Block 8 Board Review

Block 8 Board Review

Renal Disorders 7Feb14 Chauncey D. Tarrant, M.D. Chief of Residents 13-14

Pediatrics In Review Articles

QUIZ!!!

Nephrotic Syndrome

• What are the presenting signs and symptoms of Minimal Change Nephrotic Syndrome?

• What are the presenting signs and symptoms of Minimal Change Nephrotic Syndrome? Heavy Proteinuria, Hypoalbuminemia, Hyperlipidemia, Edema -Anorexia, Irritability, Fatigue, Abdominal discomfort, diarrhea

• What lab Findings might you find in a child with MCNS?

• What lab Findings might you find in a child with MCNS? Hypoalbuminemia, hyperlipidemia, hyponatremia (Why??), low serum calcium (iCa is normal), UPr/Cr ratio >3

• What would the initial treatment for a child with MCNS be??

• What would the initial treatment for a child with MCNS be?? Prednisone

• When would you consider other agents (alkylating agents)??

• When would you consider other agents (alkylating agents)?? frequent relapsers

• What would be your differential diagnosis for nephrotic syndrome? • If hematuria is a factor???

• What would be your differential diagnosis for nephrotic syndrome? MCNS, FSGS, CGN MPGN, Lupus Nephritis, IgA Nephritis

• What are the complications of Nephrotic Syndrome?

• What are the complications of Nephrotic Syndrome? Thromboembolic state, peritonitis, cellulitis, meningitis, pneumonitis, growth stunting, reduced mineral bone density

A 4-year-old boy presents with swelling of the face and extremities of 2 days’ duration. Physical examination reveals an otherwise happy child who has swelling of the face and pitting edema of all extremities. Vital signs and the rest of the physical examination findings are normal. Urinalysis shows 4+ proteinuria and 5 red blood cells per high-power field. Of the following, the most likely abnormality on histologic examination of this boy’s kidney is: A. Deposition of immunoglobulin A in mesangium. B. Diffuse thickening of glomerular capillary walls. C. Fusion of epithelial foot processes only. D. Mesangial cell proliferation and thickening of Bowman capsule. E. Scar tissue in segments of some glomeruli.

A 4-year-old boy presents with swelling of the face and extremities of 2 days’ duration. Physical examination reveals an otherwise happy child who has swelling of the face and pitting edema of all extremities. Vital signs and the rest of the physical examination findings are normal. Urinalysis shows 4+ proteinuria and 5 red blood cells per high-power field. Of the following, the most likely abnormality on histologic examination of this boy’s kidney is: A. Deposition of immunoglobulin A in mesangium. B. Diffuse thickening of glomerular capillary walls. C. Fusion of epithelial foot processes only. D. Mesangial cell proliferation and thickening of Bowman capsule. E. Scar tissue in segments of some glomeruli.

A 4-year-old boy presents with swelling of the face and extremities of 2 days’ duration. Physical examination reveals an otherwise happy child who has swelling of the face and pitting edema of all extremities. Vital signs and the rest of the physical examination findings are normal. Urinalysis shows 4+ proteinuria and 5 red blood cells per high-power field. Of the following, the best indicator of good outcome for this child is: A. Normal C3 complement value. B. Normal serum creatinine concentration. C. Resolution of symptoms with prednisone treatment. D. Serum cholesterol less than 500 mg/dL (13.0 mmol/L). E. Urine protein:creatinine ratio less than 5.

A 4-year-old boy presents with swelling of the face and extremities of 2 days’ duration. Physical examination reveals an otherwise happy child who has swelling of the face and pitting edema of all extremities. Vital signs and the rest of the physical examination findings are normal. Urinalysis shows 4+ proteinuria and 5 red blood cells per high-power field. Of the following, the best indicator of good outcome for this child is: A. Normal C3 complement value. B. Normal serum creatinine concentration. C. Resolution of symptoms with prednisone treatment. D. Serum cholesterol less than 500 mg/dL (13.0 mmol/L). E. Urine protein:creatinine ratio less than 5.

You are treating a 9-year-old girl who has nephrotic syndrome with prednisone. Which of the following is the strongest indication for performing renal biopsy? A. Lack of response to therapy after 1 week. B. Microscopic hematuria showing more than 5 red blood cells per high-power field in urine. C. Reduced serum concentration of C3 complement. D. Serum albumin less than 1.5 g/dL (15 g/L). E. Urine protein:creatinine ratio of 1 at presentation.

