Www Medbullets Com Step1 Renal 15028 Primary Nephrotic Syndr

7/27/2019 Www Medbullets Com Step1 Renal 15028 Primary Nephrotic Syndr

1/6

pdfcrowd comopen in browser PRO version Are you a developer? Try out the HTML to PDF API

5

Author: Medbullets Team Topic updated on 08/18/13 11:50am

Primary Nephrotic Syndrome

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

STEP1 Renal

ANATOMY & PHYSIOLOGY

Top High-Yield Topics

Kidney Anatomy

Body Fluid Compartments

Glomerular Physiology

Glomerular Filtration Barrier

Renal Clearance

Glomerula r Filtration Rate (GFR)

Filtration

Nephron Physiology

Nephron Schematic

Free Water Clearance

Reabsorption and Secretion Rate

Glucose Clearance

Amino Acid Clearance

Relative Concentrations AlongProximal Tubule

Search Join now Sign in

Step 1 Step 2 & 3 QBank Cases Videos Posts Groups Colleagues Messages About Feedback

Biochem Embryo Micro Immuno Path Stats Cards Endo GI Heme Onc MSK Neuro Psych Renal Repro Pulm Anatomy Pharm
http://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://www.medbullets.com/user/dashboard?id=8&specialty=cardiovascularhttp://www.medbullets.com/user/dashboard?id=9&specialty=endocrinehttp://www.medbullets.com/user/dashboard?id=10&specialty=gastrointestinalhttp://www.medbullets.com/user/dashboard?id=11&specialty=hematologyhttp://www.medbullets.com/user/dashboard?id=18&specialty=oncologyhttp://www.medbullets.com/user/dashboard?id=12&specialty=musculoskeletal-and-connective-tissuehttp://www.medbullets.com/user/dashboard?id=13&specialty=neurologyhttp://www.medbullets.com/user/dashboard?id=9&specialty=endocrinehttp://www.medbullets.com/user/dashboard?id=10&specialty=gastrointestinalhttp://www.medbullets.com/user/dashboard?id=11&specialty=hematologyhttp://www.medbullets.com/user/dashboard?id=11&specialty=hematologyhttp://www.medbullets.com/user/dashboard?id=18&specialty=oncologyhttp://www.medbullets.com/user/dashboard?id=12&specialty=musculoskeletal-and-connective-tissuehttp://www.medbullets.com/user/dashboard?id=13&specialty=neurologyhttp://www.medbullets.com/user/dashboard?id=14&specialty=psychiatryhttp://www.medbullets.com/user/dashboard?id=15&specialty=renalhttp://www.medbullets.com/video/listhttp://www.medbullets.com/post/listhttp://www.medbullets.com/group/publichttp://www.medbullets.com/group/publichttp://www.medbullets.com/group/publichttp://www.medbullets.com/user/mycolleagueshttp://www.medbullets.com/step1-renal/15013/amino-acid-clearancehttp://www.medbullets.com/step1-renal/15011/reabsorption-and-secretion-ratehttp://www.medbullets.com/step1-renal/15010/free-water-clearancehttp://www.medbullets.com/step1-renal/15014/nephron-schematichttp://www.medbullets.com/step1-renal/15006/glomerular-filtration-rate-gfrhttp://www.medbullets.com/step1-renal/15005/renal-clearancehttp://www.medbullets.com/step1-renal/15004/glomerular-filtration-barrierhttp://www.medbullets.com/step1-renal/15003/body-fluid-compartmentshttp://www.medbullets.com/topic/dashboard?id=15&specialty=http://www.medbullets.com/user/dashboard?id=2&specialty=biochemistryhttp://www.medbullets.com/user/dashboard?id=3&specialty=embryology1http://www.medbullets.com/user/dashboard?id=all&SubCategoryID=1http://www.medbullets.com/user/dashboard?id=all&SubCategoryID=2http://www.medbullets.com/http://www.medbullets.com/http://pdfcrowd.com/http://pdfcrowd.com/redirect/?url=http%3a%2f%2fwww.medbullets.com%2fstep1-renal%2f15028%2fprimary-nephrotic-syndrome&id=ma-131020074103-c9788452http://pdfcrowd.com/customize/http://pdfcrowd.com/html-to-pdf-api/?ref=pdfhttp://www.medbullets.com/user/27691http://www.medbullets.com/step1-renal/15027/nephritic-syndromehttp://www.medbullets.com/step1-renal/15071/secondary-nephrotic-syndromehttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpghttp://www.medbullets.com/step1-renal/15071/secondary-nephrotic-syndromehttp://www.medbullets.com/topic/dashboard?id=15&specialty=http://www.medbullets.com/step1-renal/15001/kidney-anatomyhttp://www.medbullets.com/step1-renal/15003/body-fluid-compartmentshttp://www.medbullets.com/step1-renal/15004/glomerular-filtration-barrierhttp://www.medbullets.com/step1-renal/15005/renal-clearancehttp://www.medbullets.com/step1-renal/15006/glomerular-filtration-rate-gfrhttp://www.medbullets.com/step1-renal/15008/filtrationhttp://www.medbullets.com/step1-renal/15014/nephron-schematichttp://www.medbullets.com/step1-renal/15010/free-water-clearancehttp://www.medbullets.com/step1-renal/15011/reabsorption-and-secretion-ratehttp://www.medbullets.com/step1-renal/15012/glucose-clearancehttp://www.medbullets.com/step1-renal/15013/amino-acid-clearancehttp://www.medbullets.com/step1-renal/15015/relative-concentrations-along-proximal-tubulehttp://www.medbullets.com/http://www.medbullets.com/registerhttp://www.medbullets.com/loginhttp://www.medbullets.com/user/dashboard?id=all&SubCategoryID=1http://www.medbullets.com/user/dashboard?id=all&SubCategoryID=2http://www.medbullets.com/createtesthttp://www.medbullets.com/cases/postedhttp://www.medbullets.com/video/listhttp://www.medbullets.com/post/listhttp://www.medbullets.com/group/publichttp://www.medbullets.com/user/mycolleagueshttp://www.medbullets.com/message/listhttp://www.medbullets.com/abouthttp://www.medbullets.com/feedbackhttp://www.medbullets.com/user/dashboard?id=2&specialty=biochemistryhttp://www.medbullets.com/user/dashboard?id=3&specialty=embryology1http://www.medbullets.com/user/dashboard?id=4&specialty=microbiologyhttp://www.medbullets.com/user/dashboard?id=5&specialty=immunologyhttp://www.medbullets.com/user/dashboard?id=6&specialty=pathologyhttp://www.medbullets.com/user/dashboard?id=1&specialty=statshttp://www.medbullets.com/user/dashboard?id=8&specialty=cardiovascularhttp://www.medbullets.com/user/dashboard?id=9&specialty=endocrinehttp://www.medbullets.com/user/dashboard?id=10&specialty=gastrointestinalhttp://www.medbullets.com/user/dashboard?id=11&specialty=hematologyhttp://www.medbullets.com/user/dashboard?id=18&specialty=oncologyhttp://www.medbullets.com/user/dashboard?id=12&specialty=musculoskeletal-and-connective-tissuehttp://www.medbullets.com/user/dashboard?id=13&specialty=neurologyhttp://www.medbullets.com/user/dashboard?id=14&specialty=psychiatryhttp://www.medbullets.com/user/dashboard?id=15&specialty=renalhttp://www.medbullets.com/user/dashboard?id=16&specialty=reproductivehttp://www.medbullets.com/user/dashboard?id=17&specialty=respiratoryhttp://www.medbullets.com/user/dashboard?id=23&specialty=anatomyhttp://www.medbullets.com/user/dashboard?id=7&specialty=pharmacology


