tract secretions

.CSF obtained within 3wks of birth
.positive CMV specific IgM
Page
.neurologic evaluation with head CT and brain MRI is recommended

-Treatment :
.gancyclovir
+Neonatal CMV infection is common, and result from transplacental transmission, inoculation during passage through the
vaginal canal, or via the ingestion of infected breast milk
+Most cases of congenital CMV are asymptomatic
*Herpes Simplex Virus HSV

-Causative Agent (HSV 1 / HSV2)
.disseminated disease involving multiple organs, most prominently liver and lungs
+HSV 1 (localized CNS disease with or without seizures)
+HSV 2 (localized disease to the skin, eye and mouth)
-3 Patterns (keratoconjunctivitis) :
.local (skin, eye, mouth - 5-14 days)
.CNS (lethargy, seizure - 3-4 weeks)
.disseminated (highest mortality, pnemonic, shock, hepatitis - 5-7 days)
-Diagnosis :
.positive viral cultures from CSF, skin, vesicles, conjunctivae, urine, blood, mouth, nasopharynx, and/or rectum
.positive HSV PCR of CSF
-Treatment :
.IV acyclovir ASAP
+Most neonatal HSV infection is caused by HSV 2 (because it accounts for the majority of genital herpes)
+The child is infected as he or she moves through the vaginal canal
+The risk of HSV infection at delivery for an infant born vaginally to a mother with primary genital infection is 50%
+The risk of HSV infection at delivery for an infant born vaginnaly to a mother shedding HSV as a result of reactivation infection
is <5%
*Congenital Syphilis
-Causative Agent (Treponema Pallidum)
.hepatosplenomegaly
.rhinitis (snuffles)
.lymphadenopathy
.mucocutaneous lesions
.osteochondritis and pseudoparalysis
.eczematoid skin rash
.hemolytic anemia
.thrombocytopenia
.maculopapular rash - including (palms of soles, desquamates)
.failure to thrive
.skeletal manifestations
.hutchinson teeth
.clutton joints, saber shins, saddle nose, osteochondritis
.rhagades (thickening and fissure of corners of mouth)
-Diagnosis :
.nontreponemal test such as RPR or VDRL, supported by treponemal test such as IgM FTA-ABS (immunoglobulin fluorescent
treponemal antibody absorption)
-Treatment :
.penicillin
+Syphilis results from transplacental transmission of Treponema Pallidum in utero
+Syphilis in the untreated pregnant woman may be transmitted to the fetus at any time, but fetal transfer is more likely during
the first year of maternal infection
*Varicella
-Causative Agent (Varicella-zoster virus VZV)
14
+Congenital Varicella Syndrome
Page
.<20wks gestation – varicella embryopathy (1-2%)

.limp hypoplasia
.cutaneous scarring
.eye abnormalities
.CNS damage
+Neonatal Chickenpox
.>20wks gestation
.generalized, pruritic, vesicular rash
.bacterial superinfection of skin lesions
.pneumonia
.CNS involvement (cerebellar ataxia and encephalitis)
.thrombocytopenia
-Diagnosis :
.tissue culture, CSF, biopsy tissue
.PCR
-Treatment :
.Infants born to mothers with onset of varicella 5 or more days before delivery require no specific treatment other than
isolation, if kept in the hospital
.Infants whose mothers have onset of varicella within 5 days before delivery, or within 2 days after delivery, should receive
varicella-zoster immune globulin (VZIG), preferably at birth or within 96hrs
.Infants with acute vericella in the first weel of life should receive acyclovir for 10 days
+Varicella virus infection in the neonate results from transplacental transmission of varicella-zoster virus in utero
+90% of women of childbearing age are immune to VZV, so congenital and neonatal vericella are rare
*Human Immunodeficiency Virus Infection HIV

.generally asymptomatic during the neonatal period
-Diagnosis :
.HIV-1 PCR
-Treatment (treatment that may decrease mother-to-child transmission of HIV) :
.oral administration of zidovudine to pregnant women with HIV infection beginning at 14-34wks gestation and continuing
throughout pregnancy
.IV administration of zidovudine during labor until delivery
.oral administration of zidovudine to the infant for the first 6wks of life
+HIV infection is the neonate results from transplacental transmission of HIV in utero
+80% of perdiatic AIDS cases result from maternal transmission
+Postnatal transmission of HIV from infected mothers through breast milk to infants who were not affected perinatally occurs
in 15% of infants
+HIV may cause acquired immunodeficiency syndrome
+HIV is particularly tropic for cells expressing CD4 including helper T-cells, monocytes and macrophages (it is the infection and
destruction of these cells that cause immunodeficiency)
**Neonatal Seizures
-Differs from childhood/adulthood seizure (Classic tonic-clonic seizure is uncommon)
-Types :
.focal (rhythmic twitching – face/extremities)
.multifocal (involves many muscle group)
.tonic (rigid posturing - fixed eye deviation)
.myoclonic (jerks of distal muscle groups)
.subtle (chewing – staring – blinking – apnea)
-Causes :
st
.hypoxic-ischemic encephalopathy (M.C.C in 1 12-24hr after birth)
.intraventricular hemorrhage (more in preterm infant at 1-3days)
.metabolic causes (hypoglycemia, hypocalcemia, hypomagnesemia, Vit-B 6 deficiency)
.infection (meningitis)
15
+Notes
Page
+Maternal Disease – effect on baby

.cyanotic heart disease – IUGR
.graves – transient thyrotoxicosis
.hyperparathyroidism – hypocalcemia
.ITP (Immune Thrombocytopenic Purpura) – thrombocytopenia
.myasthenia – transient neonatal myasthenia
.SLE – congenital heart block
+Causes Cyanosis
.Congestive heart failure
.Polycythemia
.Cold
.Cor pulmonale
+Associated With Hypoglycemia In The Newborn
.Neonatal asphyxia
.Cold stress
.Diabetic mother
.Sepsis
.It may associate neonatal asphyxia
.It may be manifestation of nesidioblastosis
.Glucagon and steroids may be used as treatment line
.Blood sugar of 45mg% is normal in full term infants
+Causes Of Prematurity
.RDS
.NEC
.jaundice
.intraventricular hemorrhage
.sepsis – GBS (M.C)
.undescended testes

16
Page
Genetics And Dysmorphology
**Genetic Disorders
*Autosomal recessive
-2 abnormal gene to develop disease
.25% (normal)
.50% (carrier, without disease)
.25% (at risk for the disease)
-Examples :
.sickle cell anemia
.cystic fibrosis
.phenylketonuria
.galactosemia
.thalassemia
*Autosomal dominant
-1 abnormal gene is enough to develop disease
.50% (normal)
.50% (at risk for the disease)
-Examples :
.familial hypercholesterolemia
.polycystic kidney disease
.neurofibromatosis type 1-2
.hereditary spherocytosis
.marfan syndrome
.vWD
.hereditary nonpolyposis colorectal cancer
.achondroplasia
.tuberous sclerosis
*X-linked recessive
-Caused by mutations in genes on the X chromosome / Mostly affect males (females – carriers)
.50% of boys (at risk for disease)
.50% of girls (carriers)
-Examples :
.Hemophilia A-B
.G6PD
.becker muscular dystrophy
.duchenne muscular dystrophy
*X-linked dominant
17
-Caused by mutations in genes on the X chromosome / Very rare

-Examples :
Page
.hypophosphatemic rickets
.rett syndrome
.incontinentia pigmenti type 2
.aicardi syndrome
.klinefelter syndrome
*Y-linked
-Also called holandric disorders, are caused by mutations on the Y chromosome. These conditions display may only be
transmitted from the heterogametic sex (e.g. male humans) to offspring of the same sex. More simply, this means that Y-linked
disorders in humans can only be passed from men to their sons; females can never be affected because they do not possess Y-
autosomes. Y-linked disorders are exceedingly rare but the most well-known examples typically cause infertility
-Example :
.hairy pinna
**ABNORMALITIS OF CHROMOSOMES
*Trisomy 21 (Down Syndrome)
-M.C pattern of human malformation
+Increases with advancing maternal age, nondisjunction (The most likely reason for the extra chromosome in Down's syndrome
is Nondisjunction)
-Findings :
.upward slanting palpebral fissures
.speckling of iris (Brushfield spots)
.inner epicanthal folds
.small stature
.mouth open with tongue protrusion
.mild microcephaly
.short neck, flat occiput
.single palmar crease (simian crease)
.hypotonia
.clinodactyly
.recurrent chest infections
.hearing loss
.primary gonadal deficiency
.cardiac anomaly (M.C is ECD, VSD, PDA, MVP)
.gastrointestinal anomalies (duodenal atresia, hirschsprung disease)
.hypothyroidism
.mental retardation,variable
.atlantoaxial instability
.acute lymphocytic leukemia
.early onset of alzheimer disease
*Trisomy 18 (Edwards Syndrome)

-2nd M.C pattern of human malformation
+Increases with advancing maternal age, nondisjunction
-Findings :
.clenched hand
.low set ears
.rocker bottom feet, hammer toe
.omphalocele (GI)
.prominent occiput
.microcephaly
.cardiac anomaly (VSD, ASD, PDA)
.growth deficiency
.mental retardation
18
.many spontaneous abortion

.most don`t survive first year
Page
*Trisomy 13 (patau Syndrome)

+Increases with advancing (older) maternal age
-Findings :
.single umbilical artery
.CNS defects
.severe mental retardation
.microcephaly; microphthalmia
.cleft lip,palate or both
.polydactyly
.cardiac anomaly (VSD, ASD, PDA)
*Klinefelter Syndrome (XXY)
-M.C findings manifested at puberty
-Findings :
.gynecomastia (NO secondary sexual characteristics – FSH high)
.mental retardation (average IQ 85-90)
.behavioral problems
.tall stature and long limbs
.slim
.azoospermia, infertility
.hypogonadism and hypogenitalism (small testes)
*Turner Syndrome (XO)

+NO older maternal age seen
+Paternal chromosome more likely to be missing
-Findings :
.small stature female
.gonadal dysgenesis
.congenital lymphedema
.NO breast (or secondary sexual characteristic)
.broad chest, wide-spaced nipples
.short neck
.renal defects
+A Cardiac Disorder Commonly Associated With Turner's Syndrome Is :
.mitral stenosis
.aortic stenosis
.bicuspid aortic valve
.Pulmonary stenosis
.tetralogy of Fallot
.absence of pulmonary valves
.hypertension
*Fragile Syndrome (X) in boys - (big)

-M.C.C of mental retardation in boys
-Findings :
.macrocephaly
.large testis (macroorchidism)
.large ears
.normal life span
.mild mental retardation
.repeated segment of CGG on X-chromosome
**EARLY OVERGROWTH WITH ASSOCIATED DEFECTS

*Beckwith-Wiedemann Syndrome
19
-Findings:
.macrosomia
Page
.macroglossia
.pancreatic beta cell hyperplasia (hypoglycemia)
.hemihypertrophy (increased risk of abdominal tumors’wilms’)
**CONNECTIVE TISSUE DISORDERS

*Marfan Syndrome
-Autosomal dominant / Mutation in fibrillin gene 15q21.1
-Findings:
.tall stature with long, slim limbs and little fat
.arachnodactyly
.joint laxity with kyphoscoliosis
.pectus excavatum or carinatum
.lens subluxation
.ascending aortic dilation with or without dissecting aneurysm
**Metabolic Disease
+Regarding Acrodermatitis Enteropathica :
.Chronic diarrhea is present
.Caused by zinc deficiency
.Perioral dermatitis
.Loss of hair
+All of the following metabolic disorders are treated by a specifically modified formula and or diet :
.Phenylketonuria
.Maple syrup urine disease
.Galactosemia
.Fructosemia
20 Page

Growth And Development
**Breast Feedin
-Timing (4-6 months)
-Contraindications :
.HIV
.CMV/HSV (if lesion on breast)
.HBV
.Acute maternal disease (TB, sepsis)
.Breast cancer
.Substance abuse
.galactosemia
.phenylketonuria
+Mastitis (NOT contraindication for breast feeding)
-Drugs (absolute contraindications) :
.antineoplastics
.radiopharmaceuticals
.lodide / mercurials
.lithium
.nicotine
.alcohol
.tetracycline
.steroids
.atropin
.metranidazole
.sulfanamides
.chemotherapy
.immunosuppressive
-Step-wise introduction of strained foods :
1.vegetables and fruits
2. dairy, meats(6-9 months; stage 1and 2)
+Foods better saved for 2yrs (Egg whites, Chocolate, Nuts, Citrus, Wheat products, Fish, Honey)
+Don`t give cow-milk until one year of age
+cow-milk (can cause iron deficiency)
+goot-milk (can cause folic acid deficiency)
*Human Milk *Cow Milk

1.water/sloids .same .same
2.calories .20cal/oz .20cal/oz
3.protein .1-1.5% (whey domi.) .3.3%(ceasein domi.)
4.carbohydrate .6.5-7% lactose .4.5% lactose
5.fat .high in LCFAs .high in MCFAs
21
6.minerals .iron better absorbed .low iron and copper

7.Vit .low in K .low in C,D
Page
8.digestibility .faster .after 45days

9.renal solute load .low .higher
+Do nit give infants cow milk prior to one year of age
+Colostrum :
.It has higher protein content than mature milk
.It has less sugar (carbohydrate) content than mature milk
.Sodium and potassium content are higher than in mature milk
.It contains protective antibodies and leukocytes
.Contains less fat than mature breast milk
.Daily secretion is 10-40 ml
.It has an alkaline reaction
+In Breast Milk Feeding :
.Less allergic symptoms than bottle feeding
.Less incidence of gastroenteritis
.Less incidence of URTI
.Increased emotional attachment
.Lactose content in human milk is higher than in cow's milk
.Sodium content in human milk is lower than in cow's milk
.Proteins are lower than in cow's milk
.Protects against infections
.Human milk (breast milk) contains (Vit-A, C, D, B2)
.Human milk is relatively low in (Iron)
+Vitamin deficiency :
.Vit A (night blindness-xerophalmia)
.Vit D (rickets)
.Vit K (bleeding)
.Vit C (gum bleeding-bruises)
.Vit B1(thiamine) (heart failure, beriberi, periphreal neuritis)
.Vit B3(niacin) (dermatitis, diarrhea, pellagra)
.Vit B6(pyridoxal) (dermatitis, neuropathy)
+Failure to thrive (Causes) :
.emotional deprivation or abuse
.malnutrition
.malabsorption
.hypothyroidism
.immunodeficiency
.chronic diseases
+Cow-milk allergy
.protein casein can produce allergy / starts early after exposure to cow milk
.symptoms (GI – diarrhea, constipation, GI bleeding, GERD / Dermatology – skin rash, atopic dermatitis / Respiratory – wheezes)
.treated by (Soy-based formula)
**Newborn Refluxes
+Moro Reflex (Appears - Birth) (Disappears - 4-6 Months)
+Grasp Reflex (Appears - Birth) (Disappears - 4-6 Months)
+Rooting Reflex (Appears - Birth) (Disappears - 4-6 Months)
+Stepping Reflex (Appears - Birth) (Disappears - 4-6 Months)
+Parachute Reflex (Appears 6-8 Months) (Disappears - NO)
**Development
+2-3 months
.social smile (smiles in response to touch and voice)
.head in midline while held sitting
+4 months
22
.laughs
.orients to voice
Page
.coos
.like to look around
+6 months
.rolls over
.sits with pelvic support (tripod)
.feet in mouth is supine
.babbles
.stranger anxiety
+7 months
.rolls from supine to prone
.may crawl
.starts to sit without support
+9 months
.sits alone
.separation anxiety
.creeps-crawls
.pulls to stand
.starting to cruise
.holds bottle
.throws object (not overhand)
.plays gesture games
.says bye-bye, mama-baba, understand (NO), understand gestures
+12 months
.walk with hand support (may walk alone, must by 18months)
.mature pincer grasp
.object permanence (from 10 months)
.says 1-2 words (other than mama-baba)
.comes when called
.cooperates with dressing
+15 months
.creeps up stairs
.walks backward (may walk alone)
.scribbles and builds towers of 2 blocks in imitation (3 cub tower)
.4-6 words
.uses cup and spoon (variable untile 18 months)
+18 months (1.5 years)
.runs
.throws objects overhand while standing
.scribbles spontaneously
.15-25 words
.knowns 5 body parts
.builds tower of 3 blocks cubs
.plays in company of other children
+24 months (2 years)
.walks up and down stairs one foot at a time
.imitates stroke (up or down) with pencil
.builds tower of 7 blocks
.removes clothing
.50 words
.2 word sentences
.follows 2 step commands
.uses pronouns inappropriately
.alternates feet going up the stairs
.pedals tricycle
23
.copies a circle
.undresses completely
Page
.dresses partially
.unbuttons
.dries hand
.>250 words
.3 word sentences
.plurals
.all pronouncs
.group play
.shares
.takes turns
.knows full name, age and gander
.alternates feet going downstairs
.hope and skips
.copies a square
.buttons clothing
.dresses completely
.catches ball
.knows colors
.recites songs from memory
.asks questions
.tells (tall story)
.skips alternating feet
.jumps over lower obstacles
.copies triangle
.ties shoes
.spreads with knife
.paints first name
.asks what a word means
.answer all (wh-) questions
.tells a story
.knows alphabet
.plays cooperative games
.likes to help in household tasks
+A child can usually roll over by age (16 weeks)

+Most normal children can walk at the age of (12 months)
+The average 18 months old child is expected to know (10 wards)
+You must take into account the number of weeks of prematurity to assess development appropriately i.e, per the preterm age,
NOT chronological (A 6 months old baby born at 32 weeks ‘2 months preterm’, must be assessed at 6-2=4 months CORRECTED
AGE. Do this until chronological age 2 yrs, then consider delays to be true
+If there appears to be a language delay, first consider conductive hearing loss. While all babies receive hearing testing within
the first month of life, that is for congenital sensorineural hearing loss. Over the first year of life, conductive hearing loss may
occur from repeated ear infections
**Physical Growth
-3 Parameters (WEIGHT, LENGTH, HEAD CIRCUMFERENCE)
*Weight
-A newborn typically loses up to 10% of BW in the first week of life due to elimination of large amount of extravascular fluid.
Should regain or surpass BW by 2nd weeks
rd th
-A neonate should gain about 30gr per day in the first month of life, which slows to about 20g/day at 3 - 4 months
.At birth (3Kg)
.Double at 6 months (6Kg)
.Triple at 1yr (9Kg)
24
+(1st 4 months - 750gr/per month)

nd
+(2 4 months - 500gr/per month)
Page
+(3rd 4 months - 250gr/per month)

-Growth rate slows further between 6 and 12 months and then appetite begins to decline through 18 months of age
-Then height and weight increase at a steady rate, but head-circumference rate of growth decreases somewhat (2-5yrs)
-Myelination complete by age 7
*Length
-Tall Stature Causes :
.Exogenous obesity
.Endocrine causes
.Androgen excess’tall as children, short as adults
.Syndromes (homocystinuria, Sotos, Klinefelter)
-Short Stature Causes :
.Familial
.pathological (craniopharyngioma, hypothyroidism, hypopituitarism, nutritional problems)
.chronic illnesses
-Boys` highest growth stops at age (18), their peak is (13.5) / Girls` peak is (11.5) and stops at age (16)
.At birth (50cm)
.1st year(increase 25cm)
nd
.2 year(increase 12cm)
+Height - Double at 4 years(100Cm) and Trible at 12 years(150cm)
*Head circumference
.At birth (35cm)
.At 1St year (47cm) (The average growth of head circumference during the first year is 12cm)
.At 12 years (53cm)
+Posterior fontanel - Close at (0-6 Months)
+Anterior fontanel - Close at (14-18 Months)
+Late closure_Hypothyroidism
+Early clousre_Craniosynostosis
**Factors Affecting Physical Growth

.Genetic and hereditary factors
.Race
.Sex (females grow facter than males in 1st 4 years and reach puberty at younger age)
.Nutrition
.Social class
.Chronic diseases
.Upper limb / Lower limb ratio (Birth-1.7) (3 years-1.3) (8 years-1)
** Dentition
-PRIMARY (deciduous)
1.Central inciso (5-7 months)
2.Lateral inciso (7-9 months)
3.Canines (16-20 months)
4.First molar (10-16 months)
5.second molar (20-30 months)
-SECONDARY (permanent)
1.Central inciso (6-7 years)
2.Lateral inciso (7-8 years)
3.Canines (9-11 years)
25 Page

Behavioral And Psychological Disorders
**EATING DISPORDERS
*Pica
-Repeated or chronic ingestion of non-nutritive substances, paint, dirt
+After year 3, needs investigation
-Predisposing Factors :
.mental retardation
.family disorganization
.poor supervision
.psychological neglect, more COMMON with autism, brain behavioral disorders
.increased risk for (lead poisoning, iron deficiency, and parasitic infections)
**ELIMINATION DISORDERS
*Enuresis
-Voluntary or involuntary repeated discharge of urine after a developmental age when bladder control should be present thirst.
Most by age of 5yrs
-Types :
1.Primary
-M.C and usually nocturnal (nocturnal enuresis)
-Causes :
.hypersecration of ADH and/or
.receptor dysfunction
.diminished arousability during sleep
.abnormal bladder function
.anatomic malformation
.pharmacotherapy (imipramine)
2.Secondary
-After a period of dryness > 6 months / F > M
-Causes :
.psychological
.urinary tract infection
.constipation
.diabetes
*Encopresis
-Passage of feces into inappropriate places after a chronologic age of 4yrs, or equivalent developmental level
-Causes :
.psychological (toilet phobia)
.aggressive management of constipation
26
.painful defecation, fissures

+May be (primary-‫( )ما وقف‬secondary-‫)وقف و رجع‬
Page
**ANOTHER DISEASE OF BEHAVIORAL DISORDERS

