1. A nine-and-one-half-pound, one-day-old girl has a bloody diaper. A large, firm, right flank mass is palpated. Ultrasound shows an enlarged right kidney and normal left kidney. Renal scan shows no function on the right. Laboratory evaluation is normal except for thrombocytopenia. The next step is I.V. hydration and:

A. antibiotic prophylaxis. B. selective renal artery perfusion with heparin. C. selective renal artery perfusion with urokinase. D. renal vein thrombectomy. E. nephrectomy. 2. In a patient with epispadias, the location of the penile neurovascular bundles relative to

the corporal bodies are:

A. located dorsally and laterally on the proximal corpora and laterally on the distal corpora.

B. located ventrally over the proximal corpora and laterally on the distal corpora. C. in the dorsal midline over the proximal and dorsal corpora. D. located dorsally over the proximal and ventral over the distal corpora. E. located ventrally on the proximal and distal corpora. 3. A 14-year-old boy has recurrent stones in a neobladder. The most frequently associated

metabolic abnormality is: A. metabolic alkalosis. B. low urine sodium. C. hypercalciuria. D. hyperphosphaturia. E. hypocitraturia. 4. A 13-year-old circumcised boy has nocturnal enuresis and a UTI. He cannot be

catheterized for a VCUG. A retrograde urethrogram confirms a 1.0 cm, 3-5 F in diameter, bulbar urethral stricture. The most reliable long-term result is obtained by:

A. flap urethroplasty. B. urethral dilation with steroid injection. C. direct visual urethrotomy. D. excision of stricture and end-to-end anastomosis. E. buccal graft urethroplasty. 5. A 14-year-old boy with myelomeningocele is dry during the day on CIC every four

hours. Evaluation after two recent UTIs reveals bilateral grade 2 VUR. Urodynamic studies reveal a total fill noncompliant bladder with a detrusor pressure of 40 cm H2O at a capacity of 220 ml, and end fill detrusor pressures with overflow incontinence of 60 cm H2O at a capacity of 260 ml. He is on oxybutynin 5 mg bid. The next step is:

A. add imipramine. B. increase oxybutynin. C. onabotulinumtoxinA. D. bilateral subtrigonal injections of Deflux®. E. enterocystoplasty. 6. An eight-year-old boy has testicular and penile enlargement and deepening of his voice.

Hormonal studies reveal a low serum cortisol. The most likely diagnosis is: A. pituitary tumor. B. Leydig cell tumor. C. adrenal adenoma. D. congenital adrenal hyperplasia. E. adrenocortical carcinoma. 7. A 13-year-old girl with spina bifida underwent a sigmoid augmentation cystoplasty two

years ago. She catheterizes herself five times per day and is continent. However, serum creatinine has increased from 1.1 to 1.6 mg/dl, and an ultrasound shows progressive bilateral hydroureteronephrosis. The most useful diagnostic test is:

A. spinal MRI scan. B. catheterization diary. C. VCUG. D. diuretic renogram. E. videourodynamics. 8. A one-year-old boy with severe hypospadias has a preputial island flap urethroplasty.

The principal blood supply to the preputial flap comes from which artery: A. external pudendal. B. internal pudendal. C. deep penile. D. dorsal penile. E. inferior epigastric. 9. A 16-year-old boy is evaluated for a small penis. His past history is unremarkable

except for the absence of the sense of smell. On examination, he is Tanner Stage I, his penis is 3.5 cm in length, and both testes are inguinal. His micropenis is best treated with:

A. dihydrotestosterone. B. bromocriptine. C. hCG. D. GnRH. E. testosterone.

10. A five-year-old boy has intermittent left flank pain and microhematuria. Evaluation reveals a 4 x 6 mm stone in a lower pole calyx with a pyelocalyceal angle 90 degrees. The best treatment is:

A. observation. B. extracorporeal shock wave lithotripsy. C. partial nephrectomy. D. ureteroscopic laser lithotripsy. E. percutaneous laser lithotripsy. 11. A four-year-old boy with cystinuria has flank pain. Non-contrast CT scan reveals a 1.8

cm non-obstructive stone at the ureteropelvic junction. Appropriate initial management is urinary alkalinization and:

A. PCNL. B. SWL. C. open pyelolithotomy. D. laparoscopic pyelolithotomy. E. ureteroscopic laser lithotripsy. 12. A 15-year-old girl with spina bifida undergoes an augmentation cystoplasty with 40 cm

of detubularized terminal ileum. Ten years later, the coagulation parameter most likely to be abnormal is:

A. prothrombin time. B. partial thromboplastin time. C. bleeding time. D. factor VIII level. E. platelet count. 13. A neonate has a deep sacral dimple. The next step is: A. observation. B. spinal MRI scan. C. videourodynamics. D. lumbosacral spine films. E. ultrasound of the spine. 14. A two-year-old boy with penoscrotal hypospadias and bilateral cryptorchidism

developed dialysis dependent renal failure, at which time a renal biopsy revealed diffuse mesangial sclerosis. He has a point mutation on chromosome 11p13. A CT scan obtained to evaluate new onset hematuria reveals a 3 cm solid mass in the lower pole of the right kidney. The next step is:

A. repeat ultrasound in three months. B. bilateral nephrectomy.

C. right partial nephrectomy. D. right radical nephrectomy. E. open renal biopsy. 15. A healthy ten-year-old girl underwent treatment seven years ago for a Stage III

favorable histology Wilms tumor with radiation, nephrectomy, and chemotherapy. As an adolescent, the most likely late effect of her therapy is:

