Document Detail


Agnathia-holoprosencephaly: a new recessive syndrome?
MedLine Citation:
PMID:  8281280     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The association between agnathia and holoprosencephaly is well documented (Pauli et al., 1981). Pauli et al. described two female sibs with agnathia and holoprosencephaly who were subsequently shown to carry an unbalanced 46,XX, der 18,t(6;18)(pter p24.1) karyotype (Pauli et al., 1983; Krassikoff and Sekhon, 1989). All other cases have been sporadic (Cohen, 1989). We present a recurrence of agnathia-holoprosencephaly in an unrelated European couple and postulate that this association may represent an autosomal recessive syndrome.
Authors:
M E Porteous; C Wright; D Smith; J Burn
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical dysmorphology     Volume:  2     ISSN:  0962-8827     ISO Abbreviation:  Clin. Dysmorphol.     Publication Date:  1993 Apr 
Date Detail:
Created Date:  1994-02-17     Completed Date:  1994-02-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9207893     Medline TA:  Clin Dysmorphol     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  161-4     Citation Subset:  IM    
Affiliation:
Department of Human Genetics, University of Newcastle-upon-Tyne, UK.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics
Abortion, Therapeutic
Chromosomes, Human, Pair 18
Chromosomes, Human, Pair 6
Female
Fetal Death
Genes, Recessive*
Holoprosencephaly / genetics*
Humans
Mandible / abnormalities*
Pregnancy
Syndrome
Translocation, Genetic

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


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