A. Congenital Adrenal Hyperplasia

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1.

Hypogonadism is usually encountered in the following conditions, except

A. Congenital adrenal hyperplasia


B. Noonan Syndrome
C. Prader-Willi Syndrome
D. Bardet-Biedl Syndrome

2. A 6 year old girl with short stature, streak gonads and mild mental retardation
has a renal ultrasound for a first urinary tract infection.
A renal anomaly is seen during the examination. Which is the most likely diagnosis?

A. Unilateral renal agenesis


B. Multicystic kidney
C. Polycystic kidneys
D. Horseshoe kidney

3. Absolute indications for surgical exploration with a 14 year old child with renal trauma
are all, except

A. Expanding, pulsatile retroperitoneal haematoma with haemodynamic instability


B. Penetrating trauma with progressive hypotension and tachycardia
C. Coexisting large bowel injury with an extensive unilateral renal injury
D. Grade 4 renal injury clasified the American Association of Trauma Surgery

4. A baby boy presents with a right sided hydronephrosis and an AP diameter of 25mm.
MAG3 scan revealed 48% differential function. The next step is

A. IVP
B. watchful waiting
C. VCUG
D. pyeloplasty

5. In a 2 year old boy with unilateral cryptorchidism laparoscopy is performed revealing an


intrabdominal testicle approx. 4 cm above the internal inguinal ring. The best treatment option is

A. two stage Fowler-Stephens procedure


B. one stage Fowler-Stephens precedure
C. inguinal incision and scrotal fixation
D. orchiectomy
6. A 3 months old boy presents with a unilateral upper pole hydronephrosis
due to a ureterocele. MAG3 scan revealed good function of the upper pole but obstructed.
Deobstructing the system is best achieved with

A. ureterocele puncture
B. percutaneous nephrostomy
C. open resection and reimplantation
D. stenting

7. A 2 year old boy has a left testicular tumor. Serum Alfafetoprotein is elevated
and serum human chorionic gonadotrophin level is normal. Computerized tomography scan shows
no evidence of a metastatic disease.The tumor is most likely to be a

A. gonadoblastoma
B. yolk sac tumor
C. epidermoid cyst
D. teratoma

8. The main site of bicarbonate reabsorption is

A. proximal tubule
B. collecting duct
C. distal tubule
D. loops of Henle

9. A young adolescent was treated for a kidney stone. Evaluation revealed a medullary sponge
kidney. The most common finding will be

A. hyperoxaluria
B. hypercitraturia
C. hypercalcuria
D. hyperphosphataemia

10. The gene associated with Von Hippel-Lindau disease is located on chromosome

A. 3
B. 9
C. 11
D. 16
11. You are urgently called to the neonatology department because a child is born with
genital ambiguity. Prenatal karyotyping has shown a 46 XX. When examining the child you can
observe a severely virilized genital and no gonads palpable. What is your next step?

A. repeat karyotype
B. HCG stimulation test
C. serum electrolytes
D. hormonal screening

12. A 13 years old boy is seen because of intermittent terminal hematuria (spotting of
underwear)
Urine culture is negative and ultrasound of the urinary tract reveals no pathology.
What is the most probable diagnosis?

A. urethral stone
B. bulbar urethritis
C. infected syringocoele
D. balanitis

13. A colleague refers you a patient with a caecoureterocele. On investigation you would expect to
find:

A. a ureterocele that empties into the colon


B. a gapping open ureterocele
C. a ureterocele descending in the submucosa through the bladder neck
D. a ureterocele with no ureteric orifice

14. The probability of disorders of sexual development (DSD) in a boy


with penile hypospadias and palpable undescended testis is:

A. 10%
B. 30%
C. 50%
D. 70%

15. Prenatal diagnosis of congenital adrenal hyperplasia (CAH) in the at-risk fetus
is most specifically made by a measurement of amniotic fluid for:

A. 17-ketosteroids
B. 17-hydroxyprogesteron
C. 11-deoxycorticosteron
D. dehydroepiandrosteron
16. The X-ray above shows

A. posterior urethral valves


B. anterior urethral valves
C. syringocele
D. valve of Guerin

17. The incidence of reflux in offspring is

A. 10%
B. 30%
C. 66%
D. 90%

18. In a newborn with a severe form of autosomal recessive polycystic kidney disease,
what is the most common cause of neonatal death?

A. Renal insufficiency
B. Liver insufficiency
C. Portal hypertension
D. Respiratory insufficiency
19. What is the primary mechanism of alpha blockers in the treatment of
children´s bladder dysfunction?

A. detrusor relaxation
B. bladder neck relaxation
C. bladder neck contraction
D. pelvic floor relaxation

20. Which is the most common electrolyte disturbance in a


gastrocystoplasty?

A. Hypochloraemic metabolic alkalosis


B. Hyperchloraemic metabolic alkalosis
C. Hypochloraemic metabolic acidosis
D. Hyperchloraemic metabolic acidosis

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