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Friday, December 9th, 2011

A 2½-year-old male presents with a 3-day history of progressive eyelid swelling. He had a URI 1 to 2 weeks ago. He has no history of pruritus or bee stings. His mother reports a decreased number of wet diapers per day, no fever, and no gross hematuria. He exhibits bilateral periorbital swelling, mild scrotal edema, and mild pitting edema over the pretibial region on PE.

Of the following, the MOST likely laboratory finding expected for this child is:A. depressed complement 1 esterase inhibitor value B. elevated bradykinin value C. elevated immunoglobulin E value D. elevated serum creatinine value E. large protein on urinalysis

The Nephrotic SyndromeAlterations of the glomerular capillary wallProteinuriaHypoalbuminemiaEdemaHyperlipidemia

Epidemiology16 per 100,000 childrenMales: females = 2:1 during childhoodIncreased familial incidenceAfrican American and Hispanic have greater

incidence and more severe disease

ClassificationPrimary (Idiopathic) Secondary

Minimal Change Nephrotic Syndrome (MCNS)

Infections (Hepatitis B, C, HIV, malaria, toxo, syphilis)

Focal Segmental Glomerulosclerosis (FSGS)

Drugs (gold, NSAIDS, pamidronate, interferon, heroin,

lithium)

Membranous Nephropathy (MN) Malignancies (lymphoma, leukemia)

Miscellaneaous (SLE, mesangioproliferative

glomerulonephritis, IgA nephropathy, DM)

90% of cases are primary

Minimal Change Nephrotic Syndrome (MCNS)85% of cases Fusion of epithelial foot processes

*Clinical FeaturesEdema

Visible when fluid retention > 3 to 5% of body weight

Low tissue resistance areas first Periorbital (mistaken for

allergy) Scrotal Labial

Becomes generalizedAnorexiaIrritabilityFatigueAbdominal discomfortDiarrhea

*Laboratory FindingsLow plasma proteinLow albumin

<2.5 g/dLHyperlipidemia

↑ VLDL, LDL, TGNormal HDL

*HyponatremiaDue to hyperlipidemia and retention of water (↑

ADH)Low total calcium*Remember complement and renal function are

normal!

Proteinuria

Start with dipstick (1+, 2+, 3+, 4+)

Quantitative measurement24-hour urine collection

>50 mg/kg/day or 40 mg/m2/hour = nephrotic Cumbersome

Urine protein/creatinine ratio <0.2 = normal (age > 2) >3 = nephrotic syndrome

*Treatment

Ancillary TherapyDiuretics to treat edema

Loops and Thiazides*May induce hypovolemia, secondary renal

failure, thromboembolism, or electrolyte disturbances

If diuretics fail can give albumin infusion Effective in children with very low serum albumin

(<1.5)

ACE inhibitorsStatins for hyperlipidemiaVaccinationsLow-sodium diet

A 4-year-old boy presents periorbital and extremity edema. Laboratory evaluation shows normal electrolyte values, BUN of 14.0 mg/dL, creatinine of 0.3 mg/dL, and albumin of 1.6 g/dL. UA demonstrates 3+ protein; and negative blood. Microscopy results are normal. Additionally, complement component (C3 and C4) values are normal, and results of serologic testing for ANA, hepatitis B and C, and HIV are negative.

Of the following, you are MOST likely to advise the parents thatA. a renal biopsy is warranted to determine the optimal treatment B. disease relapse can be expected in fewer than 25% of those

achieving remission C. patients who relapse have a similar prognosis as those who do

not respond to steroids D. remission is expected in more than 75% of patients who receive

corticosteroid treatment E. tacrolimus is the preferred treatment for patients who do not

respond to corticosteroids

CourseResponders

90% respond to steroids *Of those, 60% relapse

Frequency of relapses decreases with time Rare after puberty

Negligible risk of renal insufficiencySteroid-resistant

More common after age 210% of casesPoor prognosisRenal function deterioratesCandidates for dialysis and transplant

A 6 yo female is admitted for swelling of her face and extremities. Vital signs and PE are normal except for generalized anasarca. UA shows 4+ protein with no casts or RBCs. Serum albumin is 1.3 g/dL, cholesterol is 550 mg/dL, and creatinine is 0.4 mg/dL. This patient is at greatest risk for:

A. Centrilobular hepatic necrosisB. Cerebral edemaC. Congestive heart failureD. Myoglobinuric renal failureE. Peritonitis

*Complications of Nephrotic SyndromeSigns of acute renal failure (↓ GFR, oliguria)

Reversed with albumin infusion and diuresis

ThrombosisLoss of antithrombin III and protein SIncidence is 3%

Antiphospholipid syndrome

*Complications of Nephrotic SyndromeInfections

Peritonitis Empiric coverage with aminoglycoside and ampicillin

Cellulitis, meningitis, pneumonitis

Anasarca and pulmonary edema

Steroid useStunting of growthReduced bone mineral density

4 yo male with swelling of face and extremities x 2 days. Other than swelling, physical exam and vital signs are normal. UA shows 4+ protein and 5 RBCs/HPF. Of the following, the best indicator of good outcome for this child is:

A. Normal C3 complement valueB. Normal serum creatinineC. Resolution of symptoms with prednisone

treatmentD. Serum cholesterol less than 500 mg/dLE. Urine protein/creatinine ratio less than 5

Prognosis*Best prognostic indicator is steroid

responsiveness*95% of kids who will respond to steroids do so

within the first 4 weeksAs a result, patients with suspected MCNS are

started on Prednisone without a renal biopsy

Persistence or recurrence of hematuria often is a sign of impending steroid resistance

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 weekB. Microscopic hematuria showing more than 5

RBCs/HPFC. Reduced serum concentration of C3

complementD. Serum albumin less than 1.5 g/dLE. Urine protein/creatinine ratio of 1 at

presentation

Noon ConferenceHematuria/Proteinuria, Dr. Vehaskari