HOME HELP CONTACT US SUBSCRIPTIONS CME ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Take the CME quiz: Vol. 30 No. 3, March 2009 A correction has been published Rapid Responses: Submit a response Rapid Responses: View responses Alert me when this article is cited Alert me when Rapid Responses are posted Alert me if a correction is posted Citation Map Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet Download to citation manager Request Permissions Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Gordillo, R. Articles by Spitzer, A. Search for Related Content PubMed PubMed Citation Articles by Gordillo, R. Articles by Spitzer, A. Related Collections Renal Disorders Social Bookmarking                         What's this?

(Pediatrics in Review. 2009;30:94-105.)
© 2009 American Academy of Pediatrics

The Nephrotic Syndrome

Roberto Gordillo, MD^*
Adrian Spitzer, MD

^* Postdoctoral Fellow
Professor of Pediatrics, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Division of Pediatric Nephrology, Bronx, NY

Abbreviations: ACE: angiotensin-converting enzyme • BSA: body surface area • CNF: congenital nephrotic syndrome of the Finnish type • CNS: congenital nephrotic syndrome • FSGS: focal segmental glomerulosclerosis • HDL: high-density lipoprotein • Ig: immunoglobulin • IL: interleukin • ISKDC: International Study of Kidney Disease in Children • LDL: low-density lipoprotein • MCNS: minimal-change nephrotic syndrome • MN: membranous nephropathy • MPGN: mesangioproliferative glomerulonephritis • RAA: renin-angiotensin-aldosterone • SLE: systemic lupus erythematosus • UPr/Cr: urine protein/creatinine ratio • VLDL: very low-density lipoprotein

The first 300 words of the full text of this article appear below.

	Objectives

 
After completing this article, readers should be able to:

1. Explain the mechanism and the consequences of proteinuria.
   
2. Make a presumptive diagnosis of minimal-change nephrotic syndrome.
   
3. Interpret the signs associated with steroid resistance.
   
4. Ascertain the timing and the indications for a kidney biopsy.
   
5. Gauge the indications for referral to a pediatric nephrologist.
   
6. Determine the adequacy of the therapy.
   
7. Predict the disease course.
   

	Introduction

 
The word "nephrosis" was introduced in the medical literature at the beginning of the 20th century in an attempt to distinguish diseases of the kidney characterized by exudation and proliferation from those characterized by inflammation (nephritis). As it became apparent that this is not a single disease, not even a group of related diseases, the term "nephrosis" was supplanted by "nephrotic syndrome." The clinical features that characterize the nephrotic syndrome result from alterations of the glomerular capillary wall and consist of heavy proteinuria and hypoalbuminemia, often associated with edema and generalized hyperlipidemia.

	Pathophysiology

 
     Proteinuria and Hypoalbuminemia
Proteinuria is the result of alterations in the integrity of the glomerular filtration barrier. This barrier is composed of three layers in series: the fenestrated endothelium, the glomerular basement membrane, and the visceral glomerular epithelium, comprised of podocytes and their slit diaphragms. Podocyte is the name of the epithelial cell, and foot process is the segment of the cell that extends into the urinary space. (In the nephrotic syndrome, there is effacement of the foot process, but the rest of the cell usually is preserved.)

Endothelial cells have numerous openings that are 70 to 100 nm in diameter, called fenestrae, which form a physical barrier for passage of macromolecules from plasma into the renal tubule. Electron microscopic studies led to the identification of negatively charged particles (heparan sulfate proteoglycans) in the glomerular basement membrane, which preclude the passage of anionic macromolecules, such as albumin. Removal of these . . . [Full Text of this Article]

CiteULike    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

Rapid Responses: