2006 Renal Week Lecture 3 Hematuria and Glomerulonephritis Debbie Gipson UNC Kidney Cener

1. 2006 Renal Week Lecture 3 Hematuria and Glomerulonephritis Debbie Gipson UNC Kidney Cener debbie_gipson@med.unc.edu website: www.uncpeds.org password: pediatriclib

2. Program Announcements • UNC Nephrology Fellowship • 1 position each year • accepting applications for 2007 and 2008 • Educational and Meeting Opportunities • Univ. Miami: Pediatric Nephrology Seminar (Clinical), Miami Beach, 2007 • American Society of Nephrology • American Society of Pediatric Nephrology

3. Renal Week Evaluations • Please complete the evaluation • Topics • Format • Presentations and presenters • Return to envelope in back of room or via campus mail to Rowena Brown, CB 7155

4. Case 1 • A 17 year old previously healthy African American female presents for a well child visit. • Dipstick evaluation reveals moderate blood and 3+ proteinuria. Microscopic examination of the urinary sediment reveals 10 RBC/hpf and no casts. • Physical examination is unremarkable

5. Your assessment and plan is: • 1. Microscopic hematuria. Repeat UA x 2 • 2. Asymptomatic proteinuria and hematuria. Requires no additional evaluation • 3. Proteinuria and hematuria. Additional evaluation indicated

6. Your assessment and plan is: • 1. Microscopic hematuria. Repeat UA x 2 • 2. Asymptomatic proteinuria and hematuria. Requires no additional evaluation • 3. Proteinuria and hematuria. Additional evaluation indicated

7. Appropriate tests may include each of the following except: 1. AM Urine for protein & creatinine 2. Serum chemistries for creatinine, albumin, and cholesterol 3. Urine for calcium excretion 4. Serum complement 5. Consider hepatitis and HIV serologies 6. Renal ultrasound

8. Appropriate tests include each of the following except: • 1. 24 hour urine for protein and creatinine • 2. Serum chemistries for creatinine, albumin, and cholesterol • 3. Urine for calcium excretion • 4. Serum complement • 5. Consider hepatitis and HIV serologies • 6. Renal ultrasound

9. Hematuria + Proteinuria • Combination is an indicator of disease • Gross hematuria may have associated low grade proteinuria ( Up/c < 0.5)

11. CASE 2 • A six year old girl develops a puffy face and notices that her urine has turned brown. • No family history of renal disease. A sister complained of a sore throat one week before the onset of dark urine. • Physical exam shows generalized edema and a blood pressure of 135/ 83 mmHg. • Urinalysis contains: large hemoglobin, 2+protein

13. The most likely diagnosis is? 1. Hypercalciuria 2. Acute Post Strept GN 3. IgA nephropathy 4. Membranoproliferative GN 5. Systemic Lupus Erythematosis

14. The most likely diagnosis is? 1. Hypercalciuria 2. Acute Post Strept GN 3. IgA nephropathy 4. Membranoproliferative GN 5. Systemic Lupus Erythematosis

15. Which of the following tests will be most helpful in determining the diagnosis? 1. Serum BUN & creatinine 2. Serum complement & streptozyme 3. Serum IgA 4. Renal ultrasound 5. Serum albumin

16. Which of the following tests will be most helpful in determining the diagnosis? 1. Serum BUN & creatinine 2. Serum complement & streptozyme 3. Serum IgA 4. Renal ultrasound 5. Serum albumin

17. Post-infectious GNClassic Group A Streptococci Anticedent illness • Pharyngitis (7-21 d) or impetigo (14-21 d) • Nephritogenic strain of streptococcus • Rheumatic fever and nephritis rarely concurrent • Peak age 2 to 6 years • Males > females • Epidemics • Attack rates 10-15% • 38% Household contacts

18. Post infectious GN Hematuria 70% microscopic 30% macroscopic Proteinuria common Hypertension 75% Edema Congestive Heart Failure (elderly) Encephalopathy (children)

