| Nephrotic syndrome in childhood. | |
| | |
MedLine Citation:
|
PMID: 12944064 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Childhood nephrotic syndromes are most commonly caused by one of two idiopathic diseases: minimal-change nephrotic syndrome (MCNS) and focal segmental glomerulosclerosis (FSGS). A third distinct type, membranous nephropathy, is rare in children. Other causes of isolated nephrotic syndrome can be subdivided into two major categories: rare genetic disorders, and secondary diseases associated with drugs, infections, or neoplasia. The cause of idiopathic nephrotic syndrome remains unknown, but evidence suggests it may be a primary T-cell disorder that leads to glomerular podocyte dysfunction. Genetic studies in children with familial nephrotic syndrome have identified mutations in genes that encode important podocyte proteins. Patients with idiopathic nephrotic syndrome are initially treated with corticosteroids. Steroid-responsiveness is of greater prognostic use than renal histology. Several second-line drugs, including alkylating agents, ciclosporin, and levamisole, may be effective for complicated and steroid-unresponsive MCNS and FSGS patients. Nephrotic syndrome is associated with several medical complications, the most severe and potentially fatal being bacterial infections and thromboembolism. Idiopathic nephrotic syndrome is a chronic relapsing disease for most steroid-responsive patients, whereas most children with refractory FSGS ultimately develop end-stage renal disease. Research is being done to further elucidate the disorder's molecular pathogenesis, identify new prognostic indicators, and to develop better approaches to treatment. |
| | |
Authors:
|
Allison A Eddy; Jordan M Symons |
Related Documents
:
|
7810534 - Arginine vasopressin gene expression in rats with puromycin-induced nephrotic syndrome. 8116694 - Therapy of idiopathic nephrotic syndrome in adults. a conservative or aggressive therap... 10085454 - Nephrotic syndrome with mesangial proliferative glomerulonephritis induced by multiple ... 15879034 - Strongyloides hyperinfection and hypogammaglobulinemia. 18249054 - Mild smith-lemli-opitz syndrome: further delineation of 5 polish cases and review of th... 14992254 - Jejunal hemorrhage syndrome in 2 canadian beef cows. |
Publication Detail:
|
Type: Journal Article; Research Support, U.S. Gov't, P.H.S.; Review |
Journal Detail:
|
Title: Lancet Volume: 362 ISSN: 1474-547X ISO Abbreviation: Lancet Publication Date: 2003 Aug |
Date Detail:
|
Created Date: 2003-08-28 Completed Date: 2003-10-09 Revised Date: 2007-11-14 |
Medline Journal Info:
|
Nlm Unique ID: 2985213R Medline TA: Lancet Country: England |
Other Details:
|
Languages: eng Pagination: 629-39 Citation Subset: AIM; IM |
Affiliation:
|
Department of Pediatrics, University of Washington, Children's Hospital and Regional Medical Center, Seattle, WA 98105, USA. allison.eddy@seattlechildrens.org |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adrenal Cortex Hormones
/
therapeutic use Age Factors Child Child, Preschool Cyclosporine / therapeutic use Humans Infant Infant, Newborn Nephrotic Syndrome* / diagnosis, drug therapy |
| Grant Support | |
ID/Acronym/Agency:
|
DK54500/DK/NIDDK NIH HHS |
| Chemical | |
Reg. No./Substance:
|
0/Adrenal Cortex Hormones; 59865-13-3/Cyclosporine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Effect of losartan on sudden cardiac death in people with diabetes: data from the LIFE study.
Next Document: Assessment and management of cancer-related fatigue in adults.