A. renal insufficiency. B. second malignant neoplasm. C. cardiac failure. D. pulmonary fibrosis. E. ovarian failure. 16. A 14-month-old boy has an intratesticular mass. Ultrasound shows a combination of

calcified, solid, and cystic components. Tumor markers are normal. The next step is: A. scrotal exploration and testis biopsy. B. scrotal exploration and orchiectomy. C. inguinal exploration and testicular sparing tumor enucleation. D. inguinal exploration and radical orchiectomy. E. inguinal exploration and testis biopsy. 17. A 28-year-old woman has a twin pregnancy of 32 weeks gestation. Ultrasound shows

Twin A is normal and Twin B is a male fetus with progressive oligohydramnios, increasing bilateral hydroureteronephrosis, and bladder distention over the past three weeks. Twin B kidneys show normal echogenicity and no cysts. Twin B's bladder aspiration reveals sodium of 80 mmOl/l, chloride of 60 mmOl/l, and osmolality of 150 mOsm/l. The best management is:

A. observation and term delivery. B. amnio-amniotic window/shunt. C. vesico-amniotic shunt in Twin B. D. induce labor. E. lecithin sphingomyelin ratio of Twin A. 18. A five-year-old girl has recurrent UTI's associated with episodic urinary incontinence

and fecal soilage of her undergarments. A bladder ultrasound shows a thick-walled bladder and a calculus. A VCUG is shown. The diagnosis is:

A. dysfunctional elimination syndrome. B. spina bifida occulta. C. sacral agenesis. D. non-neurogenic, neurogenic bladder. E. anterior meningocele.

19. A six-year-old girl has urinary frequency, urge incontinence, and recurrent febrile UTIs. A VCUG shows bilateral grade 2 VUR, mild bladder trabeculation, and spina bifida occulta. She has a history of constipation and a large amount of fecal material seen on the scout film. Her physical and neurologic examinations are normal. The next steps are prophylactic antibiotics, bowel management, and:

A. timed voiding and maintenance of a voiding and bowel calendar. B. antimuscarinic medication. C. urodynamic evaluation. D. an MRI scan of the spine. E. endoscopic treatment of reflux. 20. A 30-year-old woman with ureterosigmoidostomy has done well until recently when she

developed right flank pain and fever responding to antibiotic therapy. Ultrasound reveals right ureterohydronephrosis down to the sigmoid. CT scan confirms a functional right kidney with hydroureteronephrosis to the level of the ureteroenteric anastomosis, no filling defects nor stones are noted. Sigmoidoscopy is normal. MAG-3 Lasix-induced renogram, reveals 35% right renal function with T1/2 time of infinity. Appropriate surgical intervention should include:

A. colon conduit urinary diversion. B. continent urinary diversion (Indiana pouch). C. right to left transureteroureterostomy. D. excision of ureterosigmoid anastomotic site with sigmoid cuff. E. revise ureteroenteric anastomosis. 21. A neonatal boy with bronchopulmonary dysplasia is being treated with furosemide. He

develops hematuria, and an ultrasound shows nephrocalcinosis. The calcium creatinine ratio was 0.38. The next step is:

A. alkalinization of the urine. B. acidification of the urine. C. add thiazide diuretic. D. dietary calcium restriction. E. discontinue furosemide. 22. A four-year-old boy has a 0.8 x 1.2 cm right upper pole calculus in a 2 x 2 cm posterior

calyceal diverticulum. The best management is: A. SWL. B. PCNL and ablation of the diverticulum.

C. ureteroscopic laser fragmentation of stone, stone extraction, and ablation of the diverticulum.

D. laparoscopic nephrolithotomy and diverticulotomy. E. open nephrostolithotomy and calyceal diverticulectomy.

23. A four-year-old boy is undergoing treatment of Type I, distal RTA with potassium citrate supplementation. The best test to assess the therapeutic efficacy is:

A. serum bicarbonate. B. serum chloride. C. urine calcium. D. urine citrate. E. urine pH. 24. A 13-year-old boy undergoes a radical orchiectomy for an embryonal paratesticular

rhabdomyosarcoma. Surgical margins are free of tumor. A CT scan of the chest, abdomen, and pelvis is normal. The next step is:

A. vincristine, dactinomycin, and cyclophosphamide. B. bilateral RPLND. C. ipsilateral RPLND. D. radiation to retroperitoneal nodes. E. repeat CT scan in three months. 25. An eight-year-old boy develops abdominal pain three hours after a high speed MVC. His

blood pressure is stable and hemoglobin and creatinine are normal. Urinalysis shows 5-10 RBC/hpf and no fractures are seen on abdominal and pelvic x-rays. A spiral CT scan shows good bilateral renal perfusion with a normal left renal pelvis, ureter, and bladder. There is a 1 cm lateral perinephric hematoma by the lower pole of the right kidney, opacification of the right renal pelvis is delayed and the right ureter is not visualized. The next step is:

A. observation. B. cystogram. C. MRI scan. D. delayed CT images. E. retrograde ureteropyelogram. 26. A newborn boy does not void for 48 hours after birth. A renal and bladder ultrasound

reveals normal kidneys and a partially distended bladder. Spinal ultrasound shows that the conus medullaris terminates at the L2-L3 vertebral level. The next step is:

A. observation. B. CIC. C. urodynamic evaluation. D. VCUG. E. MRI scan of the lumbosacral spine. 27. A one-year-old phenotypic boy has bilateral nonpalpable testes. Serum testosterone at

birth was within normal limits and chromosomal analysis is 46 XY. At laparoscopy, a rudimentary uterus and salpinx are found associated with normal appearing testes.