*Autism
-Develop befor 3 years, usually diagnosed at 1.5 years
-Clinical Findings :
.failure to attach to infants or mother
.delay or abscent social smile
.sterio typical movment
.out burst of angry
.solitary play
.self injury
.possible mental retardation
-Causes :
.genetic and environment (multi factorial)
.impairment of social interaction
.impairment of verbal and non-verbal communication
-Treatment :
.behavioral and psychotherapy
+NO risk for schizophrenia
**ADHD (attension-deficit hyperactivity disorder)

-M.C behavioral disorder in childhood / M > F
-Features :
.inattension
.poor impulse control
.motor overactivity
.start befor child ’7years’ for >6 months
.poor school perfomance
+No clear causes
-Treatment :
.behavioral and psychosocial management
.indecations (amphetomins, methyl phenidote)
27

Page
Immunizations
-Classification Of Vaccines :
+Live Attenuated
1.Viral
.MMR
.varicella
.yellow fever
.nasal influenza
.smallpox
.oral rotavirus
2.Bacterial
.BCG
.oral typhoid
+Inactivated
.Other vaccines
+Live vaccine is delayed (3-11 months)
-Vaccine Rules :
.for stimulation of an adequate and persisting response, 2 or more doses are ususally required
.reactions to live MMR vaccine usually occur (Within minutes after vaccination)
-The following are NOT contraindications to immunizations :
.a reaction to a previous DtaP of temperature <105F, redness, soreness and swelling
.prematurity (immunize at the chronological age)
.a family history of seizures
.a family history of sudden infant death syndrome
-Accepted precautions and contraindications :
.minor illness, with or without a fever, does not contraindicate immunization
.fever is not a contraindication (if fever or other problems suggest moderate or serious illness, the child should not be
immunized until recovered)
.documented agg allergy is not a contraindication to the MMR
.influenza vaccine (and yellow fever) does contain egg protein
.temporary (pregnancy, immunosuppression)
.permanent (severe allergy, encephalopathy)
+Rubella Vaccine :
.It is a live attenuated viral vaccine
.It should not be given to children before the age of one year
.It should not be administered to pregnant women
.It may be associated with arthritis in adult females
*Pertussis can be given to patient with T-cell deficiency
*The national vaccination program in Jordan dont includes vaccination (Hepatitis A)
+Concerning oral polio vaccine OPV (Sabin) :
.OPV is a live attenuated vaccine
28
.OPV is best stored at 2-8° C

.It is easy to administer
.It is of definite value on controlling epidemics of polio
Page
+DPT is a vaccine given to infants usually at 2 months of age, and protects from all of the following disease :
.Diphtheria
.Pertussis
.Tetanus
.Diphtheria and tetanus
+In immunosuppressed children, all of the following vaccines are contraindicated :
.Measles vaccine
.Oral polio vaccine
.Rubella vaccine
.BCG
+In immunocompromised :
.don`t give live attenuated vaccine
.poor response to inactivated vaccine
+Egg hypersensitivity (influenza, yellow fever vaccines)
+IPV, MMR (contain necmycin, streptomycin)
+In premature infants (vaccination given at chronological age)
+Recommended Immunization Schadule (Jordan Vaccination Schedule) :

+Age (1-40 days)
.B.C.G
+2 months
.DPT + polio
.hepatitis (B)
.ACT HIB
.rota virus
+3 months
.DPT + polio
.hepatitis (B)
.ACT HIB
.rota virus
+4 months
.DPT + polio
.hepatitis (B)
.ACT HIB
.P.C.V
+6 months
.P.C.V
+8 months
P.C.V
+9 months
.measles + polio
+12 months
.M.M.R
+13 months
.varicella
+15 months
.P.C.V
+18 months
.DPT + polio
.M.M.R
+19 months
.hepatitis (A)
+25 months
.hepatitis (A)
29 Page

Respiratory Disease
**Acute Inflammatory Upper Airway Obstruction
*Acute Laryngotracheobronchitis (Croup)
-Refers to virus-induced inflammation of the laryngotracheal tissues, resulting in a syndrome of upper airway obstruction.
Because of the narrow caliber of the airway below the vocal cords (Inflammation of subglottic region)
+Most cases occur during the late fall and winter (age 6-36 months) (Recurrences decrease with increasing growth of airway)
-Signs & Symptoms :
.upper respiratory infection-obstruction 1-3 days
.low-grade fever and rhinorrhea (12-24hrs prior to the onset of stridor)
.barky (seal-like) cough
.hoarse voice (sudden onset)
.inspiratory stridor
.worse at night
.respiratory distress
+Respiratory compromise varies from minimal stridor with agitation to severe distress with tachypnea, hypoxia, nasal flaring,
retraction, and impending airway obstruction
-Causes :
.parainfluenza type 1, 2, 3 (M.C)
.other viruses (influenza, respiratory syncytial virus RSV)
-Complications :
.hypoxia only when obstruction is complete
-Diagnosis :
.the diagnosis usually is mafe on the basis of clinical findings
.anteroposterior neck and chest radiographs may reveal (narrow subglottic airway – steeple sign) (X-ray NOT needed)
-Treatment :
.nebulized epinephrine
.corticosteroids (oral, IV or IM) (dexamethasone)
.admession (respiratory distress, cyanosis, ill looking, unreliable parents)
+Croup Could Be Caused By :
.Laryngomalacia
.Foreign body
.Tracheomalacia
.Laryngitis
.Epiglottitis
.Retropharyngeal abscess
+The Differential Diagnosis Of Upper Airway Obstruction :
.epiglottitis
.bacteral tracheitis
.foreign body aspiration
.anaphylaxis
30
.angioneurotic edema
*Epiglottitis
Page
-Inflammation and edema of the epiglottis and aryepiglottic folds (it is considered a life-threatening emergency because of the
propensity of the swollen tissues to result in sudden and irreversible airway occlusion) (Inflammation of epiglottis (upper
airway) – supraglottis)
+Is a medical emergency that requires anethesia for immediate intubation / emergent cricothyroidotomy
+Most cases occur during the winter months in children (3-5 years of age)
-Signs & symptoms :
.fever
.sore throat
.hoarseness
.progressive stridor (develop over 1-2 days)
.sudden onset
.dyspnea and rapidly progressing obstruction
.on examination, the child appear toxic, drools
.difficulty swallowing (dysphagia)
.stridor is a late finding
-Causes :
.H-influenza type B (M.C.C)
.streptococcus pyogenes
.streptococcous pneumoniae
.staphylococcus aureus
.mycoplasma
-Complication :
.complete airway obstruction and death
-Diagnosis :
.clinical first (do nothing to upset child), controlled visualization (laryngoscopy)
.lateral neck radiographs show (thumbrinting) of the epiglottis (X-ray NOT recommended)
-Treatment :
.emergent endotracheal intubation (first step)
.tracheostomy
.IV antibiotics (ceftriaxone) (ampicillin-sulbactam or a third-generation cephalosporin)
+Incidence decreased due to vaccination
**CONGENITAL ANOMALIES OF THE LARYNX

*Laryngomalacia
-Collapse of supraglottic structures during inspiration stridor. Less in prone position, and start in 1 st - 2nd week of life and
symptoms increased up to 6 months of life and exacerbated by any exertion
+M.C.C of stridor in infant and children
-Diagnisis :
.laryngoscopy
.branchoscopy (for associated anomalies)
-Treatment :
.supportive care
.surgery (supraglottoplasty)
*Congenital Subglottic Stenosis

nd
+2 M.C.C of stridor
+No difference (supine vs. Prone position)
-Diagnosis :
.x-ray with laryngoscopy
-Treatment :
.surgery (tracheostomy)
*Vocal Cord Paralysis

+3rd M.C.C of stridor
31
-Diagnosis :
.bronchoscopy
Page
-Treatment :
.usually resolves in 6-12 months
.may require temporary tracheostomy
*Airway Foreign Body

-The highest incidence of foreign body aspiration is noted in children (6-30 months old)
-Aspiration into the lower airways is much more common than tracheal obstruction (While the angle of the Rt. main stem
bronchus in adults favors Rt.-sided aspiration)
+Chronic foreign body aspiration should be considered in Pts. with recurrent focal pneumonias and/or abscesses
+Larynx is the M.C site of foreign body aspiration in children age <1 year
+Rt. mainstem bronchus - are the M.C site body aspiration in children age >1year
+M.C foreign body is peanuts
-Signs & Symptoms :
.acute choking
.coughing
.wheezing
.stridor
.cyanosis
.respiratory distress
+Total obstruction (acute asphyxia, severe retractions with poor chest wall movement)
+Extrathoracix, partial (inspiratory and expiratory stridor, retraction)
+Intrathoracic, partial (expiratory wheeze, stridor)
+Main stem bronchus (cough and expiratory wheeze, there may be blood-tinged sputum)
+Lobar/segmental bronchus (decreased breath sounds over affected lobe, wheezing, rhonchi)
-Complication :
.obstruction
.erosion
.infection (fever, cough, pneumonia, hemoptysis, atelectasis)
-Diagnosis :
.bronchoscopy
-Treatment :
.bronchoscopy
**INFLAMMATORY DISORDERS OF THE SMALL AIRWAYS

*Bronchiolitis
-Is an acute viral lower repiratory tract infection that result in inflammatory obstruction of the peripheral airways
+M.C in children by age <2 years
-Signs & Symptoms :
.the onset is sudden with dyspnea
.severe cough (present always)
.mild URI
.decreased appetite
.fever
.wheezy
.apnea more in young infants
+symptoms lasts average of 12 days (worse in first 2-3 days)
-Causes :
.respiratory syncytial virus RSV (M.C.C)
.parainfluenza
.influenza
.adenovirus
.mycoplasma
-Risk Factors :
.chronic lung disease
.CHD
32
.congenital or acquired immunodeficiencies

+High-risk patients only (hyperimmune RSV IVIG or monoclonal antibody to RSV F protein)
Page
-Complications :
.bacterial suprainfection (hospitalization)
.respiratory insufficiency and failure
.hypoxia
.atelectasis
-Diagnosis :
.clinical
.CXR (should be obtained for ill or hypoxia Pts. and for those wich recurrent or unexplained wheezing) (cuffing appearance)
.PCR
-Treatment :
.supportive care (oxygen, fluid support)
.beta-2 agonist nebulization (salbutamol)
+NO STERIODS ‫الستيرويد بزيد الفايرل انفكشن‬
*Pneumonia
-Inflammation of the lung parenchyma – pulmonary tissue
+(Neonate) M.C.C is GROUP B STREPTOCOCCUS - GBS
+(Children <5yr) M.C.C is virus RSV
+(Children >5yr) M.C.C is M.pneumoniae, S.pneumoniae
-Classifications :
1.Pneumonitis (lung inflammation) (consolidation)
2.Lobar pneumonia (localized inflammation of >1 lobe of lung) (consolidation)
3.Bronchopneumonia (inflammation in bronchioles) (mucopurulent exudate)
+Mycoplasma Pneumonia
.age >5yrs / called (walking pneumonia)
.causes (Streptococcus pneumoniae)
.may have non-productive cough
.CXR findings worse than Pt. Status (interstitial pattern in lower lobes)
+Chlamydia Pneumonia
.age 6wks – 6 months
.causes (chlamydia trachomatis)
.CXR findings (hyperinflation) (ground-glass appearance)
+Viral Pneumonia
.M.C.C of pneumonia in childhood
.causes (RSV, parainfluenza)
.age >3wks - <4yrs
.may have wheezes, stridor
.CXR (diffuse streaky infiltrate)
+Bacterial Pneumonia
.age (neonate-2 months) (causes - GBS, listeria monocytogenes)
.2 months – 4yrs (causes - Streptococcus pneumonia, Staphylococcus Aureus)
.5yrs – 15yrs (causes - Streptococcus pneumonia)
.high fever, dyspnea, consolidation, increased WBC-neutrophils
+Aspiration Pneumonia
.history of toxin inhalation or food aspiration
.CXR (alveolar and rarely reticular infiltrate, localized bilateral)
-Symptoms :
+Viral
st
.1 day (URI symptoms, low-grade fever)
.tachypnea
.cyanosis
.examination-crackles and wheezing
+Bacterial
.more sudden shaking chills
.high fever
33
.dry cough
.examination-breath sounds and dullness to percussion
-Treatment :
Page
.pneumococcus (penicillin)
.viral (supportive)
.chlamydia (erythromycin, azithromycin)
+Clinical Findings in Viral Versus Bacterial Pneumonoa :
.temperature (viral - high) (bacterial – high+)
.URI (viral – +) (bacterial - -)
.toxicity (viral - +) (bacterial - +++)
.WBC (viral – normal or dec.) (bacterial - +++)
.chest x-ray (viral – streaking, patchy) (bacterial – lobar)
.diagnosis (viral – nasopharyngeal washing) (bacterial – blood culture, transtracheal aspirate)
**CYSTIC FIBROSIS CF
-Is an inherited multisystem disease characterized by disordered exocrine gland function
+Is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys, and intestine
+Autosomal Recessive inheritance
+The product of the cystic fibrosis transmembrane regulator CFTR gene is a cell membrane protein that functions as a cAMP-
activated chloride channel on the apical surface of epithelial cells in the respiratory tract, pancreas, sweat and salivary glands,
intestines, and reproductive system
+Cystic fibrosis gen located on chromosome(7)
+CF is major cause of severe chronic lung disease and M.C.C of exocrine pancreatic deficiency in children
+Defect of CT transposrt (abnormal mucus)
+Typical early childhood pathogens include (Staphylococcus aureus and Haemophilus influenzae) / Late childhood and early
adolescence (Pseudomonas aeruginosa)
+Other infection with pathogens typical of CF lung disease, including (Burkholderia cepacia complex, Stenotrophomonas
maltophilia)
-Signs & Symptoms :
+Respiratory (all levels of the respiratory tract may be affected, including the nasal passages, sinuses, and lower airways)
.cough and suptum production
.wheezing and air trapping
.failure to clear mucus secretion
.bronchiectasis
.recurrent respiratory infection
.nasal polyps
.clubbing, cyanosis (late)
.chronic sinus disease
.meconium ileus
.radiographic abnormalities (changes)
+Pancreas and GI manifestations
.pancreatic insufficiency
.bowel obstruction
.rectal prolapse
.diabetes
.malabsorption steatorrhea
.failure to thrive protein-calorie malnutrition) (M.C)
.vitamin deficiency (A, D, E, K)
.hepatobiliary (cirrhosis, gallstones, hepatomegaly, varices, cholelithiasis, ascites)
.recurrent pancreatitis
.hypoproteinemia-edema
+Genitourinary tract
.obstruction azoospermia (in males)
.reduced fertility (in females)
.increased incidence of hernia, hydrocele, undescended testes
+Metabolic Abnormalities
.salt-loss syndrome
.acute salt depletion
34
.chronic metabolic alkalosis

+Sweat gland
Page
.salty taste of skin

+Failure to thrive (M.C manifestation)
-Diagnosis :
.positive newborn screen
.identification of 2CF mutations (homozygous) - DNA testing, isn`t always diagnostic
.BEST TEST (sweat test) (difficult in 1st week of life, confirm positive results, DIAGNOSIS >60 mEq/L)
-Treatment :
.nebulizers
.DNAse (mucolytic)
.antibiotics (tobramycin)
.vitamins supplementation (A, D, E, K)
.pancreatic enzyme replacement
**Sudden Infant Death Syndrome SIDS
-Sudden death of infant, unxplained by history; unxplained by post-mortem examination
-Risk Factors :
.low social class
.african
.highest at 2-4 months of age (most by 6 months)
.more in winter
.M > F
.shorter interpregnancy interval
.less prenatal care
.low birth weight, preterm, maternal smoking, post-natal smoking
.prone-position sleep
.over heating
+Prevention (supine while asleep)
**Apnea
-Cessation of breathing (>20 seconds), especially during sleep
-Types :
.central apnea
.obstructive
.mixed
-Risk Factors :
.adenotonsillar hypertrophy
.trisomy 21
.cleft palate
.macroglossia
.nasal obstruction
.neurological disorders
-Diagnosis :
.sleep-study (polysomnography) (for OSA – obstructive sleep apnea)
-Special Cases of apnea :
1.Apnea of prematurity
.in premature <36wks
.bradycardia
.treated by (theophylline, caffeine)
2.Cyanotic breath holding
.<3yrs
.after agner
3.Obesity hypoventilation
**Acute Otitis Media AOM

-Acute infection of the middle ear
+M.C in children 6-24 months of age
35
-Signs & Symptoms :

.ear pain
Page
.fever
.fussiness
.symptoms of URI (cough, rhinorrhea, congestion)
-Causes :
.viruses (respiratory syncytial virus, parainfluenza virus, influenza viruses)
.may be complicated by bacterial superinfection
.bacteria (S. Pneumoniae, Haemophilus Influenzae, Moraxella Catarrhalis)
+M.C.C is S. Pneumoniae
-Risk Factors :
.smoking
.bottle feeding
.day-care attendance
.allergic disease
.craniofacial anomalies
.immunodeficiency
.genetic tendencies
-Complications :
.tympanic perforation
.chronic suppurative otitis media
.mastoiditis (infection of the mastoid bone of the skull)
.meningitis (most serious complication)
+The diagnosis of AOM (should be made when there is an acute history of symptoms)
-Treatment :
.ampicillin (oral) or ceftriaxone-zinnat
**Tonsillitis
-Is inflammation of the tonsils (Severe throat inflammation) most commonly caused by viral or bacterial infection
-Signs & Symptoms :
.sore throat
.red, swollen tonsils
.pain when swallowing
.high temperature (fever)
.coughing
.headache
.tiredness
.chills
.a general sense of feeling unwell (malaise)
.white pus-filled spots on the tonsils (follicular and membranous exudation)
.swollen lymph nodes (glands) in the neck
.pain in the ears or neck
.weight loss
.difficulty ingesting and swallowing meal/liquid intake
.difficulty sleeping
-Causes :
.viral infection (M.C.C) (adenovirus, rhinovirus, influenza, coronavirus, and respiratory syncytial virus)
+It can also be caused by Epstein-Barr virus, herpes simplex virus, cytomegalovirus, or HIV
.bacterial infection (second M.C.C) (Group A β-hemolytic streptococcus (GABHS), which causes strep throat)
-Complications :
.peritonsillar abscess
.rheumatic fever
.post-streptococcal glomerulonephritis PSGN
-Treatment :
.pain relief, anti-inflammatory, fever reducing medications (paracetamol/acetaminophen and/or ibuprofen)
.sore throat relief (warm salt water gargle, lozenges, dissolved aspirin gargle (aspirin is an anti inflammatory, do not take any
other anti inflammatory drugs with this method), and warm/hot liquids.
+If the tonsillitis is caused by group A streptococcus, then antibiotics are useful, with penicillin or amoxicillin being primary
choices
36
+Chronic cases may be treated with tonsillectomy (surgical removal of tonsils) as a choice for treatment
Page
**Sinusitis
-The maxillary and ethmoid sinuses are present at birth; the sphenoid and frontal sinuses develop later in childhood
+Acute bacterial sinusitis has two common clinical presentations :
.(A) persistent respiratory symptoms (>10 to 14 days) (nasal discharge, cough)
.(B) severe symptoms (high fever, purulent nasal discharge for at least 3 days)
-Differential Diagnosis :
.viral URIs
.allergic rhinitis
.nasal foreign body
-Treatment :
.antibiotics coverage (is similar to that for OM) (should continue longer 14 to 21 days)
+Children with recurrent or chronic sinusitis should be evaluated for cystic fibrosis, ciliary dyskinesia, or primary immune
deficiency
**Allergic Rhinitis
-Nasal congestion and rhinorrhea without upper respiratory infection
+Generally by 6 years of age
-Signs & Symptoms :
.nasal congestion, pruritus
.worse at night with snoring
.mouth breathing watery
.itchy eyes
.Post-nasal with cough
.wheezing
.headache
.rhinorrhea
+Food allergies more common (nuts, seafood) in young children, then skin, gastrointestinal and less often, respiratory
-Physical Examination :
.allergic shiners
.chemosis
.cobblestoning of tarsal conjunctiva
.transverse nasal crease
.pale nasal mucosa
.post-nasal drip (posterior pharynx)
.otitis media with effusion is common
-Risk Factors :
.introduction of formula or solids
.mother smoking before child is 1 year old
.heavy exposure to indroor allergens
.family history of allergic disease (Atopy-asthma)
-Complications :
.chronic sinusitis
.asthma
.middle ear effusion
.tonsil, adenoid hypertrophy
.emotional, psychological problems
-Treatment:
.antihistamine (diphenhydramine...mine) (Drug of choice)
**Insect Venom Allergy

-Systemic allergic responses are IgE-mediated
-Signs & Symptoms :
.local-limited swelling (extensive swelling) / pain <1 day
.angioedema, pruritus, anaphylaxis
37
.toxic, fever, malasie

.vomiting, nausea
.late response-serum sickness
Page
.nephrotic syndrome
.vasculitis
.neuritis
.encephalitis
-Diagnosis :
.skin testing
-Treatment :
.cold compresses
.topical antipruritic
.oral analgesic
.systemic anatihistamine
.remove stingers by scraping
**Food Reactions
-Systemic allergic responses are IgE-mediated
st
+Most infants and young children outgrow and egg allergy (half in 1 3 years)
+Food allergic reaction are M.C.C of anaphylaxis seen in E.R (Increased IgE)
-Signs & Symptoms :
+skin
.urticaria (M.C)
.angioedema (M.C)
.atopic dermatitis
+gastrointestinal
.oral pruritus
.nausea
.vomiting
.diarrhea
.abdominal pain
.eosinophilic gastroenteritis
.enterocolitis
.proctocolitis (presents with bloody stool)
+respiratory
.nasal congestion
.rhinorrhea
.sneezing
.laryngeal edema
.dyspnea
.wheezing, asthma
+cardiovascular
.dysrhythmias
.hypotension
-Diagnosis :
.skin test (IgE-specific allergens are useful for IgE sensitization)
-Treatment :
.epinephrine
**Anaphylaxis
-Severe allergic reaction that can cause laryngeal edema and hypotension
-Sudden release of active mediators with cutaneous, respiratory, cardiovascvular, gastrointestinal symptoms
-Most Common Reasons :
.in hospital (latex, antibiotics, IVIg intravenous immunoglobulin), radiocontrast agents
.out of hospital (food like peanuts, insect sting, oral medications, idiopathic)
+Reactions from ingested allergens are delayed (minutes to 2 hours)
-Treatment :
.injectable epinephrine (drug of choice)
.oxygen and airway management
38
.epinephrine IM (IV for severe hypotension)

