Document Detail


Mixed gonadal dysgenesis and Denys-Drash syndrome: urologists should screen for nephrotic syndrome.
MedLine Citation:
PMID:  18163932     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVE: We present a child with Denys-Drash syndrome recognized after surgery for mixed gonadal dysgenesis, and discuss screening procedures the urologist should consider in similar circumstances. CASE REPORT: A 1-year-old child with XY gonadal dysgenesis underwent genital reconstruction. The postoperative period was complicated by incisional drainage, which led to the recognition of a nephrotic syndrome. Molecular analysis of the WT-1 gene confirmed a mutation associated with the Denys-Drash syndrome. CONCLUSION: The Denys-Drash syndrome should be suspected in children with XY gonadal dysgenesis. The presence of urine protein should be sought in such children, and if present, consultation with genetic and nephrology specialists is warranted.
Authors:
Oleg Shapiro; Thomas R Welch; Maryellen Sheridan; Susan Stred; Jyoti Upadhyay
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  The Canadian journal of urology     Volume:  14     ISSN:  1195-9479     ISO Abbreviation:  Can J Urol     Publication Date:  2007 Dec 
Date Detail:
Created Date:  2007-12-31     Completed Date:  2008-04-11     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9515842     Medline TA:  Can J Urol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  3767-9     Citation Subset:  IM    
Affiliation:
State University of New York, Upstate Medical University, Department of Urology, Syracuse, New York, USA.
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MeSH Terms
Descriptor/Qualifier:
Denys-Drash Syndrome / complications*
Gonadal Dysgenesis, Mixed / complications*
Humans
Infant
Male
Nephrotic Syndrome / complications*,  diagnosis*
Urology

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