Testicular biopsies reveal normal preadolescent testicular architecture. The most likely diagnosis is:

A. mixed gonadal dysgenesis. B. ovotesticular disorder. C. persistent Mullerian duct syndrome. D. androgen insensitivity syndrome. E. pure gonadal dysgenesis. 28. A three-month-old boy with a UTI has a partially duplicated left collecting system with

lower pole hydronephrosis. VCUG shows no reflux. MAG-3 scan shows 15% of total renal function is attributed to the left lower pole with a T ½ of > 40 minutes. The next step is:

A. antibiotics and ultrasound in three months. B. percutaneous nephrostomy and repeat MAG-3. C. left lower pole cutaneous pyelostomy. D. left lower to upper pyeloureterostomy. E. left lower pole partial nephrectomy. 29. A six-year-old boy with previously treated posterior urethral valves has a poorly

compliant, unstable bladder on urodynamic testing. In his early teen years, bladder urodynamics are expected to show:

A. no change. B. decreased compliance. C. increased compliance. D. increased detrusor instability. E. normal study. 30. At six weeks of fetal development, there is rupture of the cloacal membrane prior to

complete advancement of the urorectal septum and infolding of the lateral mesodermal folds. At term, this neonate will have:

A. epispadias. B. classical bladder exstrophy. C. cloacal exstrophy. D. common urogenital sinus. E. common cloaca. 31. In the female fetus bilateral failure of ureters to separate from the common excretory

ducts during embryologic development will result in: A. bilateral renal ectopy. B. ureteral duplication. C. bladder neck incompetence.

D. bilateral ureteroceles. E. laterally positioned, refluxing orifices. 32. A nine-year-old girl with developmental delay and seizures has acute right flank pain.

CT scan shows a large mixed density renal mass with retroperitoneal hematoma. She is at increased risk for:

A. hepatic fibrosis. B. chronic renal failure. C. renal cell carcinoma. D. thyroid cancer. E. cerebellar hemangioma. 33. A one-year-old girl has a febrile UTI. Ultrasound reveals a solitary right kidney and an

enlarged bladder with absence of the uterus. On physical examination, the external female genitalia appears normal; however, no vaginal opening is identified. The most likely associated anomaly is:

A. aniridia. B. coarctation of the aorta. C. spinal anomaly. D. inguinal hernia. E. malrotation of the bowel. 34. A 12-year-old, complete C4 quadriplegic girl is managed with diaper dependent voiding.

She is hospitalized for treatment of recurrent decubitus ulcers. Renal ultrasound reveals bilateral pelviectasis. Urodynamics demonstrate a total fill non-compliant bladder with end-fill detrusor pressures and detrusor LPP of 50 cm H2O at 100 cc capacity (overflow incontinence) with external sphincter DSD. Management of the urinary tract should be changed to:

A. CIC. B. ileocystoplasty. C. ileal conduit. D. appendicovesicostomy. E. suprapubic tube. 35. A 16-year-old boy with a history of PUV and stage 4 renal failure, develops a UTI. An

effective antibiotic that can be given at its normal dose and interval is: A. ceftazidime. B. ciprofloxacin. C. sulfamethoxazole - trimethoprim. D. ceftriaxone. E. nitrofurantoin.

36. A newborn infant is referred for evaluation of ambiguous genitalia. Physical examination reveals septated genital folds, a solitary perineal opening, and no palpable gonads. FISH for SRY is negative. A pelvic ultrasound is shown. The diagnosis is:

A. 21-hydroxylase deficiency. B. 11-beta-hydroxylase deficiency. C. 3-beta-dehyrogenase deficiency. D. Mayer-Rokitansky syndrome. E. persistent cloaca.

37. A three-month-old infant boy has an abdominal mass and multiple subcutaneous

nodules. CT scan shows a 5 cm localized right suprarenal mass and liver metastasis. Biopsies of the bone marrow and mass show small round blue cells. N-Myc is not amplified, and DNA flow analysis reveals an aneuploid pattern. Bone scan and chest CT scan are negative. The next step is:

A. observation. B. biopsy of skin nodule. C. resection of the primary tumor. D. six weeks of vincristine, actinomycin D, cyclophosphamide. E. resection of primary tumor followed by multimodal chemotherapy. 38. A six-month-old boy has left grade 4 hydronephrosis. Nuclear renography indicates 25%

function of the left kidney and a T1/2 of 35 minutes. Two months after a left dismembered pyeloplasty, an ultrasound shows persistent grade 4 hydronephrosis.