.intravenous fluid expansion
Page
.H1 antagonist
.corticosteroids
.nebulized
.short-acting beta-2 agonist (with respiratory symptoms)
.H2 antagonist (if oral allergen)
**Asthma
-Is a chronic disease of the airways characterized by reversible airway obstruction, inflammation and bronchial hyper-
responsiveness
+Most with onset <6 years of age / M > F
+Most common reasons for hospitalization in pediatric practice
+Bronchiolitis is important differential diagnosis
+Common Precipitants (cigarette smoke, upper respiratory infections, pet dander, dust mites, weather changes, exercise,
seasonal or food allergens)
-Signs & Symptoms :
.cough (M.C symptom)
.wheezing (with viral respiratory infections)
.prolonged respiratory infections
.decreased exercise tolerance
.persistent day or nighttime coughing
.prolonged expiratory phase (resulting from obstruction of airflow)
.dyspnea, nasal flaring
.decreased breath sounds
.increased work of breathing
.worse at night
+NO clubbing / Cyanosis is Uncommon
-Risk Factors :
.genetic predisposition (parents with asthma or allergy)
.atopic dermatitis
.allergic rhinitis
.food allergy
.severe respiratory tract infection
.smoking exposure
.low birth-weight
.living in urban areas
-Diagnosis :
.spirometry (gold standard) FEV1 / FVC <0.8
.peak flow PE monitoring (useful for Pts. with moderate to severe asthma)
.exercise challenge (worsening in FEV1 of at least 15%)
+CXR (NOT diagnostic)
.intraluminal inflammation or failure to clear secretion (cystic fibrosis, bronchiolitis, gastroesophageal reflux with aspiration,
tracheoesophageal fistula, primary ciliary dyskinesia)
.intraluminal mass effects (foreign body aspiration, tracheal or bronchial tumors or granulation tissue)
.dynamic airway collapse (tracheobronchomalacia)
.intrinsic narrowing of the airway (congenital or acquired stenosis)
-Treatment :
.inhaled bronchodilators (treatment of choice in an acute asthma exacerbation)
.inhaled corticosteroids (treatment of choice for Pts. with persistent asthma)
+Signs Of Severe Life Threating Asthma :
.silent chest (no wheezes)
.Co2 is normal or high (respiratory acidosis)
.inability to talk a sentence in one breath
.Po2<60 mmHg
.pulse>120
39
+Management Of This Patients (asthmatics) :

Page
.Chest X-ray
.IgE level
.CBC
.Arterial blood gases
+Treatment Of Status Asthmatics :
.Aminophyllin
.Oxygen by nasal catheter
.Intravenous fluids
.Corticosteroids
+Bronchiolitis vs. Asthma
+Etiology
.most RSV
.reversible bronchoconstriction with chronic inflammation
+Age
.infants <1yr
.most start age <5yrs)
+Timing
.winter
.all year, most with URI in winter
+Best initial test
.clinical Dx, CXR only if severe and therefore possibility of secondary bacterial pneumonia
.worsening of FEV1 / FVC with exercise and improvment with beta-agonist
+Treatment
.oxygen, if needed / supportive Rx / may try beta-agonist / ribavirin in severe or worsening cases may prevent the need for
intubation and ventilation
.oxygen / short-acting beta-oxygen / add oral steroid for acute attack / may need chronic maintenance Rx
+Diagnosis key words
.URI from another household contact / getting worse / fever / tachypnea / bilateral expiratory wheezing + respiratory distress /
apnea
.repeated episodes of expiratory wheezing / chronic non-productive cough / chest tightness / respiratory distress / may have
other atopic disease + family history / may occur primarily with URI / cannot make diagnosis of asthma for first time wheezing
in infant with fever
+NOTES
+C1-C9 (increased risk of infection)
+M.C.C of hypercalcemia is primary hyperparathyroidism
+M.C.C of hypercalcemia in ‘in-Pt.’ (malignancy – Bone metastases)
+M.C.C of hypercalcemia in ‘out-Pr.’ (primary hyperparathyroidism)
+Graft versus host disease GVHD (occur after Bone marrow transplant)
+Blood transfusion is contraindicated in Bone marrow transplant
+T-cell (CD4, CD8 ...) (Cell mediated immunity)
+B-cell (plasma cell, bacterial antibody, immunoglobulin, humeral immunity)
+Complement (C1-C9) deficiency (More risk for N.meningitis)
+Neutrophil defect (Increased risk of Streptococcus aureus)
40Page
Cardiology
**Congenital Heart Disease
-Diagnosis usually made by age 1 month in most / 1% in all children
-Murmurs may not be heard in early life (because of increased pulmonary vascular resistance)
-Causes :
.most are unknown
.associated with teratogenic, alcohol, rubella (PDA)
.diabetic mother
.genetic predisposition (trisomies; marfan, noonan syndrome, DiGeorge syndromes)
*Acyanotic Heart Disease (Lt. to Rt. Shunts)
1.ventriculare Septal Defects VSD

-M.C congenital heart disease CHD
+M.C type is membranous
+Shunt determined by (pulmonary vascular resistance-systemic vascular resistance)
+Is an acyanotic congenital heart defect, aka a Left-to-right shunt, so there are no signs of cyanosis in the early stage
-Findings :
.holosystolic murmur (pansystolic murmur)
.loud S2
.heart failure
.Eisenmenger's Syndrome
.dyspnea
.feeding difficulties
.poor growth
.sweating
.pulmonary infection (recurrent infections)
-Complications :
.large defects lead to heart failure
.failure to thrive
.endocarditis
.pulmonary hypertension
.cyanosis
-Diagnosis :
.chest X-ray (large heart, pulmonary edema)
.ECG (LVH)
.echo (BEST)
+Surgery In First Year (Indications) :

.failure to thrive or unable to correct medically
.infants at (6-12) months with large defects and pulmonary artery hypertension
.more than 24 months of age with QP; QS >2:1 (shunt fraction)
2.Atrial Septal Defect ASD

41
-Is a congenital heart defect in which blood flows between the atria (upper chambers) of the heart / increased pulmonary flow /
mostly asymptomatic
Page
+M.C type of AST is (ostium secundum defect)

-Findings :
.systolic ejection murmur (from increased pulmonary flow)
.loud S1
.wide-fixed splitting of S2
-Causes :
.Down syndrome
.Ebstein's anomaly
.Fetal alcohol syndrome
.Holt–Oram syndrome
.Lutembacher's syndrome
-Diagnosis :
.chest X-ray (varying hear enlargement-right ventricular and right atrial)
.ECG (right-axis deviation and RVH)
.echo (BEST)
-Complications :
.dysrhythmia
-Treatment :
.most in infants close spontaneously
+Surgery or transcatheter device closure for all asymptomatic Pts.
+Endocarditis is rare (prophylaxis NOT required)
3.Endocardial Cushion Defect ECD

-When both ASD & VSD
+Pt. with trisomy 21 are at a higher risk for ECD
-Findings :
.heart failure early in infants (hepatomegaly, failure to thrive)
.severe increase in heart size with hyperdynamic precordium
.wildly fixed split S2
.holosystolic murmur
-Complications :
.without surgery (death from heart failure)
.with surgery (arrhythmias, congenital heart block)
-Diagnosis :
.CXR (significant cardiomegaly,increased pulmonary artery and pulmonary blood flow and edem)
.ECG (signs of biventricular hypertrophy, right atrial enlargement, superior QRS axis)
.echo (BEST)
-Treatment :
.surgery
4.Patent Ductus Arteriosus PDA

-Flow of blood from aorta to pulmonary artery
+More in premature babies (F > M)
+Associated with (rubella infection, prematurity)
-Signs & Symptoms :
.if small (no symptoms)
.if large (heart failure, machinery murmur, bounding arterial pulse, wide pulse pressure)
-Complications :
.congestive heart failure
.infective endocarditis
-Treatment :
.may close spontaneously
.indomethacin – NSAID
.surgery
+Rarely close after infancy (repair)
42
5.Pulmonary Stenosis
Page
6.Aortic Stenosis
-M.C.C is bicuspid aortic valve,usually asymptomatic in childrens
-Signs & Symptoms (depends on severity of obstruction) :
.angina
.chest pain
.syncope
.early systolic murmur
.left ventricular hypertrophy
-Treatment :
.balloon valvuloplasty
.valve replacement
7.Coarctation Of The Aorta
-Narrowing of aorta distal to subclavian artery
+Associated with turner syndrome / M > F
-Symptoms :
.increased Bp in upper limb (pink)
.decreased Bp in lower limb (cyanosis)
.delay femoral pulse
.can be presented with HTN in childhood
.systolic murmur
.left ventricular hypertrophy
-Diagnosis :
.CXR (rib notching)
.echo (best)
-Treatment :
.PGE1 (to open ductus arteriosus)
.surgery
*Cyanotic Heart Disease (Rt. to Lt. Shunt)

Cyanotic Lesions Associated With Decreased Pulmonary Blood Flow
1.Tetralogy Of Fallot TOF

-M.C of cyanotic heart disease
-Features (causes Rt. to Lt. shunt) :
.pulmonary stenosis
.right ventricular hypertrophy
.VSD
.overriding aorta
-Signs & Symptoms :
.associated with clubbing
.if sever (early cyanosis)
.cyanosis relieved by squatting position
.Pt. usually has polycythemia (hypoxia)
.paroxysmal hypercyanotic attack
.tachypnea
.syncope
-Diagnosis :
.echo (best)
-Treatment :
.PGE1
.surgery (at age 4-12 months) to remove obstructive muscle valvotomy and patching of VSD
2.Tricuspid Atresia
-No blood from Rt atrium to Rt ventricle, blood pass through foramen ovale to left atrial
-Symptoms :
43
.cyanosis
.increased left ventricle hypertrophy
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-Diagnosis :
.chest X-ray (pulmonary under circulation)
.ECG (left axis deviation plus left ventricular hypertrophy)
.echo (best)
-Treatment :
.PGE1
.surgery
3.Ebstein Anomaly
-Usually associated with lithium use
+Right ventricular output is decreased / Right atrium is huge
-Signs & Symptoms :
.may not present until adolescence or adulthood
.cyanosis
.huge heart
.holosystolic murmur
-Diagnosis :
.chest X-ray (increased right atrium, decreased pulmonary blood flow)
.ECG (normal or prolonged PR interval)
-Treatment :
.PGE1
.surgery
+Ebstein Anomaly (these Pts. may have wolff-parkinson-white syndrome and present with episodes of supraventricular
tachycardia)
Cyanotic Lesions Associated With Increased Pulmonary Blood Flow
4.Transposition Of The Great Vessels TGA

-Cyanosis immediate at birth
-Signs & Symptoms :
.patent ductus arteriosis PDA or ASD
.cyanosis
.tachypnea ensue
.loud S2
.murmur absent or soft systolic ejection murmur at mid left sternal border
-Diagnosis :
.chest X-ray (‘egg on a string’ appearance)
.ECG (norma)
.Echo (BEST)
-Treatment :
.PGE1
.surgery in first 2 weeks
5.Truncus Arteriosus
-Single arterial trunk arise from heart receive blood from both ventricle
+VSD is always present
+pulmonary blood flow is greatly increased and results in heart failure
-Diagnosis :
.chest X-ray (heart enlargement)
.ECG (biventricular hypertrophy)
.echo (BEST)
-Treatment :
.treat heart failure
.surgery in first few weeks of life
6.Eisenmenger`s Syndrome
44
**Infective Endocarditis
Page
+Subacute infective endocarditis / M.C bacteria is (Streptococcus viridans)

+Acute infective endocarditis / M.C bacteria is (staphylococcus aureus)
+Most Pts. have pre existing congenital heart disease
+Most commonly occur after surgical or dental procedures
-Signs & Symptoms :
.prolonged fever
.wight loss
.fatigue
.headache
.clubbing
.murmur
.splenomegaly
.stroke
.petechiae
.CNS abscess, hemorrhage
.arthralgia
.osler nodes (painful nodes on finger or toes)
.janeway lesions
.splinter hemorrhage
.roth spots
-Risk Factors :
.VSD (highest risk Pts. are those with VSD) (Lt. Sided heart obstructive disease)
.TOF
.aortic stenosis
.PDA
.valve replacement
-Complications :
.M.C is heart failure
.pulmonary emboli
.meningitis
.abscess brain, heart, kidney
-Diagnosis :
.positive blood culture
.echo
-Treatment :
.IV antibiotics for 4-6 weeks
+Streptococcus viridans (penicillin) (M.C.C, especially after dental procedures)
+Staphylococcus aureus (nafcillin or oxacillin)
+MRSA (vancomycin)
+Mitral valve (M.C valve infected)
+Streptococcus bovis (associated with colon cancer)
+Staphylococcus epidermidis (prosthetic valve)
+Fungal (post-open heart surgery)
**Acute Rheumatic Fever

-Occur post tonsillitis by group A-streptococcus – B-hemolytic streptococcus / After upper respiratory infection with Croup A
+Peak in children 5-15yrs / 1-3wks post UTI (NOT post skin infection)
+Mitral valve is the M.C valve affected
-Diagnosis :
.by jones criteria -
+Major +Minor
.carditis .fever
.polyarthritis .arthralgia
.erythema marginatum .elevated ESR, CRP
.chorea .prolonged PR interval in ECG
45
.subcutaneous nodules .plus evidence of preceding streptococci infection

-Treatment :
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.bed rest
.antibiotics (pencillin or erythromycin) for 10 days
.anti-inflammatory (aspirin)
**Hypertrophic Obstructive Cardiomyopathy HOCM

-Obstructive left sided congenital heart disease
-Signs & Symptoms :
.weakness
.fatigue
.dyspnea on exertion
.angina
.palpitation
.syncope
.perfusion to brain
.risk of sudden death
.left ventricular enlargement
.systolic murmur (no diastolic)
-Diagnosis :
.echo (best)
-Treatment :
.B-blocker or Ca+ channel blocker
+Digoxin + diuretics are contraindicated
**Dilated Cardiomyopathy
-Extensive left ventricular dilatation
+Decreased contraction of heart
-Signs & Symptosm :
.tachycardia
.decreased pulses
.increased JVP
.hepatomegaly
.lower limp edema
.associated with mitral or tricuspic insuffeciency
-Causes :
.idiopathic (M.C)
.infection
.hypothyroidism
.systemic diseases
.muscular dystrophies
**Myocarditis
-Inflammation of heart muscle
-Signs & Symptoms :
.left side heart failure
.arrhythmia
.sudden death
.can cause pericardial effusion and tamponade
-Causes :
.secondary to infection viral (M.C coxsackie B – adenovirus)
.idiopathic
.TB
.other infections
-Treatment :
.treat heart failure
.transplant
46
.IviG (Intravenous immunoglobulin)

Page
**Pericarditis
-Signs & Symptoms :
.pericardial pain (M.C symptom)
.sharp stabbing pain (M.C symptom)
.aggravated by supine position
.relieved by sitting or leaning forward
-Causes :
-Viral (M.C coxsackie B)
.bacterial infection
.SLE
.rheumatic fever
.uremia
.RA
.leukemia or lymphoma
.post MI (dressler syndrome)
-Diagnosis :
.ECG (diffuse ST-elevation / low voltage QRS)
-Treatment :
.idiopathic or viral (NSAID)
+Systolic Murmur :
.aortic stenosis
.pulmonary stenosis
.mitral regurgitation
.tricuspid regurgitation
+Diastolic Murmur :
.aortic regurgitation
.pulmonary regurgitation
.mitral stenosis
.tricuspid stenosis
+Pancystolic Murmur :
.VSD
.mitral regurgitation
.tricuspid regurgitation
+VSD-ASD-PDA (increased pulmonary flow)
+Common Features Of Heart Failure In Infancy :
.Tachypnea and tachycardia (first signs)
.Gallop rhythm
.Enlarged palpable liver
.Heart rate over 150/min
.Respiratory rate over 40/min
.Poor weight gain
.Excessive perspiration
.Weak cry
.Hepatomegaly
.Feeding difficulties
.Failure to thrive
47
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Gastrointestinal Disease
**ORAL CAVITY
+Fluorosis (is a cosmetic condition that affects the teeth. Its caused by overexposure to fluoride during the first eight years of
life. This is the time when most permanent teeth are being formed)
(white patches to brown discoloration of tooth from high fluride contents)
+Tetracycline (causes brown-yellow discoloration with hypoplasia of teeth)
+Delayed eruption of primary teeth :
.hypopituitarism
.hypothyroidism
.trisomy 21
.rickets
+Dental Caries :
.streptococcus mutans (produce acids - causes dental caries)
.sucrose (M.C cariogenic - decreased fluoride)
*Cleft Lip And Palate

-Multifactorial inheritance - Autosomal dominant in families
-Increase in other malformations with isolated cleft palate
-Most important early issue is feeding (special nipple needed)
-Complications :
.increased risk of otitis media (OM)
.hearing loss
.speech problems
-Treatment :
.surgical correction (lip at 3 months of age) (palate at <1 yr)
**GASTROENTERITIS
*Acute Diarrhea
-M.C.C of diarrhea in infant is ROTA VIRUS
+Causes Of Acute Diarrhea :
.Infant (gastroenteritis, systemic infection, antibiotic)
.Child (gastroenteritis, food poisoning, systemic infection)
.Adolescent (gastroenteritis, food poisoning, systemic infection)
+Causes Of Chronic Diarrhea :
.Infant (postinfectious lactase deficiency, milk/soy intolerance, chronic diarrhea of infancy, celiac disease, cyctic fibroses)
.Child (postinfectious lactase deficiency, IBS, celiac disease,lactose intolerance, giardiasis, laxative abuse)
.Adolescent (IBD, IBS, lactose intolerance, giardiasis, laxative abuse)
+Common Causes Of Acute Diarhhea :
.Bacterial-inflammatory (compylobacter, salmonella, shigella, yersinia, E.coli)
.Viral (rotavirus{M.C}, norovirus, adenovirus, astrovirus, calicivirus)
.Parasitic (giardia lamblia{M.C}, E.histolytica{ameba})
+Common Causes Of Bloody Diarrhea :
.Campylobacter jejuni
48
.Amoeba (E.histolytica)
.Shigella
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.E.coli (enterohemorrhagic / enteroinvasive)

.Salmonella
+Common Causes Of Non-bloody Diarrhea :
.cholera (watery diarrhia)
.giardia
.E.coli (enterotoxigenic-travelers diarrhea / enteropathogenic)
+Major transmission is fecal/oral or by ingestion of contaminated food or water
+NOTES
.campylobacter jejuni (associated with guillain barre syndrome)
.enterohemorrhagic E. Coli 0157:H7 (associated with hemolytic uremic syndrome HUS)
.antidiarrheal compounds should never be used in children
+Organism-Specific Associaations :
.Rotavirus (watery diarrhea, vomiting + fever) (supportive)
.Enteropathogenic E.coli (nurseries, daycare) (supportive in severe cases, neomycin or colistin)
.Enterotoxigenic E.coli (traveler`s diarrhea) (supportive care trimethoprim-sulfamethoxazole in severe cases)
.Enterohemorrhagic E.coli (hemorrhagic colitis, HUS) (supportive care only, NO antimicrobial therapy)
.Salmonella (infected animals and contaminated eggs, milk, poultry) (treatment indicated only fot Pts. who are <3months)
.Shigella (person-to-person spread, contaminated food) (trimethoprim, sulfamethoxazole)
.Campylobacter (person-to-person spread, contaminated food) (self-limiting)
.Yersinia enterocolitica (pets, contaminated food, arthritis, rash) (no antibiotics therapy)
.Clostridium difficile (history of antibiotics use) (metronidazole or vancomycin)
.Staphylococcus aureus (food poisoning-onset within 12hrs of ingestion) (supportive care, antibiotics rarely indicated)
.Entamoeba histolytica (acute blood diarrhea) (metronidazole)
.Giardia (anorexia, nausea, abdominal distension, watery diarrhea, weight loss) (metronidazole, furazolidone)
.Cryptosporidium (mild diarrhea in immuno-compromised infants; severe diarrhea in AIDs Pts.) (raising CD4 count to normal is
best treatment. No proven therapy-antimicrobial, strong supportive care, may try rifabutin)
*Chronic Diarrhea And Malabsorption

-Patterns (from bitrh, after introduction of a new food)
-Clinical Presentation :
.chronic nonspecific diarrhea of infancy (weight, height, and nutritional status is normal and no fat in stool / excessive intake of
fruit juice, carbonated fluids, low far intake usually present in history)
.diarrhea with carbohydrates (CHO malabsorption)
.weight loss and stool with high fat malabsorption
-Workup Of Chronic Diarrhea :
.history and physical, nutritional assessment; stool for pH, fat blood, leukocytes, culture, C.difficile toxin, ova and parasites
.blood studies (complete blood count and differential, ESR, electrolytes, glucose, BUN and creatinine)
.sweat test, 72hr fecal fat, breath hydrogen tests
-Initial Evaluation :
+FAT
.most useful screening test is fat in stool (sudan red test)
.steatorrhea is most prominent with pancreatic insufficiency
.confirm with 72hrs stool for fecal fat (gold standard for steatorrhea)
.serum trypsinogen is also a good screen (reflects residual pancreatic function)
+Screen for CHO malabsorption
.breath hydrogen test (after a known CHO load, the collected breath hydrogen is analyzed and malabsorption of the specific
CHO is identified)
+Protein loss
.cannot be evaluated directly
+Screen – spot stool alpha-antitrypsin level
-More common differential diagnosis of malabsorption (Causes of malabsorption) :
.giardiasis - the only primary infection causing chronic diarrhea and malabsorption
.Hiv (aids) - M.C.C is Cryptosporidium (is the organism most commonly isolated in HIV-positive patients presenting with
diarrhea)
49
.small-bowel disease - gluten enteropathy

