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2006 Renal Week Lecture 3
Hematuria and Glomerulonephritis
Debbie GipsonUNC Kidney [email protected]: www.uncpeds.orgpassword: pediatriclib
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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
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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
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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
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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
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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
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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
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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
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Hematuria + Proteinuria
• Combination is an indicator of disease
• Gross hematuria may have associated low grade proteinuria
( Up/c < 0.5)
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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
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The most likely diagnosis is?
1. Hypercalciuria
2. Acute Post Strept GN
3. IgA nephropathy
4. Membranoproliferative GN
5. Systemic Lupus Erythematosis
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The most likely diagnosis is?
1. Hypercalciuria
2. Acute Post Strept GN
3. IgA nephropathy
4. Membranoproliferative GN
5. Systemic Lupus Erythematosis
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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
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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
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Post-infectious GN Classic 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
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Post infectious GN
Hematuria70% microscopic30% macroscopic
Proteinuriacommon
Hypertension 75%EdemaCongestive Heart Failure (elderly) Encephalopathy (children)
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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
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Proliferative GN
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Classic subepithelial humps
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Starry Night pattern (C3>IgG)
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Acute Postinfectious GNSubepithelial Humps
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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
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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
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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.
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What does this patient have?
1. Glomerular hematuria
2. Non-glomerular hematuria
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What does this patient have?
1. Glomerular hematuria
2. Non-glomerular hematuria
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The most likely diagnosis is?
1. Acute Post Strept GN
2. Hypercalciuria
3. Alport’s Syndrome
4. IgA nephropathy
5. Hemolytic Uremic Syndrome
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The most likely diagnosis is?
1. Acute Post Strept GN
2. Hypercalciuria
3. Alport’s Syndrome
4. IgA nephropathy
5. Hemolytic Uremic Syndrome
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Mesangial IgA Mesangial hypercellularity
IgA Nephropathy
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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
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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
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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
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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
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Indicators - proteinuria > 1 gm - nephrotic syndrome
- sustained HTN - male + gross hematuria
2%/year progress to ESRDOverall 20-40% progress to ESRD
IgA NephropathyPrognosis
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IgA Therapy
• ACEi (proven)
• Corticosteroids
• Mycophenolate (trials)
• Fish Oil
• Lipid control
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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
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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
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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
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Henoch-Schönlein Purpura
Vasculitis with IgA-dominant immune depositsaffecting small vessels, i.e. capillaries, venules, or arterioles. Typically involves skin, gut & glomeruli, and is associated with arthralgias or arthritis.
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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
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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