Repeat nuclear renography demonstrates 23% function in the left kidney and a T1/2 of 30 minutes. The next step is:

A. repeat renal sonogram in three months. B. Whitaker test. C. double-J stent. D. endoscopic incision of the ureteropelvic junction. E. repeat pyeloplasty. 39. A seven-year-old boy has had testicular pain and acute scrotal swelling for the past 48

hours. Examination reveals diffuse scrotal tenderness with swelling and erythema. Cutaneous examination reveals purpura. Urinalysis is negative for proteinuria with 5-10 RBC/hpf, 0-1 WBC/hpf. Doppler scrotal ultrasound reveals areas of marked testicular hyperemia with spotty areas of poor blood flow bilaterally. The best treatment of his scrotal pain is:

A. antibiotics. B. platelet transfusion. C. scrotal exploration. D. steroids. E. nonsteroidal anti-inflammatory drugs. 40. A 15-month-old girl with congenital adrenal hyperplasia has a UTI. The VCUG is

normal, but the renal/pelvic ultrasound reveal a 1 cm solid left ovarian masses. The next step is:

A. MRI scan of the abdomen and pelvis. B. increase glucocorticoids. C. percutaneous biopsy. D. excisional resection. E. left oophorectomy. 41. At 20 weeks gestation, amniocentesis demonstrates a 45 X/46 XY karyotype. The most

likely clinical presentation for this child will be: A. normal male phenotype. B. hypospadias with unilateral undescended testis. C. bilateral undescended testes. D. normal female phenotype. E. clitoral hypertrophy and nonpalpable gonads. 42. An eight-year-old boy with spina bifida has undergone augmentation cystoplasty. He is

asymptomatic and catheterizes every four hours. A renal bladder ultrasound shows normal kidneys, a large bladder, and a cystic abdominal mass. A noncontrast CT scan is shown. The next step is:

A. catheter drainage. B. CT cystogram with drainage film. C. percutaneous aspiration. D. diagnostic laparoscopy. E. neurosurgical evaluation.

43. A 12-month-old girl has spina bifida. She is on 1 mg of oxybutynin twice each day and CIC every four hours. She is completely continent day and night. The voiding diary reveals a total daily volume of 400 ml with first AM catheterization volume of 85 ml and average catheterization volume of 60 ml. Renal ultrasound is normal. Urodynamic testing is shown. There is no VUR. The next step is:

A. continue present management. B. increase frequency of CIC. C. increase antimuscarinic medication. D. onabotulinumtoxinA into the detrusor. E. urethral dilation.

44. The life threatening condition associated with the abnormality shown is: A. pulmonary insufficiency. B. biliary atresia. C. single ventricle. D. autosomal recessive polycystic kidney disease. E. posterior urethral valves.

45. A three-year-old, 12 kg boy has a history of PUV. His baseline serum creatinine is 0.6

mg/dl. He undergoes bilateral tapered ureteral reimplantations for Grade 5 VUR. Postoperatively, he receives D5 LR at 50 ml/hr. His urine output over the next 18 hours averages 8 ml/kg/hr. On the first post-operative day, his serum sodium is 165 mEq/l, creatinine is 1.0 mg/dl serum osmolality is 360 mOsm/kg, and urine osmolality is 228 mOsm/kg. The best treatment is:

A. DDAVP. B. furosemide. C. D5 1/4 NS at 25 ml/hr. D. D5 1/4 NS at 100 ml/hr. E. D5 1/2 NS at 50 ml/hr. 46. A five-year-old girl receives a living related renal transplant. Two months post-

transplant, her serum creatinine rises from 0.5 mg/dl to 2.0 mg/dl. Renal ultrasound reveals hydronephrosis of the renal transplant with a fluid collection adjacent to the bladder and transplant. VCUG is normal. The next step is:

A. double-J stent placement. B. percutaneous aspiration of the perivesical fluid collection. C. MAG-3 renal scan. D. percutaneous nephrostomy tube placement. E. laparoscopic marsupialization. 47. A 13-year-old girl with recalcitrant acute lymphocytic leukemia undergoes myeloablation

with cyclophosphamide and whole body radiation, followed by a bone marrow transplant. Acute hemorrhagic cystitis develops two weeks following the transplantation. This complication has most likely developed secondary to:

A. thrombocytopenia. B. acrolein. C. radiation cystitis. D. polyoma BKV virus. E. Epstein-Barr virus. 48. An eight-month-old infant with 46 XY/45 X karyotype has a penoscrotal hypospadias,

descended right testis, and non-palpable left testicle. Laparoscopy reveals a 3 cm left intraabdominal testicular mass. Orchectomy is performed with pathology revealing a gonadoblastoma. The next step is:

A. observation. B. serial AFP and hCG evaluations. C. annual testicular ultrasounds. D. right testicular biopsy at puberty. E. right orchiectomy.

49. A two-year-old girl undergoes an anterior pelvic exenteration and ileal conduit urinary

diversion for management of a pelvic rhabdomyosarcoma that had failed chemo and radiation therapy. On the fourth postoperative day, she complains of abdominal pain. The stoma is black, and there is pain to direct palpation over the stoma and incisional site. There is no evidence of rebound nor indirect tenderness. A pelvic drain has put out 30 ml over the past eight hour. Bilateral ureteral stents are functioning well. Creatinine from the drain fluid is 0.5 mg/dl. The next step is:

A. observation. B. loopogram. C. loop endoscopy. D. abdominal CT scan. E. bilateral retrograde pyelograms. 50. A newborn boy is referred for a bilateral hemiscrotal masses found on neonatal

examination. AFP level is 70 IU/ml are noted. Testicular ultrasound reveals bilateral echogenic masses adjacent to the spermatic cords located above the testes. KUB is shown. The next step is:

A. serum cortisol levels. B. serum catecholamines. C. genetic testing for n-myc. D. genetic testing for cystic fibrosis. E. excision of bilateral inguinal cord masses.