.pancreatic insufficiency - fat malabsorption (cystic fibrosis is most common congenital disorder associated with malabsorption)
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.Most common anomaly causing incomplete bowel obstruction with malabsorption is MALROTATION
.short bowel - congenital or postanatal loss of >50% of small bowel with or without a protion of the large intesting (is a
malabsorption disorder caused by the surgical removal of the small intestine)
.gluten enteropathy - celiac disease
.abetalipoproteinemia – sever fat malabsorption from birth
-Laps :
.blood for anti-tissue transglutaminase (IgA) and serum (IgA)
.definitive test (small intestinal biopsy)
+Celiac Disease
-Associated with exposure to gluten / Start 6 months-2yrs (NOT from birth)
-Permanent intolerance / Genetic predisposition
-Signs & Symptoms :
.diarrhea (bloody diarrhea)
.failure to thrive
.growth failure (short stature)
.delayed puberty
.vomiting
.anorexia
.anemia (pallor)
.weight loss
.irritability
.hyposplenism
.decreased Ca+2
.dermatitis herpetiformis (itchy skin rash that looks like eczema)
.mouth sores
-Diagnosis :
.best by small bowel biopsy (loss of microvilli)
.antibody (anti endomysial, anti gliadin) (BEST)
-Treatment :
.gluten-free diet (life-ling)
+Some cases may develop to lymphoma
+Causes of Hyposplenism :
.celiac disease
.ulcerative colitis
.sickle cell anemia
+M.C.C of vomiting in infant is over feeding
*Esophageal atresia (EA)

-Congenital esophageal atresia represents a failure of the esophagus to develop as a continuous passage instead, it ends as a
blind pouch
-M > F / More common in 1st born males
-Signs & Symptoms :
.excessive salivation
.choking
.coughing and sneezing
.cyanosis
.abdomen is flat or scaphoid
.regurgitation
-Associations :
.with other defects (anomalies) – VACTERL :
(Anal atresia, Cardiac defects, Tracheoesophageal fistula and/or Esophageal atresia, Renal & Radial anomalies and Limb defects
association polyhydramnios)
-Diagnosis :
.antenatal ultrasound
-Complications :
50
.aspiration pneumonia
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*Tracheoesophageal Fistula (TEF)

-Is an abnormal connection (fistula) between the esophagus and the trachea. TEF is a common congenital abnormality, but
when occurring late in life is usually the sequela of surgical procedures such as a laryngectomy
+TEF can also occur due to pressure necrosis by a tracheostomy tube in apposition to a nasogastric tube (NGT)
-Signs & Symptoms :
.salivation
.choking
.coughing
.vomiting
.cyanosis coincident with the onset of feeding
+Esophageal atresia and the subsequent inability to swallow typically cause polyhydramnios in utero. Rarely it may present in
an adult
-Associations :
.dysphagia and thoracic problems
+Children with TEF can also be born with other abnormalities, most commonly those described in VACTERL association - a group
of anomalies which often occur together, including heart, kidney and limb deformities. 6% of babies with TEF also have a
laryngeal cleft
*Gastroesophageal Reflux Disease (GERD)

-Relaxed lower esophageal sphincter
-Signs & Symptoms:
.post-prandial regurgitation
.esophagitis
.failure to thrive
.obstructive apnea
.stridor
.lower airway disease (cough, wheezing)
+Symptoms during first few months of life and resolves by (12-24) months of age
-Diagnosis:
.esophageal pH monitoring (BEST) (normal pH in lower esophagus is <4)
.endoscopy (erosive esophagitis and complications)
.laryngotracheobronchoscopy (for extraesophageal GERD)
-Treatment:
.H2 receptor antagonist (ranitidine, cimetidine, famotidine) - FIRST LINE
.proton pump inhibitor (omeprazole, lansoprazole, pantoprazole)-for severe reflux and esophagitis
.surgery-fundoplication for (refractory esophagitis, chronic pulmonary disease, continued obstructive apnea)
*Pyloric Stenosis
-Is narrowing (stenosis) of the opening from the stomach to the first part of the small intestine duodenum, due to enlargement
(hypertrophy) of the muscle surrounding this opening (the pylorus) / Hypertrophy of smooth muscle of pylorus
-Signs & Symptoms :
.first 2-4wks to months of life / Family history / More in first born males
.nonbilious projectile vomiting (recurrent vomiting can cause – hypokalemic, hypochloremic, metabolic alkalosis)
.weight loss
.dehydration
.constipation
.is commoner in 1st born infant
.on exam palpation of the abdomen may reveal a mass in the epigastrium (olive sign)
.peristaltic waves (from Lt. to Rt.)
-Diagnosis :
.ultrasound
-Treatment :
.surgical pyloromyotomy
*Doudenal Atresia
51
-Is the congenital absence or complete closure of a portion of the lumen of the duodenum / Complete obstruction or stenosis
of duodenum caused by an ischemic insult during development of failure of recanalization
-It causes increased levels of amniotic fluid during pregnancy (polyhydramnios) and intestinal obstruction in newborn babies
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-During pregnancy, duodenal atresia is associated with increased amniotic fluid in the uterus, which is called polyhydramnios.
This increase in amniotic fluid is caused by the inability of the fetus to swallow the amniotic fluid and absorb it in their digestive
tract
-Anatomic Location :
.85% are distal to the ampulla of vater
.15% are proximal to the ampulla of cater (these present with nonbilious vomiting)
-Signs & Symptoms :
st
.bilious vomiting (green vomit) – at 1 day (if distal to the ampulla)
.epigastric distention
.plain abdominal film revealing (double bubble)
-Complications :
.serious congenital anomalies.
+Late complications may occur in about 12% of patients with duodenal atresia, and the mortality rate for these complications is
6%
-20-40% of all infants with duodenal atresia have Down syndrome (trisomy 21) / 8% all infants with Down syndrome have
duodenal atresia
-Diagnosis :
.X-ray of the abdomen (double bubble sign)
-Treatment :
.suctioning out any fluid that is trapped in the stomach, providing fluids intravenously
.surgical repair of the intestinal closure
+Causes Of Non Bile Stained Vomiting :
.Feeding problem
.Gastro-esophageal reflux
.Pyloric stenosis
.Hidden infection (meningitis)
*Malrotation and Volvulus

-Incomplete rotation of intestine during fetal development
-Signs & Symptoms:
.most present in 1st year of life with acute or chronic incomplete obstruction
.bilious vomiting
.recurrent abdominal pain with vomiting
-Diagnosis:
.ultrasound or barrium
-Treatment:
.surgery
*Meckel Diverticulum
-A true congenital diverticulum, is a slight bulge in the small intestine present at birth and a vestigial remnant of the
omphalomesenteric duct (also called the vitelline duct or yolk stalk)
-More common in males – most in the first 2yrs of life, but can occur at any age
+Major differential diagnosis (appendicitis)
+M.C congenital GI anomalies / M.C malformation of the GI
+M.C symptoms is painless lower GI bleeding
-Signs & Symptoms :
.the majority of people with a Meckel's diverticulum are asymptomatic (An asymptomatic Meckel's diverticulum is called a
silent Meckel's diverticulum - If symptoms do occur, they typically appear before the age of two years)
.painless rectal bleeding such as melaena-like black offensive stools (M.C symptom)
.intestinal obstruction, volvulus and intussusception
.Meckel's diverticulitis may present with all the features of acute appendicitis (RLQ pain)
.severe pain in the epigastric region is experienced by the patient along with bloating in the epigastric and umbilical regions
.the symptoms are so painful that they may cause sleepless nights with acute pain felt in the foregut region, specifically in the
epigastric and umbilical regions
+In most cases, bleeding occurs without warning and stops spontaneously. The symptoms can be extremely painful, often
52
mistaken as just stomach pain resulting from not eating or constipation

-Possible Complications :
.intestinal hemorrhage (painless)
Page
.intestinal obstruction (M.C complication in adults)

.inflammation (perforation)
+M.C ectopic tissue in a Meckel`s diverticulum (gastric mucosa)
-Diagnosis :
.technetium-99m (99mTc) pertechnetate scan, also called Meckel scan (this scan detects gastric mucosa)
-Treatment :
.surgical, potentially with a laparoscopic resection
+In patients with bleeding, strangulation of bowel, bowel perforation or bowel obstruction, treatment involves surgical
resection of both the Meckel's diverticulum itself along with the adjacent bowel segment, and this procedure is called a "small
bowel resection"
+In patients without any of the aforementioned complications, treatment involves surgical resection of the Meckel's
diverticulum only, and this procedure is called a “simple diverticulectomy”
+Rule of 2's in Meckel's diverticulum :
.occur in 2% of the population
.are 2 inches (5cm) long
.are 2 feet (60cm) from the ileocecal valve
.2/3rds have ectopic mucosa
.2 types of ectopic tissue are commonly present (mostly gastric and pancreatic)
.2% become symptomatic
.symptoms start before 2 yrs of age
*Intussusception
-Is a medical condition in which a part of the intestine invaginates (folds into) into another section of intestine, similar to the
way the parts of a collapsible telescope retract into one another. This can often result in an obstruction. The part that prolapses
into the other is called the intussusceptum, and the part that receives it is called the intussuscepiens
-Telescoping of bowel inside each other / M > F
-Obstruction caused by bowel telesoping into the lumen of adjacent distal bowel
+M.C.C of small bowel obstruction in toddlers <2yrs old (Intussusceptoin)
-The Usual Age At Presentation :
.4-12 months of age (60%)
.by 2yrs of age (80%)
-M.C Site :
.terminal ileum involving ileocecal valve (M.C site is ileocecal)
.extending into ascending colon
-Signs & Symptoms :
.abdominal pain (intermittent moderate to severe cramping abdominal pain)
.nausea, vomiting (sometimes green in color from bile)
.pulling legs to the chest area / cry, draw their knees up to their chest
.Later signs include rectal bleeding, often with (red currant jelly) stool (stool mixed with blood and mucus), and lethargy
.physical examination may reveal a (sausage-shaped) mass (RLQ mass), felt upon palpating the abdomen / empty RLQ on
palpation (dance`s sign)
.dyspnea (difficult or painful breathing) with paroxysms of pain
.Fever is not a symptom of intussusception (intussusception can cause a loop of bowel to become necrotic, secondary to
ischemia due to compression to arterial blood supply. This leads to perforation and sepsis, which causes fever)
+Complication of Henoch-Schönlein purpura (HSP), an immune-mediated vasculitis disease in children
-Causes :
.hyperplasia of Peyer's patches (many Pts. have prior viral illness)
.infections (rotavirus, adenovirus) (M.C.C)
.anatomical factors
.altered motility, duplication
.Meckel's diverticulum
.polyp
.appendix
.idiopathic
+The causes of intussusception in older Pts. (Meckel's diverticulum, polyps, tumors)
53
.acute gastroenteritis (Abdominal pain, vomiting, and stool with mucus and blood are present in acute gastroenteritis, but
diarrhea is the leading symptom)
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.rectal prolapse (Rectal prolapse can be differentiated by projecting mucosa that can be felt in continuity with the perianal skin,
whereas in intussusception the finger may pass indefinitely into the depth of sulcus)
-Diagnosis :
.ultrasound
.barium enema
-Treatment :
.hydrostatic pressure
.surgery
+Other Causes Of Rectal Bleeding :
.anal fissure (M.C.C of lower GI bleeding in infancy)
.accidental swallowing of maternal blood (do Apt test)
.peptic ulcer disease
**CONSTIPATION
*Functional Constipation
-Delay or difficulty in stooling for at least 2 weeks; typically after age 2 years
-may have blood in stool; in physical examination (large volume of stool palpated in suprapubic area; rectal exam shows vault
filled with stool
-Treatment:
.relief of impaction (enema, stool softner - laxative)
.behavioral modification
*Hirschsprung Disease
-Also called congenital megacolon or congenital aganglionic megacolon, is a form of megacolon that occurs when part or all of
the large intestine or antecedent parts of the gastrointestinal tract have no ganglion cells and therefore cannot function
+M > F and 10% of cases are familial / M.C site is Rectum / M.C.C of bowel obstruction in neonate (0-28 days)
+Hirschsprung's disease is diagnosed shortly after birth, although it may develop well into adulthood, because of the presence
of megacolon, or because the baby fails to pass the first stool (meconium) within 48 hours of delivery / Some cases are
diagnosed later, into childhood, but usually before age 10
+It may be associated with an increased incidence of Down's syndrome
-Signs & Symptoms :
.green or brown vomit
.explosive stools after a doctor inserts a finger into the rectum
.swelling of the abdomen (meconium ileus)
.lots of gas and bloody diarrhea
.fecal retention, constipation or abdominal distention
+M.C.C of death in children with HD is (enterocolitis) / Complication (enterocolitis)
-Diagnosis :
.rectal biopsy (Best)
-Treatment :
.surgical removal (resection) of the abnormal section of the colon, followed by reanastomosis
+Functional Constipation VS Hirschsprung Disease :

.onset constipation (after 2yrs of age) (at birth)
.failure to thrive (uncommon) (possible)
.enterocolitis (no) (possible)
.abdominal distention (usually not) (yes)
.poor weight gain (usually not) (common)
.anal tone (normal) (normal)
.rectal (stool in ampulla) (no stool)
.anorectal manometry (distension of rectum – relaxation of internal sphincter) (no sphincter relaxation)
.barium enema (large amount of stool; no transition zone) (transition zone with delayed evacuation)
+Cow's milk protein allergy may present in all of the following ways :
.An exacerbation of eczema
54
.Infantile colic
.Chronic wheezing in the first year of life
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.Bloody colitis in infancy

+The M.C.C of minimal bleeding per rectum in children is fissure in anus
Renal And Urologic Disorders

**Urinary Tract Infection (UTI)
-UTI more common in boys than in girls until after 2nd year
+M.C.C of abdominal pain in children
-Causes :
.E.coli (M.C)
.Klebsiella
.Proteus
.S.saprophit
-Types:
1.Cystitis - inflammation of bladder
.dysuria
.urgency
.frequency
.suprapubic pain
.incontinence
.no fever (unless very young)
2.Pyelonephritis – inflammation of kidney
.abdominal or flank pain
.fever
.malaise
.nausea, vomiting
.diarrhea
.nonspecific in newborns and infants
3.Asymptomatic bacteriuria
.positive urine culture without signs or symptoms
.can become symptomatic if untreated
.F > M (short urethra 4cm)
.urine culture >100.000 bacteria
-Risk Factors :
.F > M
.females (wiping, sexual activity, pregnancy)
.males (uncircumcised)
.both (vesicoureteral reflux VUR, constipation, renal antomic abnormalities)
.in small children (from foley`s catheter or suprapubic)
-Diagnosis :
.urine culture (BEST)
-Treatment :
+cystitis (lower UTI) (amoxicillin, trimethoprin-sulfamethoxazole or nitrofurantion - if no fever)
+pyelonephritis (start with oral antibiotics, unless patient requires hospitalization and IV fluids)
.obtain ultrasound for anatomy, suspected abscess, hyrpnephrosis, recurrent UTI
.obtain voiding cystourethrogram (VCUG) in recurrent UTIs or UTIs with complications or abnormal ultrasound findings (is
needed in febrile UTI to rule out vesicourethral reflux)
**Vesicoureteral Reflux (VUR)

-Is a condition in which urine flows retrograde, or backward, from the bladder into the ureters/kidneys. Urine normally travels
55
in one direction (forward, or antegrade) from the kidneys to the bladder via the ureters, with a 1-way valve at the
ureterovesical (ureteral-bladder) junction preventing backflow
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+Occurs when the submucosal tunnel between the mucosa and detrusor muscle is short or absent
+Could be primary or secondary (obstruction or neurogenic bladder)
+M.C symptom (recurrent UTI)
-Predispose To :
.pyelonephritis (M.C)
.scarring of kidney
.nephropathy
.end stage renal disease (ESRD)
-Signs & Symptoms :
.most children with vesicoureteral reflux are asymptomatic
.hydronephrosis or hydroureter (abnormal widening of the ureter)
.increases risk of urinary tract infection or acute pyelonephritis
.fever
.lethargy
.poor appetite
.foul-smelling urine
.dysuria
.frequent urination
-Grading :
1.reflux to mid-ureter, NO dilation (common for anyone)
2.reflux to renal pelvis, NO dilation
3.mild dilation of the ureter, relnal pelvis
4.dilation of the renal pelvis with moderate ureteral tortuosity
5.gross dilation of the ureter, pelvis, ureteral tortuosity
-Indications for VCUG :
.one UTI (in male)
.2 UTI (in female)
.any febrile UTI
-Diagnosis :
.VCUG cystourethrogram (for diagnosis and grading)
.DMSA scan (for renal scarring and function)
-Treatment :
.amoxicillin or ampicillin (for infants younger than 6 weeks)
.trimethoprim-sulfamethoxazole (co-trimoxazole) (6 weeks - 2 months)
+After 2 months the following antibiotics are suitable :
.nitrofurantoin {5–7 mg/kg/24hrs}
.nalidixic acid
.bactrim
.trimethoprim
.cephalosporins
+Surgery (if medical therapy fails, if grade 5 reflux, if or any worsening on VCUG or renal scan)
**Obstructive Uropathy
-Is a structural or functional hindrance (or obstuction) of normal urine flow, sometimes leading to renal dysfunction (obstructive
nephropathy)
-Signs & Symptoms :
.hydronephrosis
.upper abdominal or flank pain
.pyelonephritis, UTI (recurrent)
.weak, decreased urinary stream
.failure to thrive
.diarrhea
.VUR
+Hydronephrosis (M.C.C of palpable abdominal mass in children)
-Causes :
.urolithiasis
56
.posterior urethral valves

.ureteral herniation
Page
+Posterior urethral valves (M.C.C of severe obstructive uropathy in children; mostly in boys (Males > Females), can lead to end
stage renal disease ESRD, present with mild hydronephrosis to severe renal dysplasia, suspect in a male with a palpable,
distended bladder and weak urinary stream)
+Complications (Pulmonary hypoplasia)
-Diagnosis :
.catheterization
.U/S, CT-scan
.VCUG
-Treatment :
.decompress with catheter
.antibiotics
.vesicostomy
.surgery (transurethral destruction)
**DISEASES PRESENTING PRIMARILY WITH HEMATURIA

*Acute postStreptococcal Glomerulonephritis (PSGN)
-Is a disorder of the glomeruli (glomerulonephritis), or small blood vessels in the kidneys
+It is a common complication of bacterial infections, typically skin infection by Streptococcus bacteria types 12, 4 and 1
(impetigo) but also after streptococcal pharyngitis; mostly in cold weather, for which it is also known as postinfectious or
poststreptococcal glomerulonephritis
.common in children (5-12) years old
.occur (1-2) weeks after strep-pharyngitis (strep-tonsilitis) or (3-6) after skin infection (impetigo)
.ranges from asymptomatic microscopic hematuria to acute renal failure
.hematuria, hypertension, mild edema
.malaise, lethargy, fever, abdominal or flank pain
.can cause acute renal failure (azotemia), heart failure
-Diagnosis :
.urinalysis (RBCs, RBC casts, protein 1-2 + polymorphonuclear cells)
.mild normochromic anemia (hemodilution and low-grade hemolysis)
.low C3 (returns to normal in 6-8 weeks) (C3-C4 low)
.need positive throat culture or increasing antibody titer to streptococcal antigens; best single test in the anti-Dnase antigen
.consider biopsy only in presence of acute renal failure, nephrotic syndrome, absence of streptococcal or normal complement
+Renal biopsy (isn`t routinely indicated)
-Complications :
.hypertention
.acute renal failure
.congestive heart failure
.electrolyte abnormalities
.seizures
.uremia
-Treatment :
.antibiotics for 10 days
.sodium restriction, diuresis
.fluid and electrolyte management
.control hypertension (calcium channel blocker, vasodilator or angiotensin converting enzyme inhibitor)
**OTHER GLOMERULONEPHRITIDES
*IgA Nephropathy (Berger disease)
+M.C type of glomerulonephritis in adults worldwide / M.C.C of glomerulonephritis in world
-Signs & Symptoms :
.macroscopic haematuria (visibly bloody in urine)
.IgA deposits
.a urinalysis will show RBCs
57
.affects young males within (24-48hrs) after an upper respiratory tract or GI infections (M > F)
.can cause (HTN, renal failure)
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.proteinuria usually less than 2gm/day

+Worse prognosis (HTN, male, proteinuria, renal failure) / >20% of adults develop end-stage renal disease (ESRD)
-Treatment :
.cortisone
.cyclophosphamide
.ACE inhibitor for HTN
**Alport Syndrome
-Is a genetic disorder affecting children, characterized by glomerulonephritis, end-stage kidney disease, and hearing loss. Alport
syndrome can also affect the eyes, though the changes do not usually affect sight, except when changes to the lens occur in
later life
+Hereditary nephritis (X-linked dominant); renal biopsy shows foam cells (Genetic family history)
-Signs & symptoms :
.hematuria
.proteinuria
.hearing loss (hearing is normal at birth. Hearing loss develops progressively, usually at the stage when kidney function is
normal)
.eye changes (abnormalities are often be seen including lenticonus, keratoconus, cataracts as well as retinal flecks in the macula
and mid-periphery. Severe cases may require a corneal transplant)
.aortic dissection
.leiomyomas (tumours of smooth muscle affecting the oesophagus and female genital tract, may occur in a rare overlap
syndrome involving the adjacent COL4A5 and COL4A6 genes)
+Membranous Glomerulopathy (M.C.C of nephrotic syndrome in adults)

