Glomerular disorders

Question 1

Define the 4 features of nephrotic syndrome

Answer 1

Proteinuria (+++ or ++++, or >40mg/m2/hr)
Edema
Hypoalbuminemia (<3g/dL)
Hyperlipidemia

Question 2

What disease process accounts for 80 - 85% of nephrotic syndrome in childhood?

Answer 2

Minimal change glomerulonephritis

Question 3

Percentage of children with minimal change disorder who have microscopic hematuria?

Answer 3

15 - 20%

Question 4

Which glomerulonephridities are associated with hypocomplementemia?

Answer 4

Postinfectious
SLE
Membranoproliferative GN

Question 5

2nd most common cause of primary nephrotic syndrome in children?

Answer 5

Focal segmental glomerulosclerosis

10-15% of cases will progress to ESRF, which can reoccur in transplant

Question 6

Recurrence risk in transplant in FSGS (focal segmental glomerulosclerosis)?

Answer 6

~40%

Question 7

Classification of SLE nephritis (Types I-VI)

Answer 7

Type I - abnormal urine sediment with normal light microscopy
Type II - mesangioproliferative GN
Type III - membranproliferative GN wqith 50% of glomeruli affected, immunoflurescence microscopy for IgG and C3 evident (most common)
Type V - membranous nephropathy
Type VI - advanced sclerosing lupus nephritis

Question 8

Drugs that can cause an interstitial nephritis

Answer 8

beta-lactams
sulphonamides
rifampicin
ethambutol
erythromycin

Question 9

Biopsy features in minimal change disease

Answer 9

Normal on light microscopy

Loss of foot processes on electron microscopy

Question 10

3 most common causes of GN in kids?

Answer 10

Acute post-streprococcal GN
IgA nephropathy
SLE nephritis

Question 11

How long post streptococcal infection does post-strep GN occur?

Answer 11

1-3 weeks

Question 12

Which of the following will be elevated with which preceding streptococcal infection?
ASO
Anti-DNase B

Answer 12

ASO: elevated post strep pharyngitis in 80-85% pts

Antihyaluronidase + Anti-DNase B: elevated post skin infection

Question 13

How long after post-strep GN should serum complement levels return to normal?

Answer 13

Complement levels should return to normal within 8 weeks of post-strep GN

Question 14

Persistently depressed serum complement (>8wks duration) is indicative of?

Answer 14

Membranoproliferative GN

Question 15

Gross hematuria with associated URTI is indicative of what?

Answer 15

IgA Nephropathy (Berger disease)
M>F (twice as common in boys)

Question 16

Glomerulonephritis + sensorineural hearing loss + ocular abnormalities in combination are indicative of?

Answer 16

Alport syndrome

Males more severely affected –> ESRD by 20-30yrs

Question 17

Extra-renal complications of nephrotic syndrome?

Answer 17

1. Inc VLDL production by liver
2. Hypercoaguable (inc risk renal vein thrombosis)
3. Infection (loss of IgG –> encapsulated bacteria)

Question 18

Clinical features of minimal change disease

Answer 18

Proteinuria (dependent oedema)
Microscopic hematuria in ~10% pts (macroscopic not c/w MCD)
Serum C3 NORMAL
BP + renal function normal in most patients

Question 19

Which organisms are most commonly responsible for peritonitis in nephrotic syndrome?

Answer 19

Pneumococcus
E Coli (20-25%)

Question 20

Age of onset of minimal change nephrotic syndrome?

Answer 20

Between 1 yr and 8 years (outside this range, consider looking for alternative diagnoses)

Question 21

Features of Henoch-Scholein Purpura? (tetrad)

Answer 21

1. Palpable purpura (without thrombocytopenia or coagulopathy)
2. Arthritis/Arthralgia
3. Abdominal pain
4. Renal disease

Question 22

Pathogenesis of HSP?

Answer 22

Immune mediated vasculitis associated with IgA deposition

Question 23

Findings on biopsy in patient’s with HSP is identical to which other nephropathy?

Answer 23

IgA nephropathy

Question 24

Renal disease in HSP can be?

Answer 24

Hematuria
Proteinuria
Nephrotic syndrome

Question 25

Findings in HSP renal disease associated with long term renal impairment?

Answer 25

Nephrotic range proteinuria
Elevated serum creatinine
Hypertension

Question 26

ESRD develops in what percentage of patients with HSP associated renal disease?

Answer 26

~5%

Question 27

What percentage of patient’s with IgA nephropathy progress to ESRD?

Answer 27

Estimated 20 - 25%