Document Detail


Another Arab patient with overlap of Váradi-Papp/Opitz trigonocephaly syndromes?
MedLine Citation:
PMID:  8986276     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a Bedouin boy with multiple congenital anomalies/mental retardation (MCA/MR). He has frontal bossing, ridged metopic suture, bilateral ptosis, right squint, depressed nasal bridge, small nose, anteverted nostrils, lobulated tongue, polydactyly of both hands, microphallus, hypoplastic scrotum, microtestes, dysgenesis of corpus callosum, and a Dandy-Walker variant. This phenotype overlaps both the Varadi-Papp syndrome (OFD VI) and Opitz trigonocephaly (C syndrome). This phenotypic overlap is discussed in light of the concept of splitting and lumping in genetic diseases.
Authors:
M A Sabry; Q al Saleh; S Farah; D Obenbergerova; S Simeonov; S A al Awadi; T I Farag
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  68     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1997 Jan 
Date Detail:
Created Date:  1997-03-18     Completed Date:  1997-03-18     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7708900     Medline TA:  Am J Med Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  54-7     Citation Subset:  IM    
Affiliation:
Medical Genetics Centre, Maternity Hospital, Kuwait.
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MeSH Terms
Descriptor/Qualifier:
Arabs
Craniofacial Abnormalities / pathology*
Fingers / abnormalities*
Humans
Infant
Male
Phenotype
Tomography, X-Ray Computed

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