+Membranoproliferative Glomerulonephritis MPGN (M.C.C of chronic glomerulonephritis in older children and young adults)
**Henoch-Schonlein Purpura
-Small vessel vasculitis with good prognosis
-Can cause intussusception
-Signs & Symptoms :
.purpuric rash (more in gluteal region and lower abdomen)
.joint pain
.abdominal pain
.fever
.resolve spontaneously
-Treatment :
.NSAID
.steroids
**Hemolytic Uremic Syndrome (HUS)

-Is a disease characterized by hemolytic anemia (anemia caused by destruction of red blood cells), acute kidney failure (uremia),
and a low platelet count (thrombocytopenia)
+M.C.C of acute renal failure in children
+HUS is more common in children and it is associated with E.coli (food poisoning)
-Signs & Symptoms :
.HUS occurs after ingestion of a strain of bacteria expressing shiga toxin(s), usually types of E. coli, that expresses verotoxin (also
called Shiga-like toxin)
.once the bacteria colonized, diarrhea followed by bloody diarrhea, hemorrhagic colitis
.HUS develops about 5–10 days after onset of diarrhea (bloody diarrhea)
.oliguria (decreased urine output)
.hematuria (blood in the urine)
.thrombocytopenia (low levels of platelets)
.destruction of red blood cells (microangiopathic hemolytic anemia)
.thrombotic microangiopathy (TMA), include (abdominal pain, low platelet count, elevated lactate dehydrogenase LDH, a
58
chemical released from damaged cells, and which is therefore a marker of cellular damage)
.decreased haptoglobin (indicative of the breakdown of red blood cells)
.anemia (low red blood cell count)/schistocytes (damaged red blood cells)
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.elevated creatinine (a protein waste product generated by muscle metabolism and eliminated renally)
.proteinuria (indicative of kidney injury)
.confusion
.fatigue
.edema (swelling)
.nausea/vomiting
.signs and symptoms of acute kidney failure
.hypertension (high blood pressure)
.myocardial infarction (heart attack)
.stroke
.lung complications
.pancreatitis (inflammation of the pancreas)
.liver necrosis (death of liver cells or tissue)
.encephalopathy (brain dysfunction)
.seizure
.coma
.failure of cardiac, renal, and gastrointestinal (GI) organs
.death, can occur unpredictably at any time, either very quickly or following prolonged symptomatic or asymptomatic disease
progression
+HUS
.hemolytic anemia
.thrombocytopenia
.fever
.renal failure
.don't cause neurological symptoms
+Usually occur post-infection by E.coli 157:H7 (and causes bloody diarrhea)
+Don't give antibiotic for bloody diarrhea (increased risk of HUS) and don't transfuse platelets
-Laps :
.Hb (5-9)
.thrombocytopenia (platelet 20.000-100.000)
.elevated urea, creatinin
.low-grade microscopic hematuria and proteinuria
.WBCs up to (30,000)
-Treatment:
.fluids and electrolytes
.treat hypertension
.early dialysis
+No antibiotics if E.coli O157:H7 (bloody diarrhea) is susbected-treatment (Antibiotics increased risk of developing HUS)
**Polycystic Kidney Disease PKD-PCKD

-Is a genetic disorder (hereditary diseases) in which abnormal cysts develop and grow in the kidneys. Cystic disorders can
express themselves at any point, infancy, childhood, or adulthood
-Is characterized by the presence of multiple cysts (hence, "polycystic") typically in both kidneys; however, 17% of cases initially
present with observable disease in one kidney, with most cases progressing to bilateral disease in adulthood
-Signs & Symptoms :
.high blood pressure
.headaches
.abdominal pain
.blood in the urine
.excessive urination
.pain in the back
-Types :
.autosomal dominant polycystic kidney disease (ADPKD)
.autosomal recessive polycystic kidney disease (ARPKD)
59
*Autosomal Dominant Polycystic Kidney Disease (ADPKD) (Adult type of PCKD)

-Is the M.C of all the inherited cystic kidney diseases and is typically identified in adults
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-There are three genetic mutations in the PKD-1, PKD-2, and PKD3 gene with similar phenotypical presentations
+Gene PKD-1 is located on chromosome 16 and codes for a protein involved in regulation of cell cycle and intracellular calcium
transport in epithelial cells, and is responsible for 85% of the cases of ADPKD
+Gene PKD-2 is located on chromosome 4 and codes for a group of voltage-linked calcium channels
+PKD-3 recently appeared in research papers as a postulated 3rd gene
+Fewer than 10% of cases of ADPKD appear in non-ADPKD families
-Signs & Symptoms :
.bilateral renal cyst
.hematuria
.HTN
.renal failure
-Associated With :
.cysts of the liver and pancreas
.intracranial aneurysm (Berry aneurysm BA) (BA – if ruptured can cause subarachnoid hemorrhage ‘severe causing headache’)
.aneurysm of the circle of wills
.commonly cause microscopic hematuria
.mitral valve prolapse
.can cause hypertensive heart disease
+Mutation on gene 16/4 (autosomal dominant) / Present in adult
*Autosomal Recessive Polycystic Kidney Disease (ARPKD) (Infant type of PCKD)

-Is the lesser common of the two types of PKD and is typically identified in the first few weeks after birth (in infant)
-Signs & Symptoms :
.bilateral renal cyst
.liver fibrosis
.bad prognosis
**Diseases Presenting With Proteinuria

*Nephrotic Syndrome
-Features :
.M.C between (2-6) years age
.proteinuria (>40mg/m2/hour)
.hypoalbuminemia (<2.5g/dL)
.edema (M.C symptom)
.hyperlipidemia (reactive to loss of protein)
+No H.T.N , No hematuria
+M.C.C is the minimal change disease
+Nephrotic syndrome can cause anemia (microcytic) – resistant to iron treatment
-Treatment :
.steroid for (4-6) weeks
-Complications :
.infection – M.C bacteria is pneumococcus (M.C complication, loss of immunoglebulin)
.thromboembolism (loss of anti-thrombin III in-urine)
.microcytic anemia resistant to iron tretment (because of loss of transferrin)
+NOTES
+Children With Nephrotic Syndrome :
.Steroid resistance is associated with a poor prognosis (in children most cases respond to steroids)
.Patients should receive high protein, low fat diet
.Minimal lesion nephrosis is the most common type seen in children
.Low salt diet is important during relapse
.Gross albuminuria is a recognized feature
.Relapse is common
.Kidney biopsy (indicated)
.hyperkalemia is common
60
*Minimal Change Disease

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-Is a disease of the kidney that causes nephrotic syndrome and usually affects children (peak incidence at 2–3 yrs of age)
+M.C.C of nephrotic syndrome (NS) in children between the ages of 1-7
+People with one or more autoimmune disorders are at increased risk of developing minimal change disease. Having minimal
change disease also increases the chances of developing other autoimmune disorders
-Signs & Symptoms :
.proteinuria
.edema (start on face and around eyes, more in morning, and lower extramities)
.hypoalbuminemia
.ascites
+Creatinin is normal
-Causes :
.drugs, especially NSAIDs in the elderly
.malignancy, especially Hodgkin's lymphoma
.allergy
.bee sting
-Treatment :
.steroid for 4-6 weeks
+Indication For Biopsy (NO routinly) :
.hematuria
.HTN
.NO response to steroid
**Male Genitourinary Disorders

*Testicular Torsion
-Occurs when the spermatic cord (from which the testicle is suspended) twists, cutting off the testicle's blood supply, a
condition called ischemia / It is most common just after birth and during puberty (10-20yrs)
+M.C in young children around 15yrs
+M.C.C of testicular pain over (12) years old
-Signs & Symptoms :
.sudden, severe, testicular pain (in groin & lower abdomen)
.tenderness
.nausea and vomiting
.the testis may be higher than its normal position
.mild pyrexia and redness of overlying area may be found
.some of the symptoms are similar to epididymitis though epididymitis may be characterized by discoloration and swelling of
the testis, often with fever
.absent or decreased cremasteric reflex
-Causes :
.M.C underlying cause is a congenital malformation known as a "processus vaginalis or bell-clapper deformity" wherein the
testis is inadequately affixed to the scrotum allowing it to move freely on its axis and susceptible to induced twisting of the cord
and its vessels (tunica vaginalis)
-Diagnosis :
.doppler ultrasound
.Prehn's sign (to help determine whether the presenting testicular pain is caused by acute epididymitis or from testicular
torsion)
-Treatment :
.Orchiopexy (or orchidopexy) (is a surgery to move an undescended (cryptorchid) testicle into the scrotum and permanently fix
it there. Orchiopexy typically also describes the surgery used to resolve testicular torsion)
+Epididymo-orchitis (Is differential diagnosis)
*Testicular Tumors
-Is cancer that develops in the testicles, a part of the male reproductive system / M.C solid tumor of young adult men (20-40yrs)
+Not all lumps on the testicles are tumors, and not all tumors are malignant (cancerous)
-Tumor Markers :
.β-hCG
61
.alpha-fetoprotein (AFP)
.LDH
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-Types :
.seminoma (M.C) (radiosensitive)
.choriocarcinoma (radioresistant)
.embryonal carcinoma (radioresistant)
.teratoma (radioresistant)
-Signs & Symptoms :
.lump or swelling in the testes (painless testicular mass)
.harp pain or a dull ache in the lower abdomen or scrotum
.feeling often described as "heaviness" in the scrotum
.breast enlargement (gynecomastia) from hormonal effects of β-hCG
.low back pain (lumbago) due to the cancer spreading to the lymph nodes along the back
+It is not very common for testicular cancer to spread to other organs, apart from the lungs. If it has, however, the following
symptoms may be present :
.shortness of breath (dyspnea), cough or coughing up blood (hemoptysis) from metastatic spread to the lungs
.lump in the neck due to metastases to the lymph nodes
+Other conditions may also have symptoms similar to testicular cancer :
.Epididymitis or epididymo orchitis
.Hematocele
.Varicocele
-Diagnosis :
.scrotal ultrasound
+Metastasis to para-aortic lymph node
-Treatment :
.radical inguinal orchiectomy
+Biopsy (FNA) is contraindicated
*Varicocele
-Is an abnormal enlargement of the pampiniform venous plexus in the scrotum (dilated testicular vein - pampiniform plexus)
+The pampiniform plexus is a network of many small veins found in the human male spermatic cord. It is formed by the union
of multiple spermatic veins from the back of the testis and tributaries from the epididymis
+Varicocele is M.C.C of male infertility / Most Pt. who has varicocele are fertile
-Signs & Symptoms :
.visible or palpable (able to be felt) enlarged vein
.dragging-like or aching pain within scrotum
.feeling of heaviness in the testicle(s)
.atrophy (shrinking) of the testicle(s)
.alteration of testosterone levels
.benign prostatic hyperplasia (BPH) and related urinary problems
-Causes :
.idiopathic
+A majority of idiopathic varicoceles occur on the left side, because the left testicular vein travels superiorly and connects to the
left renal vein (at a 90-degree angle), while the right testicular vein drains directly into the inferior vena cava. Isolated right
sided varicoceles are rare (Lt. > Rt.)
.pelvic or abdominal malignancy
."Nutcracker syndrome" (non-malignant condition in which the superior mesenteric artery compresses the left renal vein,
causing increased pressures there to be transmitted retrograde into the left pampiniform plexus)
.lifestyle factors such as (activity type, bowel health, testicular temperature)
-Complications :
.affect spermatogenesis
.testicular atrophy
-Indication for surgery :
.pain
.infertility
.testicular atrophy
62
*Epidiymitis
-Ascending, retrograde urethral infection
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-Main cause of acute painful scrotal swelling in a young, sexually active male
-Urinalysis shows PYURIA (can be N.gonorrhoeae {GC} or Chlamydia)
-Treatment:
.antibiotics
.bedreast
+Testicular torsion +epididymo-orchitis

.sudden pain .gradual
.young (10-20yrs) .old age (sexually active)
.pain NOT relieved by elevation .pain relieved by elevation
.NO cremasteric reflex .present cremasteric reflex
.testis horizontal and high in scrotum .dysuria + UTI + fever
.treated by (surgery) .treated by (antibiotics)
+The average fluid requirement in a one year old child is (100-120ml/Kg/24hrs)

63Page
Endocrine Disorders
+Chronological age (actual age)
+Bone age (by using X-ray of hand wrise)
+If chronological age = bone age (genetic short steture, chromosomal abnormalitis)
+If chronological age > Bone age (constitutional delay, chronic systemic disease, endocrine disorders ‘hypothyroidism’)
+If chronological age < Bone age (obesity, precocious puberty, hyperthyroidism, congenital adrenal hyperplasia)
**PITUITARY DISORDERS
*Hypopituitarism
-Deficiency of growth hormone + other hormones (delay in puberty)
-Causes:
.congenital (autosomal dominant, recessive, or X-linked recessive)
.acquired (any lesion that damages thy hypothalamus; M.C is craniopharyngioma)
+M.C is craniopharyngioma. Other (TB, sarcoidosis, trauma)
1.Congenital hypopituitarism
.normal size and weight at birth; then severe growth failure in 1 st year
.infants-present with neonatal emergencies; apnea, hypoglycemia seizures, hypothyroidism and adrenalism in 1 st weeks or boys
with microphallus and small testes + cryptorchidism
2.Acquired hypopituitarism
.growth failure
.loss of weight
.mental retardation
.amenorrhea, hypoglycemia
.symptoms of both decreased thyroid and adrenal function
.headache, vomiting
.visual changes
.NO sexual maturation
.papilledema, cranial nerve palasies (if there is an expending tumor)
-Laps :
.screen for low serum insulin-like growth factor (IGF)-1 and IGF-binding protein-3 (IGF-BP3)
.best test is (growth hormone stimulation test)
.MRI is indicated in all patients with hypopituitarism (superior to CT scan)
.X-ray most helpful with destruction lesions (enlargement of sella, erosions)
.bone age (skeleteal maturation markedly delayed – BA 75% of CA)
-Differential Diagnosis (the major ones) :
.systemic conditions (weight is often proportionally much less than height)
.constitutional delay (delayed BA, delayed adolescent growth spurt, and pubertal development)
.familial short stature (BA=CA, short parents)
.emotional deprivation (psychosocial dwarfism)
-Treatment :
.weekly recombinant growth hormone
*Hyperpituitarism
64
-Rate (most are hormone-secreting adenomas)

+Majority are deficiencies of target organs and because of negative feedback, there are increase in hypothalamus and pituitary
Page
hormones
-Laps :
.screen (IGF-1) and (IGF-BP3) for growth hormones excess; confirm with a glucose suppression test)
.need MRI of pituitary
.chromosomes especially in tall males (decreased upper-to lower-body segment ratio suggests XXY; mental retardation suggests
fragile X)
.thyroid test
*Precocious Puberty
+Girls (sexual development < 8 years old)
+Boys (sexual development < 9 years old)
-Most Common Etiologist :
.non-familial (sporadic) and familial in girls
.hamartomas in boys
-Symptoms:
.advanced height, weight
.advanced bone age
.early epiphyseal closure
-Diagnosis :
.GnRH stimulation test; if positive then order MRI
+Causes Of Precocious Puberty :
.decreased production of cortisol
.increased ACTH
.adrenal hyperplasia
.shunting to androgen synthesis
.masculinizes external genitalia in females (internal organs normal)
*Incomplete Precocious Puberty

-Premature Thelarche :
.usually isolated, transient (from birth due to maternal estrogens)
.may be 1st sign of true precocious puberty
-Premature Adrenarche :
.early adrenal androgen production (variation of normal) – axillary, inguinal and genital hair. It is familial
**THYROID DISORDERS
*Hypothyroidism
1.Congenital hypothyroidism
.most are primary (from thyroid gland)
.may be with or without a goiter
.most common is thyroid dysgenesis (hypoplasia, aplasia, ectopia)
.defect in thyroid hormone synthesis (autosomal recessive)
.radioiodine exposure
.iodine deficiency or endemic goiter
.central hypopituitarism
.transplacental passage of thyrotropin
.exposure to maternal antithyroid drugs
-Clinical Presentation (cretinism) :
.prolonged jaundice
.large tongue
.umbilical hernia
.edema
.mental retardation
.wide fontanels
.hypotonia
65
.mouth open
.decreased appetite
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.increased sleep
.apnea
.sluggish
.decreased reflex
.anemia (macrocytic anemia)
.apathetic appearance
.decreased temperature
.feeding difficulties
.other findings (weight and length normal at birth, feeding difficulties, apnea, sluggish, decreased appetite, increased sleep,
constipation, decreased temperature, skin cold and mottled, preipheral anemia; apathetic appearance)
2.Acquired hypothyroidism
.hashimoto thryroiditis is M.C.C of acquired hypothyroidism
.may be part of autoimmune polyglandular syndrome
.typically presents in adolescence (11yrs)
.other causes (iatrogenic ‘irradiation, medication, surgery, radioiodine’; systemic disease ‘cystinosis, histocytic infiltration)
.may more girls than boys
.first sign usually deceleration of growth
.myxedema, constipation, cold intolerance, decreased energy, increased sleep, delayed osseous maturation, delayed puberty,
headache, visual problems
.diffusely increased, firm, nontender thyroid; but may be atrophic so can be nongoitrous
-Laps (for 1-2) :
.low T3-T4
.increased TSH (best test )
-Treatment :
.sodium thyroxine
+Hashimoto thyroiditis is risk for malignant thyroid lymphoma
+Hashimoto's Thyroiditis :
.Most common cause of juvenile hypothyroidism
.The etiology is autoimmune
.Thyroid antiperoxidase antibodies are positive in 90%
.Lead to growth retardation
+Autoimmune Polyglandular Disease :
.TYPE 1
.Hypoparathyroidism
.Addison disease
.Mucocutaneous candidiasis
.Small number with autoimmune thyroiditis
.TYPE 2 (Schmidt Syndrome)
.Addison disease
.Insulin-dependent DM
.With or without thyroiditis
*Hyperthyroidism
-Excess synthesis and secretion of thyroid hormone by the thyroid / Excess production of TSH “rare” or abnormal thyroid
stimulators. Amiodarone can induce thyrotoxicosis
-Types :
.diffuse toxic goiter (Graves disease) (M.C.C)
.toxic multinodular goiter (Plummer disease) (occurs in 15-20% of Pts. with thyrotoxicosis. Occurs more commonly in elderly
individuals)
.toxic adenoma (is caused by a single hyperfunctioning follicular thyroid adenoma)
+The hypermetabolic effect of thyrotoxicosis affects every organ system
+All thyroid disease occur more frequently in women than in men (F > M)
-Signs & Symptoms :
.nervousness
66
.anxiety
.increased perspiration (sweating)
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.tremor
.hyperactivity, hyperreflexia
.palpitations
.weight loss, increased appetite
.reduction in menstrual flow or oligomenorrhea (infertility)
.tachycardia or atrial arrhythmia
.systolic hypertension
.warm, moist, smooth skin
.lid lag
.exophthalmos (stare)
.muscle weakness
.heat intolerance
.diarrhea
.irritability
.emotional lability
.itchy
+Other Causes Of Thyrotoxicosis :
.struma ovarii is ectopic thyroid tissue associated with (dermoid tumors or ovarian teratomas)
.Pts. with a molar hydatidiform pregnancy or choriocarcinoma have extremely high levels of betal human chorionic
gonadotropin (beta-hCG), which can weakly activate the TSH receptor
+Graves` Disease
-M.C.C of hyperthyroidism in Pt. under age 50yrs F > M / Decreased TSH – Increased T4-T3 / Diffusely enlarged thyroid
-Graves` disease or toxic diffuse goiter = hyperthyroidism + diffuse goiter + exophthalmos + dermopathy
+This is autoimmune disorder (antibody against TSH receptor) caused by stimulatory TSH-receptor antibodies
+It is associated with other autoimmune disease (pernicious anemia, myasthenia gravis, vitiligo, type 1 DM, addison`s disease,
adrenal insufficiency)
-About Grave's Disease (Thyrotoxicosis) :

.eye disease (may be the 1st sign of G.d - exophthalmos)
.increased pulse
.tremor
.palmar erythema
.hair thinning
.lid lag
.lid retraction
.goitre
.pretibial myxoedema
.oedematous swelling
.above lateral malleolus
.thyroid acropachy (clubbing, painful finger and toe swelling)
.mild normocytic anaemia
+2
.leucopenia, Ca increased, LFT increased
.myopathy
.atrial fibrillation
-Treatment of thyrotoxicosis :
.Neomercazole
.Radioactive iodine
.Surgery
.Propranolol inderal
.Carbimazole
.Potassium perchlorate
.Propranolol
.acromegaly
67
.neurosis, anxiety
.pheochromocytoma
.cardiac disease
Page
.ophthalmoplegia and exophthalmos

