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    Hemolytic Uremic Syndrome 8.13.18

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    Emily Eresuma
    A 4-year-old female presented with bloody diarrhea and was initially diagnosed with E. coli infectious colitis but later developed worsening anemia, thrombocytopenia, and acute kidney injury, leading to a diagnosis of hemolytic uremic syndrome, a potentially life-threatening condition caused by bacterial infection leading to microangiopathy and organ damage. She was treated supportively with fluid resuscitation and monitoring for complications while her symptoms resolved over the next few weeks.

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    Hemolytic Uremic Syndrome 8.13.18

    Uploaded by

    Emily Eresuma
    461 views34 pages
    A 4-year-old female presented with bloody diarrhea and was initially diagnosed with E. coli infectious colitis but later developed worsening anemia, thrombocytopenia, and acute kidney injury, leading to a diagnosis of hemolytic uremic syndrome, a potentially life-threatening condition caused by bacterial infection leading to microangiopathy and organ damage. She was treated supportively with fluid resuscitation and monitoring for complications while her symptoms resolved over the next few weeks.

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    Pediatric Morning Report

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    A 4-year-old female presented with bloody diarrhea and was initially diagnosed with E. coli infectious colitis but later developed worsening anemia, thrombocytopenia, and acute kidney injury, leading to a diagnosis of hemolytic uremic syndrome, a potentially life-threatening condition caused by bacterial infection leading to microangiopathy and organ damage. She was treated supportively with fluid resuscitation and monitoring for complications while her symptoms resolved over the next few weeks.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    461 views34 pages

    Hemolytic Uremic Syndrome 8.13.18

    Uploaded by

    Emily Eresuma
    A 4-year-old female presented with bloody diarrhea and was initially diagnosed with E. coli infectious colitis but later developed worsening anemia, thrombocytopenia, and acute kidney injury, leading to a diagnosis of hemolytic uremic syndrome, a potentially life-threatening condition caused by bacterial infection leading to microangiopathy and organ damage. She was treated supportively with fluid resuscitation and monitoring for complications while her symptoms resolved over the next few weeks.

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    © All Rights Reserved
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    Download as pptx, pdf, or txt
    of 34

    Morning Report 8/13/18

    TED TANNER, MD MPH


    Chief Complaint: “Blood in stool”

     4-year-old female with 4 days of diarrhea, 1 day of


    tactile fever, and 1 day of bloody diarrhea
     Patient became suddenly ill 4 days ago
     Diarrhea accompanied by crampy abdominal pain,
    poor appetite, and 1 episode of non-bloody/non-
    bilious emesis
     Abdominal pain is generalized and intermittent
     Mom says the bloody diarrhea fills the toilet
     Seen at urgent care this morning, and referred to our
    ED for further evaluation
    History Continued

     History of constipation. She usually has one hard


    stool every other day for the last year. She does not
    take any medications for this.
     No known sick contacts
     No one home with similar symptoms
     Denies urinary symptoms, rash, weight loss, lethargy
    History Continued

     No significant past medical history other than


    constipation
     No medication
     No allergies
     UTD on immunizations
     No family history of IBD, or other chronic medical
    problems
     Lives with parents, 3 siblings, aunt, and 2 uncles
     Attends pre-school. Active child who likes to play outside
     No recent travel, no contact with open water sources
    Physical Exam

     T 36.6, HR 93, RR 20, BP 98/58, Wt 25.5, BMI 17.13


     Gen: alert, interactive, non-toxic
     HEENT: normal
     Resp: CTAB
     CV: RRR, no murmurs, normal cap refill
     GI: abdomen soft, mild periumbilical tenderness, non-
    distended, no organomegaly, negative heel-tap
     GU: approx 2mm anal fissure at 7 o’clock, 1 mm fissure at
    8 o’clock
     Skin: no rashes
     MSK: no joint swelling
     Neuro: normal mental status, CN intact, strength normal
    CC: “Blood in Stool”

    DDX.
    WORK-UP
    Differential Dx.
    Labs
    CC: “Blood in stool”

     E. Coli infectious colitis


     Anal fissure
     IV bolus x2 in the ED, drinking slushies, without
    complaint on re-examination
     Discharge home from ED with PCP follow-up, return
    precautions
    Returns to ED the next day

     Diarrhea has not improved


     Continues to have blood in stool
     DOI 5, now 2 days of frank, bloody stool
     Now having 20+ stools/day
     Poor oral intake, poor urine output
     Afebrile, HR 116, BP 107/75, wt 25.6
     Lips chapped, rest of exam the same
    Labs

     WBC 9.7 > 12.8  Na 139 > 139


     Hgb 14.1 > 15.2  K 3.8 > 3.9
     Hct 40.5 > 42.4  Cl 108 > 108
     Plts 244 > 200  CO2 19 > 16
     BUN 10 > 9
     Cr 0.47 > 0.57
    CC: Persistent bloody stool

     Dehydrated: received 20cc/kg NS bolus, drinking in


    the ED, perked up and was feeling well
     Dx: E. Coli infectious colitis, diarrhea
     DC home, PCP follow-up, return precautions
    She returns 2 days later!