51. A two-year-old boy has acute abdominal and left flank pain. Evaluation reveals a 9 cm

Wilms' tumor with spontaneous rupture and active retroperitoneal hemorrhage, which is controlled with angiographic techniques. He subsequently undergoes left radical nephrectomy. Pathology reveals favorable histology, tumor confined to the kidney, and negative lymph nodes. The stage and treatment are:

A. Stage 1; chemotherapy. B. Stage 2; chemotherapy. C. Stage 2; chemotherapy and radiation therapy. D. Stage 3; chemotherapy. E. Stage 3; chemotherapy and radiation. 52. A three-month-old boy with spina bifida has a vesicostomy performed for

hydroureteronephrosis and grade 5 VUR. Despite antibiotic prophylactics, he has had two subsequent febrile UTIs. There is no difficulty catheterizing the vesicostomy with a 12 Fr catheter. Random bladder catheterizations reveal residual urines of 20-30 ml of urine. The next step is:

A. change antibiotic prophylaxis. B. CIC via vesicostomy. C. revision of vesicostomy. D. bilateral ureteral reimplant and closure vesicostomy. E. endoscopic correction of VUR. 53. The increase in renal blood flow over the first six months of life is due to: A. increase renal volume. B. increased intravascular volume. C. decreased free water excretion. D. increased angiotension I. E. decreased renal vascular resistance. 54. A one-month-old with trisomy 18 undergoes a screening ultrasound as shown. The next

step is: A. no further testing indicated. B. repeat ultrasound in six months. C. MAG-3 Lasix washout renal scan. D. CT-guided renal biopsy. E. VCUG.

55. The blood supply to the appendix arises from the: A. superior mesenteric artery. B. inferior mesenteric artery. C. common iliac artery. D. internal iliac artery. E. middle sacral artery. 56. A definitive indication to evaluate the contralateral groin in a one-year-old-boy with an

ipsilateral communicating hydrocele is: A. prematurity. B. patient age less than two years. C. refractory constipation. D. history of a resolved contralateral hydrocele. E. presence of a ventriculoperitoneal shunt. 57. During exposure for an extravesical ureteral reimplantation, the relationship of the ureter

to the obliterated umbilical artery and the bladder is:

A. the ureter is lateral to the obliterated umbilical artery and the bladder is posterior to the ureter.

B. the ureter is lateral to the obliterated umbilical artery and the bladder is medial to the ureter.

C. the ureter is anterior to the obliterated umbilical artery and the bladder is medial to the ureter.

D. the ureter is posterior to the obliterated umbilical artery and the bladder is lateral to the ureter.

E. the ureter is posteromedial to the obliterated umbilical artery and the bladder is medial to the ureter.

58. A five-year-old girl undergoes a left tapered ureteral reimplantation with a psoas hitch

for an obstructive megaureter. The next day, she has numbness of the left anterior upper thigh and middle and lower mons pubis. The most likely cause is an injury to the:

A. femoral nerve. B. obturator nerve. C. genitofemoral nerve. D. ilioinguinal nerve. E. sacral nerve. 59. During an orchidopexy for a unilateral undescended testis, the surgeon has ligated the

processus vaginalis, mobilized the hernia sac proximal to the internal inguinal ring, and performed a retroperitoneal dissection. Even with these surgical maneuvers, the testis will still not reach into the scrotum. The next step is:

A. division of the inferior epigastric vessels and transversalis fascia of the inguinal

canal. B. fixation of the testis to the pubic symphysis and second stage orchiopexy. C. clamping of the gonadal vessels and Doppler evaluation of the vasal artery.

D. ligation of the gonadal vessels, leave the testis in-situ, and do a planned second stage Fowler-Stevens procedure.

E. remove the testis.

60. A 46 XY infant with bilateral palpable gonads is evaluated as shown. The test most

useful in establishing the diagnosis is: A. testosterone to dihydrotestosterone ratio. B. Mullerian inhibitory factor levels. C. serum 17-hydroxyprogesterone. D. abdominal-pelvic ultrasound. E. VCUG.

61. An 11-year-old girl with spina bifida is on CIC after an ileal bladder augmentation one

year ago. She has developed bladder stones twice over the past seven months despite high-volume daily bladder irrigation with saline solution. CT scan without contrast reveals no upper tract calculi. The next step is:

A. oral antibiotic prophylaxis. B. Polycitra-K. C. gentamicin bladder instillation. D. acetylcysteine bladder instillation. E. 24-hour urine for metabolic analysis. 62. The efferent neural activities that characterize bladder emptying are: A. pudendal-quiescent, hypogastric-quiescent, pelvic-active. B. pudendal-quiescent, hypogastric-active, pelvic-active. C. pudendal-active, hypogastric-quiescent, pelvic-active. D. pudendal?active, hypogastric-active, pelvic-quiescent. E. pudendal-quiescent, hypogastric-active, pelvic-quiescent.

63. A two-year-old boy with a normal phallus has a left palpable undescended testicle and a

right nonpalpable testicle. He undergoes a planned bilateral orchiopexy. Intraoperatively, at the time of left gonadal mobilization, the right gonad also appears in the field as shown. The next step is:

A. bilateral gonad biopsy, replace gonads, and close the wound. B. excision of the salpinx, uterus and bilateral orchiopexy.