-Complications :
.unilateral (hoarseness of voice)
.bilateral (respiratory obstruction)
.heart failure (thyrotoxic cardiomyopathy)
.angina
.osteoporosis
.ophthalmopathy
.gynaecomastia
.thyroid storm
-Diagnosis :
.TSH level
.it can be confirmed by iodine-123 uptake
-Treatment :
.antithyroid medications (methimazole, propylthiouracil) – have been used for hyperthyroidism
+Methimazole (the drug of choice for hyperthyroidism in the nonpregnant women) (not safe in pregnancy)
.therapy with radioactive iodine
.thyroidectomy
+Radioactive Iodine Therapy :
.is contraindication in children / pregnancy
+Thyroidectomy (is reserved for special circumstances) :
.severe hyperthyroidism in children
.pregnant women (who are non compliant or intolerant of antithyroid medication)
.Pts. with very large goiters or severe ophthalmopathy
.Pts. who refuse radioactive iodine therapy
+Adverse surgical effects (recurrent laryngeal nerve damage-branch from vagus, hypoparathyroidism-hypocalcemia 2-5 days
after surgery)
**PARATHYROID DISORDERS
*Hypoparathyroidism
-Is decreased function of the parathyroid glands with underproduction of parathyroid hormone. This can lead to low levels of
calcium in the blood. Often causing cramping and twitching of muscles or tetany (involuntary muscle contraction)
-Signs & Symptoms :
.low blood calcium level
.normal muscle contraction
.nerve conduction
.paresthesia
.fatigue
.headaches
.bone pain
.insomnia
.crampy abdominal pain
.hypocalcemia
.phenomenon known as Trousseau's sign of latent tetany
.seizures
.severe irregularities in the normal heart beat
.spasm of the upper part of the airways or the smaller airways known as the bronchi (both potentially causing respiratory
failure)
-Causes :
.after thyroid or parathyroid gland surgery (thyroidectomy)
.autoimmune invasion and destruction is the most common non-surgical cause. It can occur as part of autoimmune
polyendocrine syndromes
.hemochromatosis (can lead to iron accumulation-iron overload and dysfunction of a number of endocrine organs)
.absence or dysfunction of the parathyroid glands is one of the components of chromosome 22q11 microdeletion syndrome
(other names: DiGeorge syndrome, Shprintzen syndrome, velocardiofacial syndrome)
68
.magnesium deficiency
.DiGeorge syndrome, a disease in which hypoparathyroidism can occur due to a total absence of the parathyroid glands at birth.
Page
Familial hypoparathyroidism occurs with other endocrine diseases, such as adrenal insufficiency, in a syndrome called
autoimmune polyglandular failure syndrome type 1 (APS-I)
.idiopathic (of unknown cause), occasionally familial (e.g. Barakat syndrome (HDR syndrome) a genetic development disorder
resulting in hypoparathyroidism, sensorineural deafness and renal disease)
-Diagnosis :
.by measurement of calcium, serum albumin (for correction) and PTH in blood
.ECG for abnormal heart rhythms, and measurement of blood magnesium levels
-Laps :
.decreased calcium (5-7mg/dL)
.increased phosphorus (7-12mg/dL)
.normal or low alkaline phosphatase
.normal magnesium
.low parathyroid hormone
.low 1,25 {OH2}D3 (calcitriol)
.ECG (prolongation of Q-T)
-Treatment :
.IV 10% calcium gluconate and then 1,25 {OH2}D3 (calcitriol); this normalizes the calcium
.Vit D
*Hyperparathyroidism
-Is overactivity of the parathyroid glands resulting in excess production of parathyroid hormone (PTH). The parathyroid
hormone regulates calcium and phosphate levels and helps to maintain these levels
+Primary (M.C)
-Primary hyperparathyroidism results from a hyperfunction of the parathyroid glands themselves
-Over secretion of PTH due to :
.parathyroid adenoma
.parathyroid hyperplasia
.rarely, a parathyroid glands carcinoma
+Secondary
-Secondary hyperparathyroidism is due to physiological secretion of parathyroid hormone (PTH) by the parathyroid glands in
response to hypocalcemia (low or normal blood calcium levels). The M.C.C are Vit D deficiency and chronic kidney failure
+Vit D deficiency lead to absorption of Ca+2 from GI
+2 +2
+PTH (parathyroid hormones) lead to increased Ca / Calcitonin (thyrocalcitonin – parafollicular cells) lead to decreased Ca
-Signs & Symptoms :
.weakness, fatigue
.depression
.bone pain
.muscle soreness (myalgias)
.decreased appetite
.vomiting, nausea
.constipation
.polyuria
.polydipsia
.cognitive impairment
.kidney stones, renal osteodystrophy
.osteoporosis, pathologic fractures, osteomalacia, rickets
.racquet nails (trachyonychia)
.Parathyroid adenomas (very rarely detectable on clinical examination)
+In Hyperparathyroidism
.In primary hyperparathyroidism, parathyroid hormone (PTH) levels will be either elevated or "inappropriately normal" in the
presence of elevated calcium
.In cases of primary hyperparathyroidism or tertiary hyperparathyroidism heightened PTH leads to increased serum calcium
(hypercalcemia)
.In primary hyperparathyroidism, serum phosphate levels are abnormally low as a result of decreased renal tubular phosphate
69
reabsorption
.Alkaline phosphatase levels are usually elevated in hyperparathyroidism
Page
+Vitamin D Deficiency
-M.C.C of rickets
+Low serum phosphate, normal to low serum calcium lead to increased PTH and increased alkaline phosphatase
+Increased 25-hydroxy Vit D
+Fractures, rachitic rosary, craniotabe bone deformities
-Treatment :
.initial Vit D replacement
.I.V 10% Ca+2 gluconate
**ADRENAL DISORDERS
*Congenital Adrenal Hyperplasia (CAH)
+21 Hydroxylase deficiency (M.C.C)
+Autosomal recessive enzyme deficiency
-Signs & Symptoms :
.weakness
.hypotension
.dehaydration
.weight loss, anorexia, vomiting, dehydration
.hypoglycemia
.hyponatremia
.hyperkalemia
-Laps :
.increased 17 hydroxy progestrone
.low sodium (Na) and glucose, cortisol, aldosterone
.increased potasium (k) and acidosis; androstenedione; plasma renin
-Treatment :
.hydrocortisone
.fludrocortisone if salt losing
.corrective surgery for females
+Is a syndrome associated with increased adrenal androgen production because of enzymatic defects
+CAH is the M.C adrenal disorder of infancy and childhood / CAH arises from autosomal recessive mutations
+Common Enzymatic Defects Associated With CAH :
.C-21 (hydroxylase deficiency) – is associated with reduction in aldosterone secretion in one third of Pts (CAH is associated with
virilization) (Pts. may be male at birth with macrogenitosomia)
.C-11 (hydroxylase deficiency) – this can lead to hypertension and hyperkalemia
.C-17 (hydroxylase deficiency) – is characterized by hypogonadism, hypokalemia and hypertension
*Cushing Syndrome
-Is a collection of signs and symptoms due to prolonged exposure to cortisol (group of clinical abnormalities caused by
prolonged exposure to increased amounts of cortisol or related corticosteroids)
-Signs & Symptoms :
.high blood pressure
.abdominal obesity but with thin arms and legs reddish stretch marks, a round red face, a fat lump between the shoulders
.weak muscles, weak bones (proximal myopathy)
.acne, and fragile skin that heals poorly
.changes in mood
.headaches
.chronic feeling of tiredness
.moon face
.excess sweating (dilation of capillaries)
.hirsutism
.insomnia
.impotence (in men)
.amenorrhoea, oligomenorrhea and infertility
.hypercortisolism (which feeds back into the hypothalamus resulting in decreased levels of GnRH release)
.hypokalemia
70
.hypertension
.hypocalcemia
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.hypercholesterolemia
.osteoporosis
.psychiatric symptoms
.ecchymosis
.immune suppression
.rapid weight gain
.irritability, or depression
.muscle and bone weakness
.memory and attention dysfunction
-Causes :
.exogenous administration of glucocorticoids (M.C.C)
.pituitary adenoma secrets ACTH (also known as Cushing's disease)
.adrenal Cushing's, excess cortisol is produced by (adrenal gland tumors, hyperplastic adrenal glands, or adrenal glands cancer)
.small cell lung cancer (increased ACTH)
-Diagnosis :
.the 1-mg overnight dexamethasone suppression test (is the best initial diagnostic test to establish a diagnosis of cushing
syndrome or glucocorticoid excess)
.CT scan and MRI
+High plasma ACTH levels = pituitary or ectopic source
+Low plasma ACTH levels = adrenal tumors or hyperplasia
-Treatment :
.surgical or medical (unresectable tumors are treated with ketoconazole, metyrapone, mitomycin)
*Tall Stature
-Causes :
.familial tall stature
.infant of diabetic mother
.beckwith wiedemann syndrome
.excess growth hormone
.hyperthyroidism
.precocious puberty
.marfan syndrome
.kleinfilter syndrom (47XXY)
.fragile X-syndrome
*Short Stature
.Chronic renal disease
.Hypothyroidism
.Down's syndrome
.Low birth weight
.Emotional deprivation
.Turner's syndrome
.Gluten enteropathy
.Achondroplasia
.Familial
.Corticosteroid therapy
+Mental retardation may be caused by inherited errors of metabolism and can be treated by dietary measures :
.Phenylketonuria
.Hypothyroidism
.Galactosemia
.Tyrosinemia
.Maple syrup urine disease
.Emotional disturbances
.Birth trauma
.Meningitis
.Cerebral lipidoses
71
+The first sign of puberty in males is (Increase size of testicles)

+The first visible sign of puberty in girls is (The appearance of breast buds)
Page
+Precocious puberty is diagnosed in female when secondary sexual characteristics appear before (8 yrs age)
+Caloric requirements by the beginning of the second week of life are (100 Kcal/kg)
+The daily requirement of vitamin D for the newborn is (400IU)
+The daily requirement of (50 mg of vitamin C / 110 calories per kg / 150 ml of water per kg)
+Circumcision is contraindicated in babies with (Hypospadia)
+Causes Of Failure To Thrive
.Emotional deprivation
.Intestinal malabsorption
.Renal tubular disorders
.Chronic heart failure
+Craniotabes Could Be Seen In :
.Rickets
.Hydrocephaly
.Syphilis
.Premature baby
.Marasmus
.Thalassemia
+Clinical Signs Of Marasmus :
.Loss of turgor of skin
.Poor appetite
.Hypotonia
.Subnormal temperature
+Feature Of Kwashiorkor :
.Anorexia
.Liability to infection
.Moon face
.Loss of muscle tissue is present
.Hepatomegaly is due to fatty infiltration
.Pigmented skin is common
.Edema of limbs is usually present
.Failure to thrive and edema are presenting features
.Caused by low proteine diet
.Hypopigmentation of the hair is a feature
.Abdominal distension is a feature
+Enuresis :
.Most children achieve bladder control during day time and night by age 5 years
.Is more common in boys than in girls
.Spontaneous resolution can occur
.Conditioning devices is modality of treatment
+Characteristic of breath holding spells (attacks) :
.It can occur up to age of 4 or 5 years
.It is self-limited
.It does not occur in mentally retarded children
.Are easily confused with a generalized seizure
.May be precipitated by a minor injury
.The history is very important for the diagnosis (it sometimes runs in families)
72

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Rheumatic And Vasculitic Disorders

**Juvenile Idiopathic Arthritis (JIA)
-Idiopathic synovitis of peripheral joints associated with soft tissue swelling and joint effusion
-Criteria :
.age of onset <16 yrs
.arthritis in one or more joints
.duration > 6 weeks
-Gene :
.HLA-DR4 (poor prognosis)
.HLA-DR5 / HLA-DR8
-Pathophysiology :
.vascular endothelial hyperplasia and progressive erosion of articular cartilage and contiguous bone
.DR8 and DR5
-Signs & Symptoms :
.morning stiffness; easy fatigability
.joint pain later in the day
.joint swelling
.joints warm with decreased motion
.pain on motion (no redness)
-Category of disease with 3 types of onset :
.pauciarticular (<5 joints) - joints of lower extremity; almost never upper extremities (NOT upper)
.polyarticular (5 or more joints) - both large and small joints / rheumatoid nodules on extensor surfaces of elbows and achilles
tendon / may have cervical spine involvement
.systemic onset - arthritis and prominent visceral involvement, hepatosplenomegaly, lymphadenopathy, iritis scleritis, serositis,
iridocyclitis, pancytopenia, infection – felty syndrome / increased temperature at least 39c for 2 or more weeks / salmon-
colored rash
-Laps :
.no best test
.increased antinuclear antibodies (ANA) (Good prognosis), mostly with poly-and pauciarticular disease
.positive rheumatoid factor (RF+) (bad prognosis)
-Treatment :
.NSAIDs
.DMARD (methotrexate, corticosteroids, antimalarial, cyclophosphomid, sulfasalozine) (decreased folic acid)
**Systemic Lupus Erythematosus (SLE)

-Autoantibodies against self-antigens
-Increased levels of anti-double-standed DNA (anti-dsDNA)
+Association with HLA B8, DR2, DR3
+Exacerbations promoted by exposure to sunlight and infections
.onset before age 8 unusual;females
.fever, fatigue, arthralgia, arthritis, rash
.skin (malar rash, discoid lesion, livedo reticularis)
.renal (glomerulonephritis, nephrotic syndrome, hypertension, renal failure)
73
.cardiovascular (pericarditis, cardiomegaly, heart failure)

.neurologic (seizures, stroke, aseptic meningitis, psychiatric changes)
.pulmonary (pleuritic pain, pulmonary hemorrhage)
Page
.hematologic (coombs-positive hemolytic anemia, anemia of chronic disease, thrombocytopenia, leukopenia)

.gastrointestinal vasculitis (pain, diarrhea, bleeding, hepatitis, splenomegaly)
.arterial and venous thromboses
-Criteria (Diagnosis) : (Diagnosis >4/11 criteria)
.malar rash (Butter-fly rash)
.discoid rash
.serositis (libman-sacks endocarditis / non-infective endocarditis)
.oral or genital ulcers
.ANA-positive (antinuclear antibody)
.photosensitivity
.neurologic disorders (seizure, stroke, psychosis)
.hematologic disorders (hemolytic anemia, pancytopenia, decreased C3-C4/type hypersensitivity)
.arthritis (Non-determining arthritis)
.immune disorders
.renal disorders (lupus nephritis, glomerulonephritis, nephrotic syndrome, HTN, renal failure)
.anti-DNA antibody / anti-smith antibody / anti-cardiolipin antibody / anti-phospholipid antibody (DVT, PE, recurrent abortion,
inc. PTT)
.other symptoms (fever, fatigue, alopecia, raynaud phenomenon)
-Laps :
.best screen (ANA) – not specific
.best test (anti dsDNA antibody) – most specific, related to disease activity
-Treatment :
.NSAIDs (if no renal disease)
.immunosuppressive
.methotrexate
.patients with thrombosis and antiphospholipid syndrome should receive anticoagulation (use heparine during pregnancy)
.steroids for kidney disease and acute disease
.cyclophosphamide for severe disease
.renal transplant (if renal failure)
+Signs Of Active SLE :
.increased anti dsDNA
.increased ESR
.decreased CRP (C-reactive protein)
.decreased C3, C4 (hypocomplementemia)
+RA (inc. CRP – inc.ESR) / SLE (inc.ESR – dec. CRP) / CRP (c-reactive protein)
**Kawsaki Disease
-Severe acute vasculitis of all blood vessels / M.C medium-sized arteries, especially coronary; 80% are under 5yrs
-Criteria (Diagnosis) :
.fever for > 5 days and not improved with ibuprofen (always present) + 4 of the following :
.intra-oral erythema (without exudate), strawberry tongue, dry and cracked lips
.erythema and swelling of hands and feet; desquamation of fingertips 1-3 weeks after onset
.bilateral conjunctivitis (without exudate)
.non-suppurative cervical lymphadenitis
.rash (NOT vesicular)
-Others :
.irritability, diarrhea, hepatitis, urethritis, arthritis, otitis media
.cardiac (early myocarditis – M.C complication/pericarditis, coronary artery aneurysms in the 2 nd to 3rd week)
.aseptic meningitis
.hydrops of the gallbladder
-Laps :
.WBC-normal to increased;
.increased neutrophil
.increased ESR
.normocytic anemia of chromic disease
.platelets high / normal in 1st week, then significant increase in 2nd -3rd weeks (more than a million)
74
.increased hepatic transminases

.CSF pleocytosis
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-Diagnosis :
.most important ECO; repeat at 2-3 weeks / if normal repeat it 6-8wks and ECG
-Treatment :
.intravenous immunoglobulin (IViG) and high-dose aspirin / warfarin / steroid (if fever)
.infleunza vaccines if in winter (risk of developing reye syndrome)
+Kawasaki disease is one of the few instences in pediatrics for wich you would use aspirin (it is usually avoided because of the
risk of developing reye syndrome) (Aspirin can cause Reye syndrome in children)
+Any child suspected of having kawasaki disease should have an ECHO (the most serious sequelae of kawasaki disease are
cardiac-related)
**Henoch-Schonein Purpura (HSP)

-IgA-mediated vasculitis of small vessels; M.C.C of non-thrombocytopenia purpura in children
+Usually follows an upper respiratory infection
+Most common in children usually 2-8 yrs of age; in winter; M>F
-Signs & Symptoms :
.low-grade fever anf fatigue
.pink maculopapular rash below waist (lower abdomen, gluteal)
.gastrointestinal (abdominal pain, occult blood in stools, diarrhea or hematemesis, can cause intussusception)
.arthritis (large joint)
.renal (25-50% with glomerulonephritis or nephrosis)
.hepatosplenomegaly, lymphadenopathy
.rarely, CNS (seizures, paresis, coma)
-Laps :
.increased platelets, WBC, ESR
.anemia
.increased IgA, IgM
.urine__RBCs, WBCs, casts, albumin
-Diagnosis :
.definitive diagnosis (rarely needed – NOT routinely) with skin biobsy
.renal biopsy
-Treatment :
.intestinal or GI complication (corticosteroids oral or intravenous)
.self limited (symptomatic treatment)
-Complication :
.renal insufficiency/failure (chronic renal insufficiency)
.bowel perforation
.intussusception
.testicular torsion
.scrotal edema
75 Page
Oncology
**LEUKEMIA AND LYMPHOMA
*Acute Lymphoblastic Leukemia (ALL)
-M.C tumor of children (poor prognosis age <1 or >10 rears at diagnosis)
-Signs & Symptoms :
.bone and joint pain, especially lower extremities (M.C)
.signs of bone marrow failure (pallor, bruising, epistaxis, petechiae, purpura, mucous membrane bleeding, lymphadenopathy,
hepatosplenomegaly, joint swellimg)
-Diagnosis :
.anemia
.thrombocytopenia
.WBC mostly <10,000 (atypical lymphocytes)
+Best test is bone marrow biobsy __lymphoblasts
-Treatment :
.remission induction-chemotherapy (vincristine, doxurubcin, prednisolon) (side effect of doxurubcin is cardiotoxicity)
.CNS involvement (methotrexate)
+(maintenance phase for 2-3yrs)
-Complication :
.majority is relapse (increased intracranial pressure ICP or isolated cranial palsies-testicular relapse)
.pneumocystis pneumonia
.other infections because of immunosuppression
.tumor lysis syndrome (cell lysis; result of initial chemotherapy ’hyperuricemia-kalemia’ – ‘hypophosphatemia-calcemia’)
+Treatment for lysis syndrome (IV fluid hydration, alkalinization of urine, prevent uric acid)
-Poor Prognosis :
.WBC >100,000
.age <1 or >10yrs
.slow response to chemotherapy
.chromosomal abnormalities
**Hodgkin Lymphoma
-Most in 15-19 yrs olds
+Ebstein-Barr virus (EBV) may play a role (is risk); immunodeficiencies may predispose
+Reed-Sternberg Cell on Histology
-4 major histologic subtypes :
.lymphpcytic predominant
.nodular sclerosing
.mixed cellularity
.lymphocyte depleted-worst prognosis
-Signs & Symptoms :
.painless and firm cervical or supraclavicular lymph nodes (most common presenting sign)
.anterior mediastinal mass
.night sweats, fever, weight loss, lethargy, anorexia, pruritus
-Diagnosis :
.biobsy
-Treatment :
76
.chemotherapy
.radiation
Page
**Non-Hodgkin Lymphoma
-Malignant proliferation of lymphocytes of T-cell, B-cell, or intermediate-cell origin
-Signs & Symptomos :
.anterior mediastinal mass (respiratory symptoms)
.abdominal pain, mass
.hematogenous spread
.CNS involvement
-Risk Factors :
.EBV_associated with Burkitt lymphoma
.immunodeficiency
-Diagnosis :
.biobsy
-Treatment :
.chemotherapy
.radiation
.surgical excision of abdominal tumors
**BRAIN TUMORS
nd
-2 M.C tumor in children; More common in children <7 yrs of age; High mortality
-Types :
1.infratentorial – M.C in children
2.supratentorial – M.C in adult
+CT scan best initial test for all
+MRI best test, best imaging
*Infratentorial Tumors
(M.C juvenile pilocytic astrocytoma) (classic site is cerebellum) (treatment: surgery, radiation, chemotherapy)
-Others :
+Malignant Astrocytoma GBM (glioblastoma multiforme-poor outcome)(M.C tumor in adult)
+Medulloblastoma (2nd M.C brain tumor in children; M>F; 5-7yrs)(site; midline cerebellar vermis) (treatment : radiation,
chemotherapy)
+Brain stem tumors
+Ependymoma (most posterior fossa)
*Supratentorial Tumors
-Types :
1.Craniopharyngioma
-M.C supratentorial tumors
-M.C.C od panhypopituitarism
-X-ray (calcification)
-Causes :
.panhypopituitarism
.growth failure
.visual loss
-Treatment :
.surgery
.radiation
.No role for chemotherapy
2.Optic nerve glioma
-M.C tumor of optic nerve, benign, slowly progressive
-Associated with neurofibromatosis type 1
-Symptoms :
.unilateral visual loss
.proptosis
77
.eye deviation
.optic atrophy
Page
.strabismus
.nustagmus
-Treatment :
.chemotherapy (if chiasm is involved)
.radiation (if chiasm is involved)
.surgery (if proptosis with visual loss)
**OTHER MALIGNANCIES
*Wilms Tumor
-Nephroblastoma (Wilm`s tumor)
-2en most common malignant abdominal tumor; usual age 2-5yrs; one or both kidneys (bilateral in 7%)
-M.C renal mass in children >1yr; rare befor 1 yr; rarely bilateral
-Associations :
.hemihypertrophy
.aniridia
.genitourinary anomalies
.WAGR (wilms tumor; aniridia; genitourinary anomalies; mental retardation)
-Symptoms :
.abdominal mass (M.C)
.metastasis (M.C site is lung)
-Diagnosis :
.ultrasound (BEST)
.abdominal CT scan
.Treatment :
.surgery
.THEN chemotherapy and radiation
.bilateral renal-unilateral nephrectomy
*Neuroblastoma
-M.C abdominal mass in children <1yr
-Site (abdomen, adrenal, retroperitoneal - cervical; thoracic; or pelvic ganglia)
-Symptoms:
.firm, palpable mass in flank or midline; painful with calcification and hemorrhage
.metastasis (long bones, skull, orbital, bone marrow, lymphnodes, liver, skin)
-Diagnosis :
.X-ray
.CT scan
.MRI (overall best)
.elevated urine (increased homovanillinc acid HVA) and (increased vanillylmandelic acid VMA)
-Treatment :
.surgery
.chemotherapy
.radiation
+Regarding Neuroblastoma :
.Elevated catecholamine level in urine is specific diagnostic feature
.Increased vanillylmindalic acid in most cases
.Final diagnosis depends upon the histological characteristic of tumor or biopsy
.Neuroblastoma is a tumor of young children
*Pheochromocytoma PCC
-Is a rare tumor of adrenal gland tissue (neuroendocrine tumor of the medulla of the adrenal glands)
+It results in the release of too much (secretes excessive amounts of) catecholamines, usually epinephrine-adrenaline and
norepinephrine-noradrenaline, hormones that control heart rate, metabolism, and blood pressure
+It usually develops in the center (medulla) of one or both adrenal glands and associated with paroxysmal hypertension
78
-Signs & Symptoms :