You are treating a 9-year-old girl who has nephrotic syndrome with prednisone. Which of the following is the strongest indication for performing renal biopsy? A. Lack of response to therapy after 1 week. B. Microscopic hematuria showing more than 5 red blood cells per high-power field in urine. C. Reduced serum concentration of C3 complement. D. Serum albumin less than 1.5 g/dL (15 g/L). E. Urine protein:creatinine ratio of 1 at presentation.

A 6-year-old girl is admitted for swelling of her face and extremities. Findings on her physical examination and vital signs are normal except for generalized anasarca. Urinalysis shows 4+ protein with no casts or red blood cells. Serum albumin is 1.3 g/dL (13 g/L), cholesterol is 550 mg/dL (14.2 mmol/L), and creatinine is 0.4 mg/dL (35.4 mcmol/L). This patient is at greatest risk for: A. Centrilobular hepatic necrosis. B. Cerebral edema. C. Congestive heart failure. D. Myoglobinuric renal failure. E. Peritonitis.

A 6-year-old girl is admitted for swelling of her face and extremities. Findings on her physical examination and vital signs are normal except for generalized anasarca. Urinalysis shows 4+ protein with no casts or red blood cells. Serum albumin is 1.3 g/dL (13 g/L), cholesterol is 550 mg/dL (14.2 mmol/L), and creatinine is 0.4 mg/dL (35.4 mcmol/L). This patient is at greatest risk for: A. Centrilobular hepatic necrosis. B. Cerebral edema. C. Congestive heart failure. D. Myoglobinuric renal failure. E. Peritonitis.

A 4-year-old boy is seen in the emergency department because of recurrent facial swelling. The mother reports that the boy has been evaluated by her pediatrician on several occasions with a similar complaint. Each time the boy was treated with 3- to 5-day courses of an antihistamine or oral steroid. The mother maintains full adherence with these treatment recommendations. Physical examination shows a healthy-appearing boy who has normal growth parameters. He is afebrile with a respiratory rate of 18 breaths/min, heart rate of 84 beats/min, and blood pressure of 90/60 mm Hg. The only finding of significance is facial puffiness and periorbital edema (Item Q232).

Of the following, the MOST appropriate next step is to A. obtain C1 esterase concentration B. obtain a specimen for urinalysis C. prescribe a 5-day course of diphenhydramine and prednisone D. reassure the mother and discharge the patient home E. refer the patient for an allergy evaluation

Of the following, the MOST appropriate next step is to A. obtain C1 esterase concentration B. obtain a specimen for urinalysis C. prescribe a 5-day course of diphenhydramine and prednisone D. reassure the mother and discharge the patient home E. refer the patient for an allergy evaluation

Renal Dysplasia

• Neonate with unilateral flank mass…..

• Neonate with unilateral flank mass….. – Think Multicystic Dysplastic Kidney

• • • •

Associated with Hypertension Associated with Intracranial Aneurysms Ultrasound is the preferred diagnostic method 3rd to 5th decades

Autosomal Dominant Polycystic Kidney Disease

• Neonates/Children/Adolescents • Associated with Portal Hypertension • Bilaterally enlarged kidneys

Autosomal Recessive Polycystic Kidney Disease

• • • • •

Polydypsia Polyuria Anemia Growth Failure Retinal Disease

Juvenile Nephronopthisis

Syndromes/Associations

Potter’s Sequence

• • • • • •

Imperforate Anus Vertebral Abnormalities TE fistula Renal Abnormalities Limb abnormalities (radial agenesis) Cardiac Defects (VSD, ASD, ToF)

VATER/VACTERL Association

• • • •

Male Predominance Hydronephrosis Undescended Testes Absence of Abdominal Wall Muscles

Prune Belly Syndrome

• Congenital Nephropathy • Wilms Tumor • Gonadal Dysgenesis

Denys Drash • Congenital Nephropathy • Wilms Tumor • Gonadal Dysgenesis

• • • • •

GU abnormalities Wilm’s Tumor Chromosome 11 affected Aniridia “Retardation”

• • • •

Wilm’s Tumor Aniridia GU abnormalities Retardation

PREP

A 14-year-old boy is brought to the emergency department after being struck by a car. On arrival, he is unresponsive and hypotensive. You intubate him endotracheally, place two large-bore intravenous lines, and infuse 3 L of 0.9% saline. Following these measures, his heart rate is 100 beats/min and blood pressure is 100/60 mm Hg. On secondary survey, you find a large swelling on the back of his head, a distended abdomen, blood at the urethral meatus, guaiac-positive stool, and a right femur fracture. Of the following, the procedure that is CONTRAINDICATED in this patient is

A. diagnostic peritoneal lavage B. femoral traction splint placement C. orogastric tube placement D. retrograde urethrography E. urethral catheter placement

A. diagnostic peritoneal lavage B. femoral traction splint placement C. orogastric tube placement D. retrograde urethrography E. urethral catheter placement

A 17-year-old sexually active girl presents for a follow-up evaluation after her third episode of a urinary tract infection. She is currently asymptomatic. The results of renal ultrasonography and voiding cystourethrography are negative. She asks you how to prevent further episodes. Of the following, you are MOST likely to advise her to

A. drink cranberry juice frequently B. increase her daily water intake

C. make sure to void after intercourse D. self-medicate with antibiotics for 3 days when symptomatic E. use single-dose postcoital antibiotic prophylaxis

A. drink cranberry juice frequently B. increase her daily water intake

C. make sure to void after intercourse D. self-medicate with antibiotics for 3 days when symptomatic E. use single-dose postcoital antibiotic prophylaxis

A 5-year-old girl presents with mild flank pain. She has no history of fever, trauma, gross hematuria, frequency, urgency, or dysuria. Physical examination of the afebrile child reveals a heart rate of 90 beats/min, respiratory rate of 20 breaths/min, blood pressure of 106/62 mm Hg, and normal growth parameters. There are no other findings of note. A dipstick urinalysis reveals a specific gravity of 1.020; pH of 8.5; 2+ protein; and negative for blood, leukocyte esterase, and nitrite. A urine protein-tocreatinine ratio performed on this specimen is subsequently reported as 0.02. Of the following, the MOST likely explanation for this girl’s urinary findings is

A. alkaline urine B. minimal change disease

C. orthostatic proteinuria D. urinary tract infection E. urolithiasis

A. alkaline urine B. minimal change disease

C. orthostatic proteinuria D. urinary tract infection E. urolithiasis

You are examining a 1-day-old term male infant during rounds in the newborn nursery and palpate a right upper quadrant abdominal mass. The remainder of the examination findings are normal. The infant’s mother is an otherwise healthy 22year-old gravida 1, para 1, woman who received no prenatal care. You note on the chart that the infant voided at 6 hours of age. Of the following, the MOST likely diagnosis in this infant is

A. posterior urethral valves B. ureterocele

C. ureteropelvic junction obstruction D. urolithiasis E. Wilms tumor

A. posterior urethral valves B. ureterocele

C. ureteropelvic junction obstruction D. urolithiasis E. Wilms tumor

You are examining a newborn who has wrinkling of the abdominal wall skin. His mother recalls her obstetrician mentioning that her “fluid was low.” The infant was born at 37 weeks’ gestation, and his birthweight was appropriate for gestational age. Of the following, the MOST likely additional findings expected in this infant are cryptorchidism and

A. bilateral hydronephrosis B. hypospadias

C. nephrocalcinosis D. polycystic kidney disease E. unilateral renal agenesis

A. bilateral hydronephrosis B. hypospadias

C. nephrocalcinosis D. polycystic kidney disease E. unilateral renal agenesis

A 3-year-old boy presents with difficulty voiding. His mother states that he appears fussy and seems to strain when trying to void. He is otherwise well and has no history of fever, vomiting, difficulty feeding, swelling, or gross hematuria. He does have a history of acute pyelonephritis at 10 months of age. Ultrasonography and voiding cystourethrography (VCUG) results were reported as normal. His mother noted that he had some visible blood in his urine immediately after undergoing his VCUG, which cleared several hours later. The boy was toilet trained at 2½ years of age. He has had no recent urinary tract infections. Physical examination reveals an uncircumcised male who has easily retractable foreskin and no sacral dimples or hair tufts on the back. Urinalysis results include a specific gravity of 1.020; pH of 6; and no blood, protein, leukocyte esterase, or nitrite. Microscopy is negative. Of the following, the MOST likely explanation for this boy’s difficulty voiding is