C. detach salpinx from uterus bilaterally, mobilize the salpinx with the inguinal cord and proceed with orchiopexy.

D. split the rudimentary uterus in the midline, mobilization of the Mullerian ducts, and bilateral orchiopexy.

E. bilateral gonadectomy, salpingectomy, and hysterectomy.

64. A ten-year-old boy with primary nocturnal enuresis has failed desmopressin (DDAVP)

therapy. Evaluation of his lower urinary tract is likely to reveal: A. reduced functional bladder capacity. B. low maximal urinary flow rate. C. decreased bladder compliance. D. prolonged EMG lag time. E. elevated postvoid residual. 65. A 13-year-old boy has a history of posterior urethral valves and a small left kidney. He is

being considered for enterocystoplasty due to continued incontinence despite age appropriate antimuscarinics. His serum creatinine is 1.0 mg/dl. The parameters most helpful in decision-making about this procedure are:

A. serum bicarbonate and chloride. B. serum chloride. C. serum potassium. D. serum bicarbonate and 24-hour urinary volume. E. serum chloride and 24-hour urinary volume. 66. A 12-year-old girl is undergoing a laparoscopic partial oophorectomy for a dermoid

tumor. At the end of the procedure, it is noted that the ureter has been clipped at the level of the iliac vessels. The clip is removed from the intact ureter, and there is no evidence of ureteral necrosis or urine leak. The next step is:

A. observation. B. cystoscopy and placement of a ureteral stent. C. ureteral reimplantation with a psoas hitch. D. resection and primary end-to-end anastomosis. E. transureteroureterostomy. 67. A 28-year-old woman has an ultrasound at 22 weeks of gestation demonstrating a male

fetus with bilateral hydroureteronephrosis, a distended bladder, and oligohydramnios. A bladder tap is done revealing the following chemistries: sodium 120 mEq/l (normal ?100 mEq/l), chloride 100 mEq/l (normal ? 90 mEq/l), osmolality 240 mOsmol/l (normal ? 210 mOsmol/l), calcium 8 mmOl/l (normal ?2 mmOl/l) and beta-2 microglobulin 100 mg/l (normal ? 2 mg/l). The next step is:

A. insertion of vesico-amniotic shunt. B. serial bladder decompression. C. fetoscopy and ablation of posterior urethral valves. D. repeat bladder aspiration for urine chemistries. E. recommend termination of pregnancy. 68. Metabolic acidosis is associated with: A. hypernatremia. B. hyperkalemia. C. decreased respiratory rate. D. decreased renal ammonia production. E. increased peripheral artery resistance. 69. An afebrile seven-year-old boy has lethargy and coffee-colored urine for one week. His

exam is unremarkable except for a 1.5 cm lesion on the back of his thigh that appears to be a healing carbuncle. Urinalysis shows 80 RBC/hpf, 6 WBC/hpf, and 3+ proteinuria, and his serum creatinine is 0.8 mg/dl. The most useful next test is:

A. serum compliment C3 levels. B. antistreptolysin-O titers C. anti-DNAase B titers.

D. renal ultrasound. E. renal biopsy.

70. A twelve-year-old girl with sickle cell disease has gross hematuria and left flank pain.

Urinalysis shows >100 RBC/hpf, 0-3 WBC/hpf. HCT is 23 and WBC is 11,000. An ultrasound shows moderate left hydronephrosis with a 8 mm soft tissue mass within the left renal pelvis. The next step is analgesics and:

A. oxygen, hydration, and urinary alkalinization. B. I.V. antibiotics. C. exchange transfusion. D. CT urogram.

E. cystoscopy, retrograde pyelogram, biopsy, and removal of mass and stent placement.

71. A three-year-old girl has a febrile UTI. Her medical history is positive for difficulty with

potty training. Physical examination reveals her perineum is continually wet. Renal and bladder ultrasound along with a VCUG are normal. The next step is:

A. antimuscarinics. B. uroflow-EMG. C. cystoscopy and examination under anesthesia. D. MR urogram. E. DMSA scan. 72. A two-month-old girl has bilateral grade 4/5 VUR. An appropriate screening option for

her two-year-old asymptomatic brother is: A. renal and bladder ultrasound. B. VCUG. C. nuclear cystogram. D. renal and bladder ultrasound and a VCUG. E. DMSA scan.

73. A 17-year-old boy has straining on urination and a palpable perineal mass. Ultrasound

of the perineum in the mid-pendulous urethral area is shown. A retrograde urethrogram is also shown. The likely origin of this abnormality is:

A. Cowper’s duct. B. gland of Littre C. prostatic utricle. D. urethral duplication. E. urethral diverticulum.

74. A seven-year-old boy is bucked from a horse and sustains a pelvic injury including a

pubic ramus fracture. A 10 Fr percutaneous suprapubic tube is placed at an outside hospital. He is transferred and a CT scan shows a moderately large pelvic hematoma. Cystogram is performed via the suprapubic tube and reveals prompt anterior extravasation near the bladder neck with contrast seen in the prostatic urethra. A retrograde urethrogram reveals the anterior and posterior urethra to be intact. The next step is:

A. convert to larger suprapubic tube. B. place urethral catheter. C. place a percutaneous perivesical drain. D. retropubic dissection and extravesical bladder repair. E. intravesical repair of bladder injury. 75. Autonomic dysreflexia is most likely to occur during bladder filling in a child with: A. an intracranial tumor. B. T6 cord injury. C. L2-L4 cord compression from tumor. D. lumbar sacral spina bifida. E. pelvic pheochromocytoma. 76. A 15-year-old boy is struck in the flank during a football game. He has persistent flank

pain for a week. Imaging obtained at that point is shown. Recommended follow-up is restriction from athletic events and:

A. urinalysis and blood pressure evaluation in three weeks.

B. urinalysis and blood pressure evaluation in six weeks. C. renal ultrasound, urinalysis, and blood pressure evaluation in three weeks. D. renal ultrasound, urinalysis, and blood pressure evaluation in six weeks E. CT scan, urinalysis, and blood pressure evaluation in six weeks.

