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STEP1 Renal
ANATOMY & PHYSIOLOGY Top High-Yield Topics Kidney Anatomy Body Fluid Compartments Glomerular Physiology Glomerular Filtration Barrier Renal Clearance Glomerular Filtration Rate (GFR) Filtration Nephron Physiology Nephron Schematic Free Water Clearance Reabsorption and Secretion Rate Glucose Clearance Amino Acid Clearance Relative Concentrations Along Proximal Tubule Endocrine

Primary Nephrotic Syndrome

Author: Medbullets Team

5 Topic updated on 08/18/13 11:50am

Snap Shot A three year old boy presents with puffy eyes to the ER. His physician had treated him for allergies but the symptoms did not improve. On PE the patient is noted to have lower extremity edema. He has a serum albumin of 2.5 g/dL, and 4+ proteinuria on urinalysis. Electron microscopy is shown on the right.

Introduction Can be broken down into two types primary nephrotic syndrome refers to disease limited to kidney subtypes include (see below) minimal change disease (MCD) focal segmental glomerulosclerosis diffuse membranous glomerulopathy membranoproliferative glomerulonephritis secondary nephrotic syndrome is a multisystem disease Associated conditions spontaneous peritonitis risk of due to Streptococcus pneumoniae infection
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thromboembolism (DVT & PE) hypercoagulable state with risk of DVT due to loss of antithrombin III in the urine Presentation Symptoms periorbital swelling Physical exam hypertension generalized edema (anasarca) caused by loss of albumin / decreased oncotic pressure Evaluation Labs hypoalbuminemia (serum albumin of <3.0 g/dl) proteinuria > 3.5 g/day nephrOtic syndrome (loss of prOtien) a high protein urine is "frothy" hyperlipidemia with lipiduria low albumin stimulates liver to produce cholesterol to correct decrease in plasma oncotic pressure Urinalysis fatty casts (Maltese crosses) resulting from hyperlipidemia key finding in nephrotic syndrome Microscopy light and electron microscopy both critical to establish diagnosis immuno critical to establish diagnosis

Glomerulopathy Comparative Table Immunofluorescence pattern Minimal change disease Negative Location of IC deposition n/a Compliment type activated n/a
Electron Microscopy

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Focal segmental Diffuse membranous glomerulopathy Membranoproliferative glomerulonephritis

Negative Granular Granular

n/a Subepithelial Subendothelial

n/a n/a Classical + Alternative

Minimal change disease (lipoid nephrosis) Introduction most common cause of nephrotic syndrome in young children results from loss of polyanions (neg charge) in the GBM selective loss of albumin but not globulins associated with allergic diseases may be triggered by a recent infection or an immune stimulus (e.g. vaccination) treating allergy often improves nephrotic syndrome Presentation periorbital edema in a normotensive child following an upper respiratory infection or immunization Evaluation serology shows hyperlipidemia and hypoalbuminemia light microscopy findings show completely normal glomeruli (hence name Minimal change) urinalysis shows maltese crosses electron microscopy findings show effacement of podocytes Treatment prednisone disease very responsive with excellent prognosis Prognosis, Prevention, and Complications infection at risk for encapsulated organism infection especially Strep. pneumonia Focal segmental glomerulosclerosis Introduction clinically similar to MCD, but occurs in adults with refractory HTN different in that protein loss is nonspecific (unlike MCD which is selective for albumin) usally idiopathic
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can be associated with IV drug use (heroin) HIV DM Sickle cell anemia Presentation typical presentation of idiopathic type is young, black, hypertensive male Evaluation immunofluorescence is negative Treatment prednisone +/- cyclophosphamide Prognosis, Prevention and Complications very poor due to refractory disease Diffuse membranous glomerulopathy Introduction most common cause of nephrotic syndrome in adults causes include infection (HBV, HCV, syphilis, malaria) drugs (captopril, gold, penicillamine) occult malignancy SLE (found in 10% of patients) Presentation general signs and symptoms of nephrotic syndrome Evaluation light microscopy shows diffuse thickening of capillaries and GBM electron microscopy shows subepithelial deposits show a "spike and dome" Treatment prednisone +/- cyclophosphamide Prognosis, Prevention, and Complications slowly progressive disorder with decreasing response to steroids Membranoproliferative glomerulonephritis Introduction
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two forms type I is slowly proliferative association of HBV > HCV type II is more aggressive results from a C3 nephritic factor (C3NeF) which is an autoantibody against C3 which leads to continuous activation of C3 Presentation similar to general nephrotic syndrome Evaluation type I EM shows "tram track" appearance of GBM due to splitting by mesangial growth type II serology shows serum C3 levels Treatment prednisone +/- plasmapheresis Prognosis, Prevention, and Complications very poor prognosis with slow progression to CRF

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0) (M1.RL.8) A 5-year-old female presents to your office with periorbital edema and proteinuria. Her mother reports that she "just got over a cold" last week. On examination, you note a pulse rate of 70/minute and a blood pressure of 118/78. Which protein(s) would likely be found in large amounts in this patient's urine? Review Topic 1. 2. 3. 4. 5. IgG IgA Albumin Albumin and IgG Albumin, IgG, and IgA

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PREFERRED RESPONSE

Recent Videos
Membranoproliferative Glomerulonephritis
Step 1 - Nephrology - Treatment Consult - Primary Nephrotic Syndrome Step I Lecture 11/4/2012 28 views

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