Document Detail

Syndromal and nonsyndromal primary trigonocephaly: analysis of a series of 237 patients.
MedLine Citation:
PMID:  9450889     Owner:  NLM     Status:  MEDLINE    
From a series of 1,713 patients with craniosynostosis hospitalized between 1976 and 1996, 237 propositi with metopic synostosis were analyzed. The prevalence of metopic synostosis was estimated in the order of 1 in 15,000 children. Family information was obtained from 184 propositi from 179 families. The male-to-female ratio was 3.3:1. There was no maternal or paternal age effect. A family history was obtained in 10 of the 179 families, giving a 5.6% figure of familial cases. The frequency of twinning was 7.8% with two concordances for metopic synostosis in two monozygotic twin pairs. The male-to-female ratio, the twinning frequency, and the proportion of familial cases in trigonocephaly are very similar to those observed in scaphocephaly, which also involves the longitudinal sutural system. Fetal exposure to valproic acid was noticed in eight cases. The series was divided into two groups: nonsyndromal trigonocephaly (n = 184) and trigonocephaly associated with other malformations (n = 53). The second group included 13 cases of well-delineated syndromes and 40 cases of trigonocephaly associated with one or more malformations, but without any known syndrome, that could be undelineated syndromes. These groups differed significantly in their mental prognosis.
E Lajeunie; M Le Merrer; D Marchac; D Renier
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  75     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1998 Jan 
Date Detail:
Created Date:  1998-03-24     Completed Date:  1998-03-24     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:  211-5     Citation Subset:  IM    
U393 INSERM, Paris, France.
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MeSH Terms
Birth Weight
Child, Preschool
Chromosome Aberrations / epidemiology
Chromosome Disorders
Craniosynostoses / chemically induced,  epidemiology*,  genetics
France / epidemiology
Infant, Newborn
Valproic Acid / adverse effects
Reg. No./Substance:
99-66-1/Valproic Acid

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

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