.abdominal-chest pain
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.irritability, anxiety
.nervousness
.pallor
.palpitations
.rapid heart rate (tachycardia)
.severe headache
.sweating (diaphoresis)
.weight loss
.hand tremor
.high blood pressure (HTN)
.sleeping difficulty
.seizures
.visual disturbances
.nausea
.constipation
-Diagnosis :
.urinary metanephrine
.VMA (vanillylmandelic acid)
*Rhabdomyosarcoma RMS
-Is a type of cancer, specifically a sarcoma (cancer of connective tissues), in which the cancer cells are thought to arise from
skeletal muscle progenitors (Malignant tumor of skeletal muscle)
+The most frequently seen malignant urinary bladder tumor in children is (Rapdomyosarcoma botryoides)
**Bone Tumors
*Osteogenic Sarcoma (osteosarcoma)
-Most common bone malignant tumor in children <10yrs; age 10-20 yrs; M>F
+Most pediatric bone tumors are benign (Most commonly osteochondroma)
-Symptoms :
.bone pain
.swelling (M.C)
+The usual age at presentation (between 10 – 20yrs) (M > F)
+The most locations (Two-thirds in the distal femur, proximal tibia – around knee)
+M.C site of metastasis (Lung)
-Diagnosis :
.X-ray (sun brust appearance)
-Metastasis (lung M.C)
*Ewing Sarcoma
-2nd most common malignant bone tumor of children: age 10-20yrs; M>F
-Diagnosis:
.X-ray (onion-like skin)
-Treatment:
.radiation
.surgery
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Neurology
**Neural Tube Defect NTD
1.Spine bifida occulta
-Midline defect of vertebral body without protrusion of neural tissue
-Most are asymptomatic
-May have patch of hair, lipoma, dermal sinus
-Diagnosis by MRI
+Drugs decreased folic acid (and this increased risk of NTD) :
.phenytoin
.methotrexate
.valproic acid
+Neural Tube Defect causes (increase in alfa-feto-protein)
+Folic acid if given preconcepation (decreased risk of NTD)
2.Meningocele
-Meninges herniate through defect in posterior vertebral arches
-Midline lumber mass converd with skin
-MRI should be done / immediate Surgery
+Choroid plexus tumor :
.increased CSF production
.cause hydrocephalus
3.Myelomeningocele
-M.C in lumbosacral lesion
-Low sacral lesion (causes; bladder and GI incontinence)
-Associated with hydrocephalus and chiari malformation
-Head CT-scan for possible hydrocephalus
-Treatment (ventriculoperitoneal shunt/V-P shunt surgery)
-Symptoms :
.falccid paralysis
.no reflexes
.no urinary control
.no bowel control
.no toch sensation
**Hydrocephalus
-Is a medical condition in which there is an abnormal accumulation of cerebrospinal fluid (CSF) in the brain
-Increased CSF amount in ventricle of brain (choroidal cell – produce CSF) (arachnoid villi – absorption for CSF)
-Types :
.obstructive (non-communication) (M.C type)
.non-obstructive (communication)
+Obstructive Hydrocephalus
.M.C type
.M.C.C is cerebral aqueduct stenosis
80
.Causes (cerebral aqueduct stenosis, TORCH infections, chiari malformation, dandy-walker syndrome)
+Non-obstructive
.subrachnoid hemorrhage
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.meningitis
-Symptoms & Signs :
.head circumference
.bulging anterior fonatnel
.distended scalp veins
.broad forehead
.’setting sun’ sign
.increased deep tendon reflexex DTRs
.poor appetite
.vomiting
.papilledema
.headache
.sixth-nerve palsy
.letharge
-Treatment :
.shunting (V-P shunt)
**Chiari Malformation
-Is a condition affecting the brain. It consists of a downward displacement of the cerebellar tonsils through the foramen
magnum (the opening at the base of the skull), sometimes causing non-communicating hydrocephalus, as a result of
obstruction of cerebrospinal fluid (CSF) outflow
-Progressive hydrocephalus with myelomeningocele
th
-Abnormality of hindbrain (4 ventricle)
**Dandy-walker Malformation DWS

-Is a congenital human brain malformation involving the cerebellum and the fluid-filled spaces around it. A key feature of this
syndrome is the complete absence of the part of the brain located between the two cerebellar hemispheres (cerebellar vermis)
-Cystic exponsion of 4th ventricle
-Associated with cerebellar agenesis and corpus callosum agenesis
-Present with large head size and prominent occiput / cerebellar ataxia and delayed motor development
**Seizure
-Epilepsy (2 unprovoked seizures occur >24hrs apart)
*Febrile Seizures
-Common in children (6 months- 6yrs)
-Usually positive family history
-Tempreture increse rapidly >39c
-Usually generalised ‘Tonic-Clonic’ seizure (10-15min) with postictal period
-No increased risk of epilepsy
-May occur future with fever
-You should rule out meningitis
-No routine MRI and ECG
-The younger the age more risk for recurrence
-Treatment (control fever)
*Partial Seizures
1.Simple Partial Seizure
-Asynchronous tonic or clonic movements
-Most on the (face, neck, extremities)
-Avarage duration (10-20) seconds
-Some Pt. have an aura
-May speak or talk during attack
-No post-ictal period
-ECG (multiple focal spikes)
81
-Treatment (phenytoin, anticinvulsants ‘carbamazepine’)

2.Complex Partial Seizure
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-Have an aura
-Loss of conciousness
-Automatisms common after loss of consciousness (lip-smaking, chewing, swallowing, increased salivation)
-M.C patholgy on temporal lobe (sclerosis, hemartoma, cyst, infarction, glioma)
-MRI many will show abnormalities in temporal lobe
-Pt. has post-ictal period
-Treatment (carbamazepine)
*Generalized Seizures
1.Absence (patit mal)
-Sudden cessation of motor activity or speech with blank stare and flickering eyes (5-20sec)
-More in girks; uncommon <5yrs of age
-No aura
-ECG (3sec spike)
-Treatment (ethosuximide)
2.Tonic-Clonic Seizure
-Have aura
-Loss of conciousness
-Tonic rythomic controction of all muscles followed by relaxation
-Tongue biting,apnea,salivation and loss of bladder control
-Treatment (valproic acidmphenobarbital, phenytoin, carbamazepine)
*Myoclonic Seizures
-Repetitive seizures with brief,symmetric muscle contraction and loss of body tone with falling forward
-Treatment (valproic acid)
-Carbamazepaine is contraindicated in myoclonic seizure
*Neonatal Seizures
-Usually present within 12-24hrs after birth
+Commonest type of neonatal seizures is Subtle type

+Possible causes of convulsions in neonate include :
.Hypocalcemia
.Hypoglycemia
.Pyridoxine deficiency
.Intracranial hemorrhage
**Sturge-Weber Syndrome
-Facial nerve (port wine stain) / Nerve is always present at birth (upper face or eyelid) / Glucoma in ipsilateral eye
-M.C symptom is seizure / associated with mental retardation
-Treatment :
.seizure control
.laser for nerve
**Tuberous Sclerosis
-Autosomal Dominant, half with new mutations
-The younger Pts., the higher the likelihood of mental retardation
-Associated with renal tumor
-Symptosm :
.ash-leaf macule (hypopigmented lesion on body)
.CT scan shows (calcified tubers) but may not see till 3-4yrs of age
.brain calcification (CNS tubers)
.seizures
.sebaceous adenoma (small red nodule on nose or cheeks)
82
.shahreen patch
-Diagnosis (clinical)
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-Treatment (seizure control)
**Cerebral Pulsy
-Group of motor syndromes from disorders or early brain development
-M.C.C is intrapartum asphyxia
-Types :
.spastic dysplasia (M.C)
.spastic quadriplegia
.spastic herniplegia
.extrapyramidal
-Symtpoms :
.mainly motor symptoms with mild congnitive impairment
-Risk Factores :
.asphyxia
.low birth weight <1kg
.infection (congenital)
.intraventricular hemorrhage
-Treatment :
.physiotherapy
.muscle relaxant (dantrolene, baclofen, botulinum toxin)
+In kernicterus infants staining of the brain is more intense in the basal ganglion
+Patients with severe cerebral palsy usually die because of chest infections
+Clinical features of basal ganglia disorders :
.Chorea
.Athetosis
.Dystonia
.Hypertonia
+Methylphenidate (Ritalin) is a drug used in the treatment of Hyperkinetic behavior
**Lesch Nyhan Syndrome

-X linked
-Defect purine metabolism (HPRT enzym) (excess uric acid)
-Symptoms :
.choreoathetosis
.spasticity
.renal stones
.gouty arthritis
.delayed motor development
**Muscular Dystrophies
-Duchene muscular dystrophies
-X linked recessive (defect in dystrophia)
-M.C neuromuscular diseaase or muscle fibers
-Is progressive degeneration and death of muscle fibers
-Symprotms :
.1st sign (poor head control in infancy)
.gowers sign (develop at 3-5yrs)
.hip-woddle gait and lordosis
.calf pseudo hypertrpopgy
.wasting of thigh muscle
.weel chair at 12yrs
-Risk Factors :
.respiratory infections
.scloliosis
83
.cardiomyopathy
.low IQ <70
.death around 18yrs (respiratory failure is the M.C.C)
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-Diagnosis :
.genetic test (is the best)
.muscle biopsy
.CPK (is high 15000-35000)
-Treatment :
.digoxin (for heart failure)
.physiotherapy
**Becker Muscular Dystrophy

-X-linked recessive
-Milder than Duchene muscular dystrophy
**Werdnig Haffmann Disease
-Is an autosomal recessive disease caused by a genetic defect in the SMN1 gene, which encodes SMN, a protein widely
expressed in all eukaryotic cells (gene mutation on chromosome 6)
-degeneration of anterior horn of gray-matter / Spinal muscular dystrophy
-M.C.C of hypotonia in infant
-Symptoms :
.abscent grasp reflex
.hypotonia (floppy infant)
.tongue fasciculations
.normal mentality
.normal eye movement
.weak cry and weak-cough
.respiratory paralysis (M.C.C of death)
**Poliomyelitis
-Viral infection
-Feco-oral route of transmition
-Human is the only reservation
-Affect anterior horn of gray matter
-Causes asymetrical weakness and purely motor, sensation is normal
**Mental Retardation
-IQ <75
-Grades :
.mild mental retardation (IQ 50-75) (85-90% of cases)
.moderate (IQ 35-50)
.sever (IQ 20-35)
.profound (IQ 20)
-Causes :
.genetics (fragile X-syndrome ‘M.C.C’ – trisomy 21, 18, 13)
.in born errors of metabolism (phenyl ketonurea, trosynemia, galctocemia)
.neuro degenaration disorders (lipidosis, gangliosidosis, tuberous sclerosis)
+Aquired
.congenital infection (TORCH)
.asphyxia and hypoxia
.intracranial hemorrhage
.meningitis, encephalitis
.hypothyroidism
.poisoning (lead)
**Microcephaly
-Head circumference below 5th percentila
84
-Causes :
.primary (familial, syndrome ‘down, edward’)
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.secondary (radiation, congenital infection - TORCH, meningitic and encephalitis)
**Craniosynostosis
-Premature closure of suture wich can cause samll head
-Treatment (surgery)
Infectious Disease
**MENINGITIS
*Acute Bacterial – Bacterial Meningitis (Older than a neonate)
-First 2 months of life; M.C bacteria is group ‘B.streptococcus’ GBS; E.coli, Listeria
-age 2 months to 12 years; M.C is ‘S.pneumoniae’-‘N.meningitidis’; h.influenzae B
-Pathology (most from hematogenous spread) (rearly from sinusitis, otitis media OM, mastoiditis)
-Symptoms :
.incubation period for bacteria meningitis is 2-3 days
.several days with fever, lethargy, irritability, anorexia, vomiting, nausea, seizure, poor feeding
.then meningeal irritation (photophobia, neck and back pain, and rigidity)
.kernig sign and brudzinski sign (this signs are negative on infant) (neck stiffness is not always positive in children )
.increased ICP suggested by headache, emesis, bulging fontanelles, oculomotor or abducens palsies, hypertension with
bradycardia, apnea, stupor, coma
-Diagnosis :
.lumbar puncture (LP) and blood culture
*Bacterial (decreased glucose - increased protein - WBS >300 - pressure high)
*Viral (normal glucose – slightly high or normal protein - WBC<300 - pressure normal)
.contraindications to immediate LP (increased ICP, severe cardiopulmonary problems, infection of skin)
-Treatment :
.empiric treatment (vancomycin plus either cefotaxime or ceftriaxone)
.dexamethason (if given early, decreased incidence neurologic manifestation)
-Complications :
.increased ICP with herniation and seizures
.sepsis
.cranial nerve palsy (3rd 6th 8th ){6th M.C}, stroke
.most common complication is hearing loss (especially with pneumococcus)
.DIC (Disseminated intravascular coagulation)
*Viral Meningitis (Aseptic)

-Most are self-limited, except herpes simplex virus {HSV} (HSV need treatment with acylovir IV, and usually lozalised to
temporal lobe)
-Symptoms :
.headache and hyperesthesia in older children
.irritability and lethargy in infants
.fever, nausea, vomiting, photophobia, and neck, back, leg pain
-Complications :
.guillain-barre syndrome
.transverse myelitis, hemiplegia, cerebellar ataxia
.most completely resolve without problems except for the neonate with HSV
-Diagnosis :
.PCR OF CSF is the best test
.viral culture
-Treatment :
85
.supportive
.except HSV need treatment with acylovir IV
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*PERTUSSIS (whooping cough)

-Causes (Bordetella pertussis / very contagious - respiratory droplets)
-Vaccination; don`t give life long immunity
-Symptoms :
.catarrhal phase (2 weeks) - influenza like symptoms
.paroxysmal phase (2-5 weeks) - severe coughing paroxysms, respiratory ‘whoop’, facial petechiae
.convalescent phase (>2weeks) - gradual resolution
-Diagnosis :
.nasopharyngeal culture (BEST)
.CXR-usually normal
.CBC-leukocytosis with lymphocytosis
-Treatment :
.erythromycin for 14 days—macrolides (decreased infectious period of patient)
.prophylaxis to all familly members (Vaccination)
+Notes :
.May occur in both sexes
.May occur at any age
.Lesions located principally in bronchiand bronchioles
.The incubation period is 7-10 days
.There is typically marked lymphocytosis
.The paroxismal stage lasts four to six weeks
.Specific treatment in infancy includes erythromycin
*BARTONELLA (cat-scratch disease)

-Bacteria (bartonella henselae)
-incubation period (3-30) days
-Most common cause of lymphadenitis lasting >3 weeks is bartonella
-Symptoms :
.red or white papules along the linear scratch
.chronic regional lymphadenitis
.fever, malaise, headache, anorexia
.less common; hepatosplenomegaly, weight loss, parinaud oculoglandular syndrome
-Treatment :
.usually self limited and resolve in (2-4) months
*Lyme Disease
-Causes :
.Borrelia burgdorferi
-Presentation :
.early localized (erythema migrans / resolve spontaneously)
.early disseminated (cardiac – heart block, myocarditis / neurologic – aseptic meningitis)
.late presentation (after months – arthritis)
-Diagnosis :
.serology (Ab)
-Treatment :
.doxycycline, amoclan
.I.V penicillin
**TUBERCULOSIS
-M.tuberculosis
-Primary complex-affects the lung with local infection with hilar adenopathy
-Diagnosis :
.skin testing
.sputum
86
-Symptoms :
.primary TB usually asymptomatic in children; low grade fever, mild cough, malaise
.infants more likely to have signs and symptoms
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.erosion into blood or lymph

-TB meningitis-most affects brainstem
.cranoal nerve 3, 5, 6 palsies and comminicating hydrocephalus
-Treatment :
.latent TB (INH*9 months)
.primary pulmonary disease (INH+rifampin*6 months)
**VIRAL INFECTIONS
*Measles
-RNA paramyxovirus, very contagious
-Incubation (10-12)days,before prodrome appears {C`3) : (14-21 days-less than 2 weeks)
.cough
.coryza
.conjunctivitis then koplik spots
.koplil spots
.final; rash+fever (rash;macular-starts at head, and spreads downward)
-Complications :
.otitis media (Most common)
.pneumonia
.encephalitis
-Treatment :
.supportive
.vitamin A (if deficient)
+Notes :
.Prodromal signs such as fever and anorexia last 3 to 4 days
.Koplik’s spots are visible 2 to 3 days before the onset of rash
.The rash starts behind the ears and spreads to the forehead, face and down the body
.The incubation period is one to two weeks
.The peak of incidence is among infants
.Vaccine coverage is complete
.The vaccine is made of killed bacteria
.The cold chain is weak
.In measles the rash and accompanying illness reach a climax on about the (6th day)
*Mumps
-Viral infection due to Paramyxovirus transmitted through air-borne droplets ans respiratory/oral secreations
-M.C in winter/spring; incubation period from (14-24) days; contagious 2 day before and 3 days after swellin appears
-Symptoms :
.fever, malaise, headache
.unilateral or bilateral salivary gland swelling (glandular swelling)
.orchitis and oophoritis (rare before puberty)
-Complications :
.meningoencephalomyelitis (M.C)
.pancreatitis
.thyroiditis
.myocarditis
.deafness
.dacryoadenitis
-The percent of persons who develop inapparent infection by Myxovirus parotitis (mumps) is (35%)
*Erythema Infections (fifth disease)

-Due to parvovirus B19, a DNA virus; seen most commonly in spring
87
-Symptoms :
.mild URI symptoms
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.arthritis
.slapped cheek
.lacy, raticular rash over trunk and extremitis
.rash may last up to 40 days
.the rash of erythema infectiosum appears first on the (Face)
-Diagnosis :
.clinical laps not routine except when diagnosing hydrops
-Complications :
.aplastic anemia
*Epstein-Barr Virus
-Infectious mononucleosis; transmitted in oral secretion by close contact ‘kissing disease’; incubation period (30-50) days, most
cases in infants and young children are clinically silent; 1 st human virus to be associated with malignancy :
.nasopharyngeal carcinoma
.burkitt lymphoma
.hodgkin disease
.lymphoproliferative diorders
.leiomyosarcoma in \ immunodeficiency states
-Symptoms :
.insidious onset;prodrome for (1-2) weeks with fever, fatigue, headache, myalgia, sore throat, abdominal pain
.lymphadenopathy, plenomegaly and hepatomegaly
-Diagnosis :
.lymphocytosis
.monospot test
.IgM to viral capsid (IgM-VcA-EBV) antigen is the most specific (up to 4 months)
-Treatment :
.steroids for complications
-Complications :
.splenic hemorrhage or rupture
.swelling of tonsils
.airways obstruction
.neurological complications
.aplastic anemia
.myocarditis
*Influenza Viruses
-three types (etiology); A.B and C
-Sypmtoms :
.abrupt onset with coryza, conjunctivitis, pharyngitis and dry cough
.fever (2-4) days, myalgia, malaise, headache
-Diagnosis :
.ELISA
-Treatment :
.rest and fluid intake
.antiviral drugs
-Complications :
.otitis media
.pneumonia
.secondary bacterial infection
.myocarditis
*Hand/Foot/Mouth disease (Coxsackie A)

-Causes :
.enterovirus
.coxsackie A16
88
-Symptoms :
.ulcerative lesion on oropharynx (dehydration), foot, hand
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*Diphtheria (DTP)
-Caused by gram+ (corynebacterium diphtheriae); this infection is rare nowadays (vaccination); diphtheria produce toxins
-Infection is rare nowadays because of vaccination
-Sypmtoms :
.incubation period (2-7)days
.upper respiratory symptom
.tonsils are coverd by white membrane, lead to hoarsness and inspiratory stridor
+Diphtheria produce toxins which can cause (myocarditis, neuritis in 10% of cases)
+Post-diphteric paralysis (bilateral-symmetrical paralysis motor, descending) (resolve completely within few weeks)
-Treatment :
.antitoxin (IM)
.pencillin (IM)
*Tetanus (ATS)
-Caused by clostridium tetani
-mode of infection (contamination of wound; in new born from umbilical stump)
-Symptoms :
.incubation period (3-14) days
.trismus (lock-jaw / inability to open the mouth)
.fever
.rigidity (neck-back)
.respiratory arrest (M.C.C OF DEATH)
-Treatment :
.isolation and nursing in dark-quiet room
.anticonvulsant (diazepam)
.tetanus immunoglobulin or tetanus antitoxins
.large doses of pencillin
.O2 and vantilation
+Impetigo :
.Bacterial skin infection most common among pre-school children.[1] People who play close contact sports such as rugby,
American football and wrestling or boxing are also susceptible, regardless of age. Antibiotic creams or pills are often used as a
remedy.
.Is highly contagious
.Is usually caused by a staphylococcal infection
.In an infant may be complicated by generalized exfoliation
.It effects mainly the nostrils and perioral areas
*Infectious Mononucleosis (Kissing disease)

