2006 Renal Week Lecture 3

Hematuria and Glomerulonephritis

Debbie GipsonUNC Kidney Cenerdebbie_gipson@med.unc.eduwebsite: www.uncpeds.orgpassword: pediatriclib

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

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

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

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

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

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

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

Hematuria + Proteinuria

• Combination is an indicator of disease

• Gross hematuria may have associated low grade proteinuria

( Up/c < 0.5)

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

                            

The most likely diagnosis is?

1. Hypercalciuria

2. Acute Post Strept GN

3. IgA nephropathy

4. Membranoproliferative GN

5. Systemic Lupus Erythematosis

The most likely diagnosis is?

1. Hypercalciuria

2. Acute Post Strept GN

3. IgA nephropathy

4. Membranoproliferative GN

5. Systemic Lupus Erythematosis

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

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

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

Post infectious GN

Hematuria70% microscopic30% macroscopic

Proteinuriacommon

Hypertension 75%EdemaCongestive Heart Failure (elderly) Encephalopathy (children)

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

Proliferative GN

Classic subepithelial humps

Starry Night pattern (C3>IgG)

Acute Postinfectious GNSubepithelial Humps

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

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

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.

                            

What does this patient have?

1. Glomerular hematuria

2. Non-glomerular hematuria

What does this patient have?

1. Glomerular hematuria

2. Non-glomerular hematuria

The most likely diagnosis is?

1. Acute Post Strept GN

2. Hypercalciuria

3. Alport’s Syndrome

4. IgA nephropathy

5. Hemolytic Uremic Syndrome

The most likely diagnosis is?

1. Acute Post Strept GN

2. Hypercalciuria

3. Alport’s Syndrome

4. IgA nephropathy

5. Hemolytic Uremic Syndrome

Mesangial IgA Mesangial hypercellularity

IgA Nephropathy

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

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

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

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

Indicators - proteinuria > 1 gm - nephrotic syndrome

- sustained HTN - male + gross hematuria

2%/year progress to ESRDOverall 20-40% progress to ESRD

IgA NephropathyPrognosis

IgA Therapy

• ACEi (proven)

• Corticosteroids

• Mycophenolate (trials)

• Fish Oil

• Lipid control

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

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

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

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.

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

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