Document Detail


Multiple congenital anomalies/mental retardation syndrome with multiple circumferential skin creases: a new syndrome?
MedLine Citation:
PMID:  19182162     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a combination of multiple congenital anomalies, a severe psychomotor retardation and seizures in a 9-year-old Tunisian boy with circumferential ringed skin creases. He had symmetrical circumferential skin creases on arms, legs, and penis. Craniofacial anomalies included: an elongated face, tight forehead, hypertelorism, bilateral epicanthic folds, upslanting palpebral fissures, microphthalmia, convergent strabismus, wide nasal bridge, aberrant teeth, dental caries, and low-set posteriorly rotated ears with overfolded thick helices. He had also ureterocele, hypospadias, and others anomalies. The magnetic resonance imaging of the brain showed hypoplastic vermis, hypoplastic corpus callosum, and dilatation of ventricles. Chromosomal analysis revealed a normal male karyotype with 46,XY. Skin biopsy was normal. To the best of our knowledge, this combination of anomalies has not been reported and this case may be a unique syndrome.
Authors:
Faten Tinsa; Khaoula Aissa; Mounira Meddeb; Dorra Bousnina; Khadija Boussetta; Souad Bousnina
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of child neurology     Volume:  24     ISSN:  1708-8283     ISO Abbreviation:  J. Child Neurol.     Publication Date:  2009 Feb 
Date Detail:
Created Date:  2009-02-02     Completed Date:  2009-05-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  224-7     Citation Subset:  IM    
Affiliation:
Department of Pediatrics of the Children's Hospital of Tunis, Tunis, Tunisia. faten.tinsa@hotmail.fr
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple*
Brain / pathology
Child
Craniofacial Abnormalities / pathology
Humans
Karyotyping
Magnetic Resonance Imaging
Male
Mental Retardation*
Skin / pathology
Skin Abnormalities* / pathology
Syndrome

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


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