-Caused by Epstein barr virus (EBV) / Incubation period 30-50 days / Transmitted by oral secretion (Kissing)
-Symptoms :
.fever, fatigue, headache, myalgia
.lymphadenopathy (mostly cervical lymph nodes)
.Splenomegaly (in 50% of cases)
.Hepatomegaly may present
.Skin rash (maculopapular) may present
.pharyngitis with tonsillar enlargement (can cause air obstruction)
.if Pt. treated with ampicillin (rash will develop 90%)
.ALT and AST may be elevated
.atypical lymphocytes are usually seen in the blood film
-Diagnosis :
.atypical lymphocytosis
.heterophile antibody (monospot test)
.treatment :
.acyclovir
89
+Reye’s syndrome is a disease characterized by :

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.Encephalopathy
.Encephalopathy with recent history of paracetamol intake
.Meningitis with history of salicylate intake
.Meningo-encephalitis
*Poliomyelitis has the longest incubation period
**Osteomyelitis And Septic Arthritis

-Etiology :
+Osteomyelitis
.M.C.C is Staphylococcus aureus (hematogenous)
.in neonate (GBS)
.Pseudomonas (after wonds)
.Salmonella (in sickle cell)
+Septic arthritis
.caused by Staphylococcus aureus
.most in young children, hematogenous; LE>UE and other parts pf body
-Presentation :
.M.C joint us hip
.pain with movement in infants
.older – fever, pain, edema, erythema, warmth, painful limp, or refusal to walk (acute, toxic, high fever)
-Diagnosis :
.blood culture, CBC, ESR
.radiographic studies (initial plain film, U/S for septic arthritis, best test is MRI for osteo)
.x-rays for Pts. with osteomyelitis are initially normal. Changes are not seen until 10-14 days
-Treatment :
.intravenous antibiotics (antistaphylococcus) for 4-6wks
**Osteogenesis Imperfecta
-Autosomal dominant
-M.C genetic cause of osteoporosis
-Defect in Type 1 collagen
-Symptoms :
.fragile bone
.blue sclera
.early deafness

90Page
Hematology
st
-Physiological anemia of infancy (Drop in Hb 1 2-3months, with average Hb 9-11)
-Pathophysiology anemia (decreased erythropoietin in newborn / More in preterm and NO need treatment)
+Erythropoietin (a hormone secreted by the kidneys that increases the rate of production of red blood cells in response to
falling levels of oxygen in the tissues)
*Iron deficiency anemia

-Start 9-24 months
-Risk Factors :
.low iron diet (M.C.C)
.cow milk
.GI bleeding
-Labs :
.decreased bone marrow hemosiderin, lead to
.decreased ferritin. Lead to
.decreased serum Iron, lead to
.increased TIBC (total iron binding capacity), lead to
.microcytosis (low MCV), lead to
.decreased Hb
+Reticulocyte (low)
-Treatment :
.oral ferrous gluconate
.increased dietary iron
.decreased milk uptake
*Lead poisoining
-Blood level up to 10mg/dL is acceptable
-Symptoms :
.behavioral changes (M.C is hyperactivity and aggression)
.development problems
.GI (anorexia, abdominal pain, vomiting, constipation)
.CNS (increased intracranial pressure, seizure, coma, death)
-Risk Factors :
.pre-schools
.low class
.old houses
.leaded gases and paint
-Diagnosis :
.blood lead level (BEST)
.X-ray of long bone (lead lines)
.blood film (microcytic hypochromic anemia with basophilic strippling)
91
-Treatment :
.chelating agent (EDTA/DMSA antidote)
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**Congenital Anemia
*Diamon-Blackfan anemia
-Pure red cell anemia. Is a congenital erythroid aplasia that usually presents in infancy. DBA causes low red blood cell counts
(anemia), without substantially affecting the other blood components (the platelets and the white blood cells), which are
usually normal. This is in contrast to Shwachman–Bodian–Diamond syndrome, in which the bone marrow defect results
primarily in neutropenia, and Fanconi anemia, where all cell lines are affected resulting in pancytopenia
-This is in contrast to Shwachman–Bodian–Diamond syndrome, in which the bone marrow defect results primarily in
neutropenia, and Fanconi anemia, where all cell lines are affected resulting in pancytopenia
+(low RBC / WBC and platelet ar normal / don`t cause pancytopenia)
+Shwachman–Bodian–Diamond syndrome
.is a rare congenital disorder characterized by exocrine pancreatic insufficiency, bone marrow dysfunction, skeletal
abnormalities, and short stature. After cystic fibrosis (CF), it is the second most common cause of exocrine pancreatic
insufficiency in children
-Symptoms :
.congenital anomalies
.short stature
.triphalangeal thumb
.limb and facial dysmorphysim
-Labs :
.macrocytosis
.elevated adenosine deaminase levels in red blood cells
.low reticulocyte (immature red blood cells) counts
.diminished erythroid precursors in bone marrow (decreased Bone marrow production)
.elevated serum Iron
-Treatment :
.corticosteroids and deferoxamine
.splenectomy
.stem cell transplant (BEST)
*Fanconi anemia (congenital pancytopenia)

-M.C.C of congenital pancytopenia is Fanconi pancytopenia / Age from infant to adult
-FA is the result of a genetic defect in a cluster of proteins responsible for DNA repair. As a result, the majority of FA patients
develop cancer, most often acute myelogenous leukemia. and develop bone marrow failure (the inability to produce blood
cells) by age 40
-Symptoms :
.hyperpigmentation
.abscent or hypoplastic thumb
.short stature
.associated with renal tubular acidosis
.abnormalities of the skin, arms, head, eyes, kidneys, and ears, and developmental disabilities
-Labs :
.macrocytosis
.low WBC
.low platelet
.bone marrow hypoplasia
-Complications :
.risk
-Diagnosis :
.bone marrow biopsy
-Treatment :
.corticosteroids, androgens
.bone marrow transplants (BEST)
92
**Megaloplastic Anemia
-Due to folic acid or Vit B12 deficieny
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-Hypersegmented neutrophils, low serum folate, low VitB 12 or both, macrocytosis
*Folic acid deficiency (B9)

-Age 4-7 months / source of folic acid (green vegetables, fruit)
-Causes :
.inadequate intake
.chronic hemolysis
.goat milk feeding
-Treatment :
.oral folic acid
+Women with folate deficiency who become pregnant are more likely to give birth to low birth weight premature infants, and
infants with neural tube defects NTD
*Vitamin B12 (cobalamin) deficiency
-Age 3-5yrs
-Risk Factors :
.vegetarian
.lack of intinsic factor (IF)
.pernicious anemia (antibody against – atrophic gastritis)
.malabsorption
.terminal ileum resection
-Treatment :
.parenteral B12
**Hemolytic Anemia
-Is a form of anemia due to hemolysis, the abnormal breakdown of red blood cells (RBCs), either in the blood vessels
(intravascular hemolysis) or elsewhere in the human body (extravascular)
-Hereditary (inherited) hemolytic anemia can be due to :
.defects of red blood cell membrane production (as in hereditary spherocytosis and hereditary elliptocytosis)
.defects in hemoglobin production (as in thalassemia, sickle-cell disease and congenital dyserythropoietic anemia)
.defective red cell metabolism (as in glucose-6-phosphate dehydrogenase deficiency and pyruvate kinase deficiency)
-Symptoms :
.shortness of breath
.jaundice (increased excretion of bilirubin into the biliary tract)
.increased the risk of particular long-term complications, such as (gallstones and pulmonary hypertension)
.pallor, fatigue, shortness of breath and potential for heart failure
.failure to thrive
*Hereditary spherocytosis and elliptocytosis

-Autosomal dominant / Spectrin deficiency (causes hemolysis in spleen)
+All hemolytic anemia is risk for Gallstones (pigmented Ca+2 bilirubinnate)
-Symptoms :
.anemia and hyperbilirubinemia in newborn (indirect)
.hypersplenism (splenomegaly)
.gall stones (pigmented)
-Labs :
.elevated reticulocyte
.elevated bilirubin (indirect)
.Hb 6-10
.MCV normal
-Diagnosis :
.osmotic fragility test
-Treatment :
.folic acid
.blood transfusion
.splenectomy at 5-6yrs
93
*Glucose-6-phosphate dehydrogenase deficiency G6PD / Favism

-X-linked (mainly affect male) / G6PD (hemolysis is intravascular hemolysis)
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-Two types :
.episodic hemolytic anemia M.C
.chronic hemolytic anemia
-Symptoms :
.start 1-2days after ingestion of oxidant (ASA, sulfa-drugs, antimalaria, fava beans or infection)
.rapid drop in Hb and jaundice
-Diagnosis :
.genetic measurement of G6PD
-Treatment :
.supportive folic acid
.blood transfusion
.NO splenectomy
+G6PD (don`t cause splenomegaly)
**Hemoglobin Disorders
*Sickle-cell disease
-Autosomal recessive / Mutation of B-globin gene / More in black african, protect from malaria
-Newborn (NO symptoms) / Symptoms start 2-4months / 1st symptoms is Hand-Foot syndrome (acute dactylitis)
+Hand-Foot syndrome (also called palmar-plantar erythrodysesthesia, is a side effect of some types of chemotherapy. Hand-
foot syndrome causes redness, swelling, and pain on the palms of the hands and/or the soles of the feet)
-Symptoms :
.skin ulcer
.retinopathy
.avascular necrosis of femur
.autosplenectomy (infection by uncapsulated bacteria – give vaccine pneumococcus)
.priapism
.strock
.aplastic crises with paravovirus B19
-Complications :
.increased risk of gallstones (pigmented)
.renal failure
.salmonella osteomyelitis
-Labs :
.elevated reticulocyte
.normal MCV
.ESR almost zero
-Diagnosis :
.Hb electrophoresis/Hbss (BEST)
-Treatment :
.vaccination (pneumococcus)
.penicillin prophylaxis
.folic acid
.pain control
.blood transfusion
.hydroxyurea
.bone marrow transplant (BEST)
*Thalassemia
-Both alpha and beta thalassemias are often inherited in an autosomal recessive manner
+Alpha-thalassemia
.alpha thalassemia (deletion of 2 genes)
.mild hypochromic, microcytic anemia without clinical problems
.often diagnosed as iron deficiency anemia; need molecular analysis for diagnosis
.alpha thalassemia typically results from deletions involving the HBA1 and HBA2 genes (2 genes)
94
.it is also connected to the deletion of the 16p chromosome

.HgH H disease (deletion of 3 genes – Hgb Barts)
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.typically do not require transfusions or splenocetomy

.alpha thalassemia majot (deletion of 4 genes – severe fettal anemia resulting in hydrops fetalis)
+Beta-thalassemia
.mutations in the HBB gene cause beta thalassemia. The HBB gene provides instructions for making a protein called beta-globin.
Beta-globin is a component (subunit) of hemoglobin
.beta thalassemia (β thalassemia) is a form of thalassemia caused by mutations in the HBB gene on chromosome 11, inherited
in an autosomal recessive fashion
.excess alpha globin chains (increase in HbF) (no problem with gamma-chain production)
.presents in 2nd month of life with progressive anemia, hypersplenism and cardiac decompensation (Hb <4mg/dL)
.expanded medullary space with increased expansion of face and skull (hair-on-end); extramedullary hematopoiesis,
hepatosplenomegaly
-Labs :
.infants born with HbF only (seen on Hgb electrophorosis)
.severe anemia, low reticulocytes, increased nucleated RBCs, hyperbilirubinemia microcytosis
.no normal cells seen on smear
.bone marrow hyperplasia; iron accumulates lead to increased serum ferritin and transferrin saturation
-Treatment :
.transfusion
.deferoxamine
.may need splenectomy
.bone marrow transplant curative
**Bleeding Disorder
-Minor bleeds (vWD)
-Deep bleeds (hemophilia)
-Thtombocytopenia is the M.C acquired cause of bleeding in children
*Hemophilia A (VIII) and B (IX)

-M.C is (A)
-Hemophilia A (factor VIII deficiency)
-Hemophilia B (factor IX deficiency) (christmas disease)
-X-linked (mainly affect males)
-Symptoms :
.slowing of rate of clot formation (prolonged bleeding after circumcision 1 st symptoms)
.easy bruising (with crawling and walking)
.hemarthroses (M.C) (in ankles; in older child, knee and elbows)
.large volume blood loss into iliopsoas muscle (inability to extend hip) – Vague groin pain and hypovolemic shock
.life-threating bleeding (intracranial GI bleeding)
-Labs :
.*(2-3) increase in PTT (all other normal – PT, INR, platelet, vWF)
-Treatment :
.to mild cases - DDAVP (desmopressin) (increases factor VIII levels in mild cases)
.to severe cases – (intravenous via a central line every 2-3 days)
.avoid antiplatelet and aspirin medications
*von Willbrand Disease (vWD)

-M.C hereditary bleeding disorder; autosomal dominant, but more females affected
-Symptoms :
.mucocutaneous bleeding (excessive bruising, epistaxis, menorrhagia, postoperative bleeding)
-Labs :
.increased bleeding time and PTT
-Diagnosis :
.vWFAg, vWF (ristocetin activity)
-Treatment (NEED to increase the level of vWF and factor VIII) :
95
.DDAVP (desmopressin) (for type 1)

.plasma derived vWF-containing concentrates with factor VIII (for types 2 or 3)
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*Vitamin K deficiency
-Newborn routinly given IM Vit K
-Risk Factors :
.lack of oral intake
.malabsorption (chronic pancreatitis, obstructive jaundice)
.alteration in gut flora (long-term antibiotic use)
+Vit K is fat soluble so deficiency associated with a decreased in factors (II, VII, IX, X) 1972 (proteins C and S)
+Increased PT and PTT with normal platelet count and bleeding time
**Platelet Disorders
*Immune (idiopathic) Thrombocytopenic Purpura (ITP)
-Autoantibodies against platelet surface
+M.C indication for splenectomy
-Symptoms :
.most in 1-4yrs of age – Usually after a nonspecific viral infection
.sudden onset of petechiae and purpura with or without mucous membrane bleeding (most resolve within 6 months / <1% with
intracranial hemorrhage / 10-20% develop chronic ITP)
+With ITP, the physical examination is otherwise normal; hepatosplenomegaly and lymphadenopathy should suggest another
disease
-Labs :
.platelet <20.000/mm3
.platelet size normal to increased
.bone marrow (normal to increased megakaryocytes)
.other cell lines normal
-Treatment :
.transfusion contraindicated unless life-threatening bleeding (platelet antibodies will bind to transfused platelets as well)
.NO specific treatment if platelets >20.000 an no ongoing bleeding
.if very low platelets, ongoing bleeding that is difficult to stop or life-threatening
.intravenous immunoglobulin for 1-2 days
.splenectomy reserved for older child with severe disease
+Nutritional neglect (is the M.C.C of failure to thrive)

+Risk Factors For Child Abuse :
.caregiver
.young parents age
.close pregnancy interval
.substance abuse
.spousal abuse
.single parent
.low social class
+Findings That Suggest Child Abuse :
.bruises (M.C)
.fractures (spiral fracture – twisting or complex fracture)
.burns (cigarette burn – on hand and foot)
.head trauma (M.C.C of death in abuse) (subdural hematoma / retinal hemorrhage – shaking baby syndrome)
**Disorder Of Eye and Ear

-Infant see black and white better / Full visual full acuity by 5-6yrs
-Causes of intraocular lens opacities :
.premature
.inherited
.TORCH infection (especiall Rubella)
96
.galactosemia
.chromosomal anomelies
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.drugs (steroid)
.trauma
-Causes of ectopia lentis :
.trauma (M.C.C)
.uveitis, tumor, glucoma
.marfan syndrome (superior)
.homocystinuria (inferior)
.ehler danlos syndrome
*Neonatal Conjunctivitis (ophthalmia neonatorum)
-Redness and discharge, from eye from eye in neonate
-Causes :
.chemical conjunctivitis (M.C.C in 1st day of life) (from silver nitrate or erythromycin)
.Neisseria gonorrhoeae (2-5days) (complication - corneal ulceration) (treated by ceftriaxon)
.Chlamydia trachomatis (5-14days) (complication – pneumonia) (treated by erythromycin)
-Causes Of Red Eye :
.bacterial conjunctivitis (unilateral or bilateral with exudate)
.viral conjunctivitis (usually bilateral watery discharge)
.allergy
.chemicals
.uveitis
.foreign body
*Orbital Cellulitis
-Infection of orbital tissue
-Physical Exam :
.ophthalmoplegia (can`t move his eye)
.chemosis (swelling)
.inflammation
.fever + leukocytosis
.wound or sinusitis by encapsulated bacteria
-Diagnosis :
.orbital CT-scan with contrast (BEST)
-Treatment :
.I.V antibiotics
*Retinoblastoma
-M.C primary malignant tumor of eye (intra-ocular)
-Symptoms :
.rarely discovered at birth
.1-2yrs appear as white mass (leukocoria) (M.C symptom)
.strabismus (2nd M.C symptom)
-Diagnosis :
.CT-scan (NOT biopsy)
-Treatment :
.enucleation + chemo-radiotherapy
*Choanal atresia
-Unilateral (mey be asymptomatic and 50% associated with other anomalies – CHARGE) or Bilateral (cyanosis and become pink
with crying)
-Diagnosis :
.inability to pass catheter (3-4cm into nasopharynx)
97
.rhinoscopy
-Treatment :
.surgery
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*Nasal Polyp
-Benign tumor of nasal mucosa
+M.C.C is cystic fibrosis
+May have (aspirin sensitivity – asthma)
+Aspirin is contraindication
-Can casue nasal obstruction
-Treatment :
.steroid
.surgery
+M.C.C of abdominal mass in neonate (hydronephrosis) / in children <1yr neuroblastoma / in children >1yr wilms tumor
+M.C tumor in children (ALL)
+2nd M.C tumor in children (Brain tumor)
+M.C brain tumor in children (Benign astrocytoma)
nd
+2 M.C tumor in children in children (Medulloblastoma)
+M.C brain tumor in adult (malignant astrocytoma GBM)
+M.C 3rd ventricle tumor (ependymoma)
+M.C Bone tumor in children (osteosarcoma)
+M.C Bone tumor in adult (Multiple myeloma)

98Page

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PEDIATRICS

By Dr.Mohammad Z. abu sheikha@

5.Newborn screening test

+Comparison Of Two Newborn Screening Disease

+Phenylketonuria (PKU) +Classic Galactosemia

-Presentation .mental ret., vomiting, growth .jaundice (direct), hepatomegaly,

+Galactose-1-phosphate uridyl transferase deficiency (elevated galactose 1-p. – renal/liver/brain injury)

**Gestational Age And Size At Birth

*Large For Gestational Age (LGA) – fetal macrosomia

*Small For Gestational Age (SGA)

*Infants Of Diabetic Mother IODM

*Meconium Aspiration Syndrome (MAS)

.air way obstruction

**Gastrointestinal And Hepatobiliary Disorders

+Breast feeding jaundice (treated by feeding)

**Crigler–Najjar syndrome (CNS) - Indirect

**Transplacental Intrauterine Infections TORCH

.CMV culture positive from urine, stool, respiratory tract secretions

.neurologic evaluation with head CT and brain MRI is recommended

*Herpes Simplex Virus HSV

.<20wks gestation – varicella embryopathy (1-2%)

*Human Immunodeficiency Virus Infection HIV

+Maternal Disease – effect on baby

By Dr.Mohammad Z. abu sheikha@

-Caused by mutations in genes on the X chromosome / Very rare

*Trisomy 18 (Edwards Syndrome)

.many spontaneous abortion

*Trisomy 13 (patau Syndrome)

*Turner Syndrome (XO)

*Fragile Syndrome (X) in boys - (big)

**EARLY OVERGROWTH WITH ASSOCIATED DEFECTS

**CONNECTIVE TISSUE DISORDERS

By Dr.Mohammad Z. abu sheikha@

*Human Milk *Cow Milk

6.minerals .iron better absorbed .low iron and copper

8.digestibility .faster .after 45days

+A child can usually roll over by age (16 weeks)

+(1st 4 months - 750gr/per month)

+(3rd 4 months - 250gr/per month)

**Factors Affecting Physical Growth

By Dr.Mohammad Z. abu sheikha@

.painful defecation, fissures

**ANOTHER DISEASE OF BEHAVIORAL DISORDERS

**ADHD (attension-deficit hyperactivity disorder)

By Dr.Mohammad Z. abu sheikha@

.OPV is best stored at 2-8° C

+Recommended Immunization Schadule (Jordan Vaccination Schedule) :

By Dr.Mohammad Z. abu sheikha@

**CONGENITAL ANOMALIES OF THE LARYNX

*Congenital Subglottic Stenosis

*Vocal Cord Paralysis

*Airway Foreign Body

**INFLAMMATORY DISORDERS OF THE SMALL AIRWAYS

.congenital or acquired immunodeficiencies

.chronic metabolic alkalosis

.salty taste of skin

**Acute Otitis Media AOM

-Signs & Symptoms :

**Insect Venom Allergy

Human Milk Cow Milk