Document Detail


Prenatal and postnatal presentation of severe achondroplasia with developmental delay and acanthosis nigricans (SADDAN) due to the FGFR3 Lys650Met mutation.
MedLine Citation:
PMID:  18076102     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We present prenatal and postnatal features of a patient with severe achondroplasia with developmental delay and acanthosis nigricans (SADDAN). Mutation analysis confirmed the clinical diagnosis by detecting the FGFR3 Lys650Met mutation. This case, one of only six with molecular analysis reported in the literature, confirms the severe morbidity and adds to the reports with early mortality associated with SADDAN. Clinical-radiological characteristics of all reported cases of SADDAN are reviewed and discussed.
Authors:
Andreas Zankl; George Elakis; Rachel D Susman; Garry Inglis; Glenn Gardener; Michael F Buckley; Tony Roscioli
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  146A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2008 Jan 
Date Detail:
Created Date:  2007-12-26     Completed Date:  2008-02-25     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  212-8     Citation Subset:  IM    
Copyright Information:
(c) 2007 Wiley-Liss, Inc.
Affiliation:
Genetic Health Queensland, Royal Children's Hospital, Brisbane, Australia. andreas.zankl@gmail.com
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MeSH Terms
Descriptor/Qualifier:
Acanthosis Nigricans / diagnosis*,  genetics
Achondroplasia / diagnosis*,  genetics
Amino Acid Substitution*
Developmental Disabilities / diagnosis,  genetics
Female
Fetus / abnormalities
Humans
Infant, Newborn
Lysine / genetics
Male
Methionine / genetics
Mutation, Missense
Pregnancy
Receptor, Fibroblast Growth Factor, Type 3 / genetics*
Ultrasonography, Prenatal
Chemical
Reg. No./Substance:
56-87-1/Lysine; 63-68-3/Methionine; EC 2.7.1.112/FGFR3 protein, human; EC 2.7.10.1/Receptor, Fibroblast Growth Factor, Type 3

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