Block 8 Board Review

1. Block 8 Board Review Renal Disorders 7Feb14 Chauncey D. Tarrant, M.D. Chief of Residents 13-14

2. Pediatrics In Review Articles

3. QUIZ!!!

4. Nephrotic Syndrome

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

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

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

8. 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

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

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

11. When would you consider other agents (alkylating agents)??

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

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

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

15. What are the complications of Nephrotic Syndrome?

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

17. 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.

18. 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.

19. 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.

20. 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.

21. 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.

22. 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.

23. 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.

24. 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.

25. 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).

27. 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

28. 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

29. Renal Dysplasia

30. Neonate with unilateral flank mass…..

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

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

33. Autosomal Dominant Polycystic Kidney Disease

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

35. Autosomal Recessive Polycystic KidneyDisease

36. Polydypsia • Polyuria • Anemia • Growth Failure • Retinal Disease

37. Juvenile Nephronopthisis

38. Syndromes/Associations

40. Potter’s Sequence

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

42. VATER/VACTERL Association

43. Male Predominance • Hydronephrosis • Undescended Testes • Absence of Abdominal Wall Muscles

44. Prune Belly Syndrome

45. Congenital Nephropathy • Wilms Tumor • Gonadal Dysgenesis

46. Denys Drash • Congenital Nephropathy • Wilms Tumor • Gonadal Dysgenesis

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

48. Wilm’s Tumor • Aniridia • GU abnormalities • Retardation

49. PREP

50. 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