Document Detail


A novel mutation in the renal V2 receptor gene in a boy with trisomy 21.
MedLine Citation:
PMID:  15052464     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe for the first time an infant with Down syndrome and congenital nephrogenic diabetes insipidus (NDI). The 11-day-old Japanese boy was admitted with failure to thrive and fever. Polyuria (3,000-3,500 ml/m(2) per day), low urine specific gravity (1.001-1.002), and high plasma arginine vasopressin (AVP) (18.2 pg/ml) suggested NDI. Gene analysis confirmed the diagnosis of congenital NDI due to a novel mutation of the V2 receptor gene (L309P). He also had symptoms of Down syndrome and karyotype analysis of the peripheral lymphocytes revealed trisomy 21. The relationship between pyelectasis and a risk of Down syndrome is discussed.
Authors:
Yasuko Fujisawa; Takeshi Miyamoto; Kyo Furuhashi; Shinichiro Sano; Yuichi Nakagawa; Takehiko Ohzeki
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2004-03-30
Journal Detail:
Title:  Pediatric nephrology (Berlin, Germany)     Volume:  19     ISSN:  0931-041X     ISO Abbreviation:  Pediatr. Nephrol.     Publication Date:  2004 Jun 
Date Detail:
Created Date:  2004-05-10     Completed Date:  2004-12-15     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8708728     Medline TA:  Pediatr Nephrol     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  609-11     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Hamamatsu University School of Medicine, 431-3192 Hamamatsu, Japan.
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MeSH Terms
Descriptor/Qualifier:
Diabetes Insipidus, Nephrogenic / complications*,  congenital*
Down Syndrome / complications*
Humans
Infant, Newborn
Kidney / pathology
Male
Mutation
Pedigree
Polymerase Chain Reaction
Polyuria / etiology
Receptors, Vasopressin / genetics*
Chemical
Reg. No./Substance:
0/Receptors, Vasopressin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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