19. Post-infectious GN • Laboratory • Low C3 (x 6-8 weeks) • ASO or streptozyme titers acute rise if Strep. • May increase serum Cr; uncommon renal failure • Hematuria (1 year), Proteinuria, RBC casts • Pathology

20. Proliferative GN

21. Classic subepithelial humps

22. Starry Night pattern (C3>IgG)

23. Acute Postinfectious GN Subepithelial Humps

24. Which one of the following is not associated with depressed serum complement values? 1. Acute post strept GN 2. Membranoproliferative GN 3. IgA nephropathy 4. SLE

25. Which one of the following is not associated with depressed serum complement values? • 1. Acute post strept GN • 2. Membranoproliferative GN • 3. IgA nephropathy • 4. SLE

26. CASE 5 • A 12 year old girl has a sore throat and that same day notices that her urine turns brown. • She feels well and without specific symptoms. • She has not had previous urinalyses. There is no family history of renal disease. • Her examination is normal. • The urinalysis contains large hemoglobin and 1+ protein.

28. What does this patient have? 1. Glomerular hematuria 2. Non-glomerular hematuria

29. What does this patient have? 1. Glomerular hematuria 2. Non-glomerular hematuria

30. The most likely diagnosis is? 1. Acute Post Strept GN 2. Hypercalciuria 3. Alport’s Syndrome 4. IgA nephropathy 5. Hemolytic Uremic Syndrome

31. The most likely diagnosis is? 1. Acute Post Strept GN 2. Hypercalciuria 3. Alport’s Syndrome 4. IgA nephropathy 5. Hemolytic Uremic Syndrome

33. Which of the following suggests a serious prognosis? 1. Family history 2. Proteinuria 3. Elevated serum IgA values 4. Low serum complement values 5. Abdominal pain

34. Which of the following suggests a serious prognosis? 1. Family history 2. Proteinuria 3. Elevated serum IgA values 4. Low serum complement values 5. Abdominal pain

35. IgA Nephropathy(Bergers Disease) • Most common cause of GN world wide • Forms: • Idiopathic • Familial 10% • Secondary (liver disease, chronic lung or GI d/o) • Age 15 - 30 yo • Asian > Caucasian > African Americans

36. IgA NephropathyClinical Findings • Presentation • 40% asymptomatic hematuria • 40% gross hematuria (more frequent in children) • 10% nephrotic syndrome • 10% renal failure (including rare patients with RPGN) • 5% Malignant HTN • Increase in symptoms with infection

37. IgA Nephropathy Prognosis • Indicators • - proteinuria > 1 gm • - nephrotic syndrome • - sustained HTN • - male • + gross hematuria • 2%/year progress to ESRD • Overall 20-40% progress to ESRD

38. IgA Therapy • ACEi (proven) • Corticosteroids • Mycophenolate (trials) • Fish Oil • Lipid control

39. Next Case • 6 year old male • Crampy abdominal pain without rebound • Rash on buttocks and lower extremities • Urine with 2+ blood and 2+ protein • Serum Complements are normal

40. The most likely diagnosis is? 1. Acute Post Strept GN 2. Systemic Lupus Erythematosis 3. Alport’s Syndrome 4. IgA nephropathy 5. Hemolytic Uremic Syndrome 6. Henoch Schonlein Purpura

41. The most likely diagnosis is? 1. Acute Post Strept GN 2. Systemic Lupus Erythematosis 3. Alport’s Syndrome 4. IgA nephropathy 5. Hemolytic Uremic Syndrome 6. Henoch Schonlein Purpura

43. HSP Clinical Features • Most common 4-5 years • Males > females • Clinical • sudden onset extensor surface rash • edema of hands, feet, face, scalp • arthralgia 70% • abdominal pain, vomiting 60% • Intussusception, protein losing enteropathy • nephritis 40 - 60 % • CNS symptoms

44. HSP Prognosis • Chronic renal failure 2 to 5% • Indicators • acute nephritis • persistent nephrotic syndrome • older age • glomerular crescents • Therapy • Rapidly progressive GN • The cocktail: steroids/cytoxan/pharesis