     Diarrhea has actually improved, but still having


    abdominal pain
     No more blood in the stool
     24h of severe, crampy, periumbilical abdominal pain
     Poor appetite
     No fevers, no pain with coughing, no pain with
    walking, no emesis
    Afebrile
    HR 134
    BP 104/65
    (95th %tile 112/71)
    Wt. 25.3

    CC: abdominal pain


    Labs
    Labs
    Labs

    Plts: 244 > 200 > 32


    Hgb: 14.1 > 15.2 > 10.0
    Cr: 0.47 > 0.57 > 1.73
    • E. Coli colitis
    • Anemia
    Diagnosis? • Thrombocytopenia
    • Acute kidney failure
    • Petechiae
    Hemolytic Uremic Syndrome

     Classic triad of microangiopathic hemolytic anemia,


    thrombocytopenia, and acute kidney injury
     One of the main causes of acute kidney injury in
    children
     Shiga toxin-producing E. Coli HUS is the most
    common cause of pediatric HUS, 90% of cases
     2-3 per 100,000 children less than 5 years of age
     Primary HUS: diarrhea-negative
     Secondary HUS: diarrhea-positive, infection-induced
    Primary HUS

     Complement-mediated: mutations in genes for C3


    and CD46
     Coagulation pathway
     Diacylglycerol kinase epsilon (DGKE) mutation- complement
    activation with low C3 levels
     PLG mutation- plasminogen deficiency
     Thrombomodulin mutations – cofactor in Protein C pathway
     Methylmalonic Aciduria and Homocystinuria type C
     B12 deficiency
    Infection-Induced Secondary HUS

     Strep pneumoniae: 5% of all childhood HUS cases


     Infants and young children
     Incidence of HUS after pneumococcal disease is 0.5%
     70% present with pneumonia
     More severe initial with longer duration of oliguria and
    thrombocytopenia
     In two separate case series, 70-80% underwent dialysis
     10% progress to ESRD
     HIV infection
     H1N1 Infleunza A: strep pneumo common secondary
    infection
     Shigella: 15% mortality, 40% CKD. India, Bangladesh,
    Southern Africa
    Shiga toxin-producing E. coli HUS

    CONNECT TO
    UPTODATE FOR MORE INFORMATION
    STEC-HUS

     Microangiopathic hemolytic anemia: shearing of


    RBCs through platelet microthrombi
     Hgb <8, Negative Coombs’, Smear with schistocytes and
    helmet cells
    STEC-HUS

     Thrombocytopenia: platelet count < 140,000


     Usually no purpura or active bleeding
     Degree is unrelated to renal dysfunction
    STEC-HUS

     Acute kidney injury: renal involvement ranges from


    hematuria and proteinuria to severe AKI and
    oligoanuria
     Severe AKI in 50% of cases, but recovery is favorable even in
    those who require dialysis
    STEC-HUS other organ involvement

     CNS: seizures, coma, stroke, cortical blindness (20%


    of cases due to E. coli 0157:H7)
     GI: severe hemorrhagic colitis, bowel necrosis, rectal
    prolapse, peritonitis
     CV: cardiac dysfunction due to fluid overload,
    hypertension, hyperkalemia, thrombotic
    microangiopathy, myocarditis, tamponade
     Pancreas: glucose intolerance, transient DM
     Liver: hepatomegaly and transaminitis
     Hematology: leukocytosis, prognosis is worse with
    elevated WBCs
    Post-diarrhoeal
    HUS
    Shiga toxins (Stx) target
    Gb3-rich tubular
    epithelium and
    glomerular endothelial
    cells which leads to
    apoptosis, necrosis, and
    thrombotic
    microangiopathy.
    Evaluation and Diagnosis

     A patient with recent bloody diarrhea with signs of


    other organ involvement (oliguria, AMS, anemia,
    etc.) deserves evaluation for HUS
     CBC, renal function studies, GIPCR
     Diagnosis is clinical: symptoms + triad of lab
    findings. Renal bx not required
     Differential: DIC, TTP, other infections leading to
    volume depletion and subsequently renal injury
    Management

     Prevention: avoiding undercooked meats


     No benefit from antibiotics or antimotility agents

     Vigorous fluid resuscitation


     Use of IVF up to the day of HUS diagnosis associated with
    decreased use of RRT
     Mortality increased for dehydrated patients (OR 3.71)

     Closely monitor renal function and blood counts


    Antibiotic and Antimotility Agents

     Increase the risk of subsequent development of


    STEC-HUS
     Increased risk of HUS during bloody diarrhea phase
     Do not decrease duration of diarrheal symptoms
     One study found that the use of anticholinergic agents and
    narcotics increased risk of CNS dysfunction
    Supportive Therapy

     PRBCs for Hgb <6 or Hct <18, with goal of Hgb 8-9
     Platelet transfusion if bleeding or before procedure
     Fluid management
     Depletion from vomiting, diarrhea, poor intake
     Overload from oliguria or anuria
     Closely monitor for signs of fluid overload: HTN, cardiopulmonary
    overload.
     Ok to trial loop diuretics, but should these fail, move to CRRT

     Electrolyte management
     Hypertension: fix fluid status, CCBs, switch to ACEi
    once AKI resolves
    Indications for Dialysis

     Signs and symptoms of uremia (encephalitis, acidosis)


     Azotemia: BUN >/= 80-100 mg/dL
     Severe fluid overload (cardiopulmonary compromise)
     Severe electrolyte abnormalities (hyperkalemia)
     Need for nutritional support
    Specific Therapies

     Eculizumab, mab to C5 that blocks complement


    activation
     Used in patients with STEC-HUS with severe CNS involvement
    or other severe organ involvement
     Mostly used for complement mediated HUS
     Must be given early to see benefit
     No strong evidence to support antithrombotic agents
    or oral shiga toxin-binding agents
    Prognosis

     Hematologic manifestations resolve in 1-2 weeks,


    followed by kidney recovery
     Mortality rate <5%
     Acutely due to: CNS injury, coagulopathy, sepsis, heart failure
     Long-term: 60-70% recover completely from acute
    phase
     Renal outcome: hypertension, proteinuria, CKD
     Follow-up yearly for 5 years

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