77. The American Academy of Pediatrics suggests routine urinalysis in children at: A. no time. B. one year. C. one and two years of age.

D. one and five years of age. E. each annual visit until age five. 78. At the time of exploration for a non-palpable testis, the best guide for the next step is

the: A. patency of the processus vaginalis. B. status of the testicular vessels. C. vas deferens entering the internal inguinal ring. D. size of the contralateral testis. E. presence of ipsilateral Mullerian structures. 79. A six-month-old boy with a successfully incised posterior urethral valves has bilateral

severe hydroureteronephrosis and grade 4 VUR. He has had and two documented febrile UTI's over the past three months. He takes oxybutynin 1 mg TID, and sulfamethoxazole trimethoprim elixir 2.5 ml nightly. His serum creatinine has risen from a baseline of 1.0 mg/dl to 1.8 mg/dl. DMSA scan reveals 50/50 renal function with bilateral widespread photopenic areas. Attempts at CIC have been difficult for the family. The next step is:

A. change to a long-acting antimuscarinic. B. overnight bladder drainage. C. vesicostomy. D. bilateral subtrigonal injections of Deflux®. E. bilateral ureteral reimplantation. 80. A two-day-old, 34 week estimated gestational age infant boy is in the NICU for

management of respiratory distress. He has bilateral pneumothoraces and oliguria. A representative right renal ultrasound image is shown. The finding is secondary to:

A. barotrauma with subcapsular bleed. B. pyelovenous backflow. C. lymphatic leak. D. forniceal rupture. E. injury to renal pelvis.

81. A ten-year-old boy has neurogenic bladder secondary to lipomeningocele. He reports

new onset urinary incontinence over the last six months. He catheterizes five times per day, and is on 20 mg extended release oxybutynin daily. His neurogenic bowel is managed with daily washouts from his MACE stoma with excellent fecal control. Urinalysis shows no pyuria and urine culture is sterile. A renal ultrasound shows normal upper tracts. Urodynamics show worsening hypertonicity, increased detrusor overactivity, and decreased capacity with a detrusor LPP of 64 cm H2O at a volume of 160 ml. The next step is:

A. increase frequency of CIC. B. oral antibiotics and repeat urodynamics. C. obtain an MRI scan of the lumbosacral spine. D. detrusor injection with onabotulinumtoxinA. E. augmentation cystoplasty. 82. A 12-year-old boy has recurrent calcium oxalate stones. Serum electrolytes, calcium,

phosphorous, and creatinine are normal. A 24-hour urine collection shows a volume of 750 ml, a urine calcium of 1.1 mg/kg/day (normal < 4 mg/kg/day), and a urine oxalate of 1.0 mg/kg/day (normal < 0.5 mg/kg/day). In addition to increasing fluid intake to achieve 2000 ml daily urine output, the next step is:

A. check serum parathormone (PTH). B. urinary alkalinization. C. increase dietary calcium. D. start potassium citrate. E. start thiazide diuretic. 83. A renal ultrasound in a full-term one-day-old boy shows bilateral grade IV

hydroureteronephrosis and a distended bladder. Selective images from a cystogram are shown; voiding phase reveals normal urethra. A catheter is placed and a repeat

ultrasound 48 hours later demonstrates grade II/IV hydroureteronephrosis bilaterally. On DOL#5, serum creatinine is 1.7 mg/dl. The next step is antibiotic prophylaxis and:

A. CIC. B. spinal ultrasound. C. MAG-3 renal scan. D. cutaneous vesicostomy. E. bilateral cutaneous pyelostomy.

84. A six-day-old full-term boy with a prenatally identified solitary left hydronephrotic kidney

has poor feeding and irritability. Physical examination reveals a palpably enlarged left kidney. Urine output is 1.5 ml/kg/hr. His serum potassium is 5.4 mEq/l and creatinine is 1.7 mg/dl. An ultrasound and VCUG are shown. Antibiotic prophylaxis is started. The next day, his creatinine is 2.1 mg/dl. The next step is:

A. MAG-3 renal scan. B. percutaneous nephrostomy placement. C. cystoscopy with stent placement. D. dismembered pyeloplasty. E. left ureteral reimplantation.

85. A new drug is being tested for effectiveness in nocturnal enuresis. Two study

populations are being used, one with monosymptomatic nocturnal enuresis and the other with day and nighttime incontinence. The primary outcome is the number of dry nights per month which is normally distributed in both groups. The best method for statistical comparison of this study is:

A. Chi square test. B. t-test. C. Wilcoxson rank sum test. D. analysis of variants (ANOVA).

E. linear regression. 86. The etiology of the finding in the renal ultrasound image is: A. shock. B. chronic glomerulonephritis. C. Alport's syndrome. D. renal transplant rejection. E. distal renal tubular acidosis.

87. A 17-year-old boy has recurrent symptomatic calcium oxalate stones. Twenty-four hour

urine analysis demonstrates 900 ml volume, and hypercalciuria; all other urine chemical parameters are normal. Serum calcium, phosphorous, electrolytes, and creatinine are normal. The next step is increased fluid intake and:

A. lower calcium intake. B. increase calcium intake. C. potassium citrate. D. allopurinol. E. thiazide. 88. A one-year-old boy with a new diagnosis of Lesch-Nyhan syndrome has a 2 cm renal

pelvic stone. The most likely composition is: A. cystine. B. xanthine. C. uric acid. D. calcium oxalate. E. ammonium acid urate.