A. bladder stone B. cystitis

C. neurogenic bladder D. posterior urethral valves E. urethral stricture

A. bladder stone B. cystitis

C. neurogenic bladder D. posterior urethral valves E. urethral stricture

A 7-year-old girl complains of 1 day of dysuria, vaginal itching, and perineal pain without fever or vomiting. She does not have urgency, frequency, or enuresis, but she does report a light yellow discharge. She denies any type of trauma, including sexual abuse. Her vital signs and abdominal examination findings are normal, and she has no flank tenderness. Perineal inspection shows a minimal amount of mucoid vaginal discharge and moderate erythema of the vestibule. There is no evidence of trauma. A urine dipstick evaluation is negative except for trace leukocyte esterase. Of the following, the MOST appropriate next step in the evaluation/treatment of this patient is

A. a course of oral antibiotics for urinary tract infection B. application of bland emollients as needed for symptom relief C. topical anticandidal cream for 7 days D.urine testing for gonorrhea and chlamydia E. vaginoscopy

A. a course of oral antibiotics for urinary tract infection B. application of bland emollients as needed for symptom relief C. topical anticandidal cream for 7 days D.urine testing for gonorrhea and chlamydia E. vaginoscopy

An 8-year-old boy presents with cola-colored urine without blood clots. He was well until 2 days ago, when he developed a sore throat with upper respiratory tract infection symptoms. He denies any dysuria, frequency, urgency, flank pain, or trauma. On physical examination, his temperature is 37.8°C, heart rate is 84 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 118/78 mm Hg. He has no costovertebral tenderness, abdominal tenderness, or edema. The urinalysis reveals: Specific gravity, 1.025 pH, 6.0 3+ blood 3+ protein 1+ leukocyte esterase Nitrite, negative Microscopy shows more than 100 red blood cells/high-power field (hpf) and 5 to 10 white blood cells/hpf. Other laboratory findings include: Blood urea nitrogen, 24 mg/dL (8.6 mmol/L) Creatinine, 0.9 mg/dL (79.6 mcmol/L) Complement component 3 (C3), 140 mg/dL (normal, 80 to 200 mg/dL) Complement component 4 (C4), 30 mg/dL (normal, 16 to 40 mg/dL) Antinuclear antibody, negative Of the following, the MOST likely diagnosis is

A. acute pyelonephritis B. immunoglobulin A glomerulonephritis

C. postinfectious glomerulonephritis D. urolithiasis E. viral cystitis

A. acute pyelonephritis B. immunoglobulin A glomerulonephritis

C. postinfectious glomerulonephritis D. urolithiasis E. viral cystitis

You recently diagnosed Burkitt lymphoma in one of your patients. He is an 8-year-old boy, and his pediatric oncologist is planning to treat him with CODOX-M (cyclophosphamide, doxorubicin, vincristine, methotrexate intravenously and cytarabine and methotrexate intrathecally) for cycles 1 and 3 and IVAC (ifosfamide, etoposide, cytarabine intravenously and methotrexate intrathecally) for cycles 2 and 4. As her son’s longstanding pediatrician, the boy’s mother asks you about the adverse effects of the therapy. Because her cousin is undergoing dialysis, she asks you specifically which medication is most likely to damage her son’s kidneys. Of the following, the MOST nephrotoxic medication being prescribed is

A. cyclophosphamide

B. cytarabine

C. doxorubicin

D. etoposide

E. ifosfamide

A. cyclophosphamide

B. cytarabine

C. doxorubicin

D. etoposide

E. ifosfamide

A 15-year-old girl presents with gross hematuria (bright red blood with clots) accompanied by sharp left-sided back pain. She denies fever, dysuria, frequency, urgency, or trauma. On physical examination, her temperature is 37.3°C, heart rate is 90 beats/min, respiratory rate is 18 breaths/min, and blood pressure is 116/72 mm Hg. You note no costovertebral angle tenderness, suprapubic tenderness, or edema. Urinalysis reveals: Specific gravity, 1.020 pH, 6 3+ blood Trace protein Leukocyte esterase, negative Nitrite, negative Microscopy documents 20 to 50 red blood cells/high-power field (hpf) and 5 to 10 squamous epithelial cells/hpf. Abdominal ultrasonography shows mild dilation of the collecting system on the left, with some debris in the bladder. You suspect urolithiasis. Of the following, the study that is MOST likely to establish the diagnosis is