2/6


thromboembolism (DVT & PE)

hypercoagulable state with risk of DVT

due to loss of antithrombin III in the urine

Presentation

Symptoms

periorbital swelling

Physical examhypertension

generalized edema(anasarca)

caused by loss of albumin / decreased oncotic pressure

Evaluation

Labs

hypoalbuminemia (serum albumin of 3.5 g/day

nephrOtic syndrome (loss of prOtien)a high protein urine is "frothy"

hyperlipidemiawith 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 diagnosisimmuno critical to establish diagnosis

Glomerulopathy Comparative Table

Immunofluorescence

pattern

Location of IC

deposition

Compliment type

activated

Electron

Microscopy

Minimal change disease Negative n/a n/a
http://pdfcrowd.com/http://pdfcrowd.com/redirect/?url=http%3a%2f%2fwww.medbullets.com%2fstep1-renal%2f15028%2fprimary-nephrotic-syndrome&id=ma-131020074103-c9788452http://pdfcrowd.com/customize/http://pdfcrowd.com/html-to-pdf-api/?ref=pdfhttp://upload.medbullets.com/question/1038/images/mcd%20podocyte%20effacement.jpg


3/6


Focal segmental Negative n/a n/a

Diffuse membranous

glomerulopathy

Granular Subepithelial n/a

Membranoproliferative

glomerulonephritis

Granular Subendothelial 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 GBMselective 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

immunizationEvaluation

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

prednisonedisease very responsive with excellent prognosis

Prognosis, Prevention, and Complications

infection

at risk for encapsulated organism infection especiallyStrep. pneumonia

Focal segmental glomerulosclerosis

Introduction

clinically similar to MCD, but occurs inadults with refractory HTN

different in that protein loss is nonspecific (unlike MCD which is selective for albumin)

usally idiopathic
http://pdfcrowd.com/http://pdfcrowd.com/redirect/?url=http%3a%2f%2fwww.medbullets.com%2fstep1-renal%2f15028%2fprimary-nephrotic-syndrome&id=ma-131020074103-c9788452http://pdfcrowd.com/customize/http://pdfcrowd.com/html-to-pdf-api/?ref=pdfhttp://upload.medbullets.com/topic/15028/images/fsgs.jpghttp://upload.medbullets.com/topic/15028/images/dmg.jpghttp://upload.medbullets.com/topic/15028/images/mpgn.jpg


4/6


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 negativeTreatment

prednisone +/- cyclophosphamide

Prognosis, Prevention and Complications

very poordue to refractory disease

Diffuse membranous glomerulopathy

Introduction

most common cause of nephrotic syndrome in adults

causes includeinfection (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" appearanceTreatment

prednisone +/- cyclophosphamide


slowly progressive disorderwith decreasing response to steroids

Membranoproliferative glomerulonephritis

Introduction
http://pdfcrowd.com/http://pdfcrowd.com/redirect/?url=http%3a%2f%2fwww.medbullets.com%2fstep1-renal%2f15028%2fprimary-nephrotic-syndrome&id=ma-131020074103-c9788452http://pdfcrowd.com/customize/http://pdfcrowd.com/html-to-pdf-api/?ref=pdf


5/6


Next Question

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

Evaluationtype I

EM shows "tram track" appearance of GBM due to splitting by mesangial growth

type II

serology shows serum C3 levels

Treatment

prednisone +/- plasmapheresis


very poor prognosis with slow progression to CRF

RATE CONTENT AVERAGE 0.0 of 0 RATINGS

Qbank (5 Questions)

Question: 1 of 5

TAG

0) (M1.RL.8) A 5-year-old female presents to your office with periorbital edema and proteinuria. Her mother reports that she "justgot 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. IgG

2. IgA

3. Albumin

4. Albumin and IgG

5. Albumin, IgG, and IgA
http://pdfcrowd.com/http://pdfcrowd.com/redirect/?url=http%3a%2f%2fwww.medbullets.com%2fstep1-renal%2f15028%2fprimary-nephrotic-syndrome&id=ma-131020074103-c9788452http://pdfcrowd.com/customize/http://pdfcrowd.com/html-to-pdf-api/?ref=pdfhttp://www.medbullets.com/video/view?id=44http://www.medbullets.com/step1-renal/15028/primary-nephrotic-syndrome


6/6


Me mbranoproliferative Glomerulonephritis

Step 1 - Nephrology- Treatment Consult - Primary Nephrotic Syndrome

Step I Lecture

11/4/2012

28 views

Subscribe status: On Off

PREFERRED RESPONSE

Recent Videos

Evidence & References Show References

Topic Comments

Please login to add comment.
http://pdfcrowd.com/http://pdfcrowd.com/redirect/?url=http%3a%2f%2fwww.medbullets.com%2fstep1-renal%2f15028%2fprimary-nephrotic-syndrome&id=ma-131020074103-c9788452http://pdfcrowd.com/customize/http://pdfcrowd.com/html-to-pdf-api/?ref=pdfhttp://www.medbullets.com/video/view?id=44http://www.medbullets.com/video/view?id=44http://www.medbullets.com/video/list?s=Nephrologyhttp://www.medbullets.com/video/list?c=Treatment+Consulthttp://www.medbullets.com/video/list?t=15028http://www.medbullets.com/login

Www Medbullets Com Step1 Renal 15028 Primary Nephrotic Syndr

Documents

Transcript of Www Medbullets Com Step1 Renal 15028 Primary Nephrotic Syndr