89. A five-year-old boy with cystinuria is managed with high fluid intake and alkalinization of urine with potassium citrate. He continues to have active stone formation. The next step is:

A. increase potassium citrate to maintain urine pH > 8.0. B. change to sodium citrate. C. alpha-mercaptopropionylglycine. D. D-penicillamine. E. captopril. 90. A 14 day-old 3 kg boy with a history of a high imperforate anus and rectourethral fistula

has undergone a diverting colostomy on the second day of life. He was re-admitted to the hospital on day ten of life with a febrile UTI. He defervesces on I.V. antibiotics. Renal ultrasound shows bilateral moderate hydroureteronephrosis. VCUG demonstrates bilateral grade 5 VUR and a large residual urine. Serum creatinine has increased from a value of 0.8 mg/dl at birth to 1.5 mg/dl. The next step is prophylactic antibiotics and:

A. CIC. B. vesicostomy. C. bilateral cutaneous pyelostomies. D. bilateral ureteral reimplantation. E. rectal pull through with ligation of urethrorectal fistula. 91. Acetohydroxamic acid: A. binds to cystine. B. chelates calcium. C. is a urease inhibitor. D. decreases urinary oxalate. E. is a beta lactamase inhibitor. 92. An eight-year-old girl with lumbosacral myelomeningocele has persistent urinary

incontinence despite treatment with oxybutynin XL 10 mg BID and CIC every four hours while awake. Renal ultrasound demonstrates bilateral pelviectasis. Videourodynamics show bilateral grade 2 VUR, coexisting with a total fill noncompliant bladder, with overflow incontinence established at end fill detrusor pressures of 60 cm H2O at a 150 ml. The next step is:

A. bilateral subtrigonal injection of bulking agent and rectus fascial sling.

B. bilateral subtrigonal injection of bulking agent, rectus fascial bladder neck sling and appendicovesicostomy.

C. bilateral subtrigonal injection of bulking agent and placement of artificial urinary sphincter.

D. augmentation cystoplasty. E. bilateral ureteral reimplantation and augmentation cystoplasty.

93. A fourteen-year-old boy is on CIC for a T-2 spinal injury sustained three months ago.

Video UDS show an underactive bladder. Physical examination findings reveal absence of a bulbospongiosus reflex. The next step is:

A. continue CIC. B. capsaicin treatment. C. onabotulinumtoxinA toxin injection to external sphincter. D. sacral neuromodulation. E. creation of a catheterizable channel. 94. A 14-year-old girl has a four-week history of intermittent abdominal pain associated with

urinary urgency, frequency, dysuria, and hematuria. Initial urine culture, was positive for E.coli and treated with culture appropriate antibiotics for two weeks. Her symptoms persist. The current urinalysis shows >100 WBC/hpf with 3-5 RBC/hpf, the urine culture is sterile. A renal bladder ultrasound shows posterior bladder wall thickening, with adjacent loops of small bowel having a 5 mm bowel wall thickness. The next step is:

A. urine PCR for adeno and BK virus. B. urine culture for yeast. C. GI consultation. D. cystoscopy with bladder biopsy. E. laparoscopic exploration and possible appendectomy. 95. An eight-year-old girl with a history of celiac disease has a one-week history of dysuria,

urinary frequency, and lower abdominal discomfort. Urinalysis reveals 5-10 WBC/hpf, 3-5 RBC/hpf. Urine culture is without growth. Ultrasound shows diffuse bladder wall thickening, and a CBC shows peripheral eosinophilia. The next step is:

A. oxybutynin. B. antifungals. C. antihelmintics. D. antihistamines. E. steroids. 96. Urodynamic studies performed in prepubertal patients with cerebral palsy with urinary

incontinence and inability to potty train will usually demonstrate: A. an underactive bladder with overflow incontinence. B. detrusor overactivity with a coordinated sphincter.

C. detrusor overactivity with sphincter dyssynergy with incomplete bladder emptying.

D. a non-compliant poorly contractile bladder with overflow incontinence. E. primary bladder neck dysfunction with overflow incontinence. 97. Removal of the distal ileum for augmentation cystoplasty will possibly result in:

A. platelet dysfunction. B. Vitamin B12 deficiency. C. iron-deficiency anemia. D. hypochloremic metabolic alkalosis. E. hyponatremic metabolic acidosis.

98. The results of a new blood test for anorchia are shown in the table. The positive

predictive value of this test in determining anorchia is: A. .25. B. .50. C. .75. D. .80. E. 1.0.

99. A four-year-old girl who is six months status post bilateral ureteral reimplantation for

Grade 4 VUR has two post-operative febrile UTIs. Ultrasound of both kidneys are shown and VCUG shows no reflux. A post-diuretic MAG-3 renogram image is shown. The next step is:

A. repeat MAG-3 diuretic renogram with bladder catheter. B. repeat VCUG. C. uroflow with EMG. D. MR urogram. E. timed voiding schedule.

100. A six-year-old boy has a febrile UTI and intermittent dysuria. Ultrasound is normal and

images from the VCUG are shown. The next step is: A. endoscopic correction of VUR. B. uroflow and EMG. C. CT scan abdomen and pelvis. D. transurethral incision valves. E. TUR.