A. abdominal computed tomography scan B. diethylene-triamine-penta-acetic acid (DTPA) furosemide renal scan C. magnetic resonance urography D. random urine sample for calcium and creatinine E. 24-hour urine collection for calcium and creatinine

A. abdominal computed tomography scan B. diethylene-triamine-penta-acetic acid (DTPA) furosemide renal scan C. magnetic resonance urography D. random urine sample for calcium and creatinine E. 24-hour urine collection for calcium and creatinine

A 3-week-old term infant presents with vomiting, increased fussiness, and poor weight gain. In answer to your questioning, his mother states he has a vigorous suck, decreased tearing, and several wet diapers per day. The infant is formula-fed. On physical examination, he is fussy and difficult to console. His weight is 2.9 kg (birthweight was 3.2 kg), temperature is 37.3°C, heart rate is 180 beats/min, respiratory rate is 40 breaths/min, and blood pressure is 76/36 mm Hg. His anterior fontanelle is flat and mucous membranes are tacky. His chest is clear to auscultation, he has tachycardia without murmurs, and he has no abdominal masses. Skin evaluation reveals reduced turgor, and capillary refill time is approximately 3 to 4 seconds. Laboratory results include: sodium of 168 mEq/L (168 mmol/L), potassium of 3.5 mEq/L (3.5 mmol/L), chloride of 130 mEq/L (130 mmol/L), and bicarbonate of 20 mEq/L (20 mmol/L). Urinalysis shows a specific gravity of 1.002, pH of 5.5, and otherwise negative results. Of the following, the MOST likely diagnosis for this patient is

A. Bartter syndrome B. diabetes insipidus

C. Fanconi syndrome D. Gitelman syndrome E. improper formula preparation

A. Bartter syndrome B. diabetes insipidus

C. Fanconi syndrome D. Gitelman syndrome E. improper formula preparation

You are called to attend the delivery of a newborn male who had been monitored carefully prenatally for moderate oligohydramnios. Prenatal ultrasonography suggested bilateral cystic dysplastic kidneys, with no other congenital anomalies identified. Of the following, the MOST likely immediate lifethreatening problem in this infant is

A. bladder outlet obstruction B. cardiac arrhythmias C. liver failure D. renal failure E. respiratory failure

A. bladder outlet obstruction B. cardiac arrhythmias C. liver failure D. renal failure E. respiratory failure

A 6-year-old girl presents for a health supervision visit that was scheduled as a follow-up appointment after she had an elevated blood pressure at an urgent care facility during an evaluation for abdominal pain. Her abdominal pain has resolved. Her mother recalls the blood pressure in the urgent care center as 135/90 mm Hg. The girl has had two urinary tract infections with fever in the past, and her father had hypertension diagnosed at age 45 years. On physical examination, the girl’s temperature is 37.3°C, heart rate is 90 beats/min, respiratory rate is 20 breaths/min, and blood pressure is 146/86 mm Hg. A repeat blood pressure reading is 142/88 mm Hg. The four limb blood pressures are: 142/88 mm Hg in the right arm, 144/84 mm Hg in the left arm, 156/100 mm Hg in the right leg, and 160/96 mm Hg in the left leg. You find no cardiac murmurs, abdominal bruits, or edema. Femoral pulses are 2+ and symmetrical bilaterally. Renal ultrasonography shows the left kidney to be 8.5 cm with normal corticomedullary differentiation and the right kidney to be 5.5 cm with increased echogenicity. Of the following, the MOST likely cause for this patient’s elevated blood pressure is

A. coarctation of the aorta B. essential hypertension

C. renal artery stenosis D. renal hypoplasia/dysplasia E. renal scarring from prior pyelonephritis

A. coarctation of the aorta B. essential hypertension

C. renal artery stenosis D. renal hypoplasia/dysplasia E. renal scarring from prior pyelonephritis