Document Detail


DeSanctis-Cacchione syndrome. A case report with autopsy findings.
MedLine Citation:
PMID:  931396     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
One of the first patients studied by Cleaver on the DNA repair defect to ultraviolet light damage in xeroderma pigmentosum has been subjected to autopsy examination. This patient had the DeSanctis-Cacchione syndrome (microcephaly, dwarfism, mental deficiency, and choreoathetosis). Her autopsy findings were similar to those of the other three patients with this syndrome, emphasizing olivopontocerebellar atrophy, who underwent autopsy. The patient apparently could not tolerate psoralens because of increased incidence of carcinoma formation.
Authors:
W B Reed; G I Sugarman; R A Mathis
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Archives of dermatology     Volume:  113     ISSN:  0003-987X     ISO Abbreviation:  Arch Dermatol     Publication Date:  1977 Nov 
Date Detail:
Created Date:  1978-01-27     Completed Date:  1978-01-27     Revised Date:  2008-03-17    
Medline Journal Info:
Nlm Unique ID:  0372433     Medline TA:  Arch Dermatol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1561-3     Citation Subset:  AIM; IM    
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Autopsy
Brain / abnormalities
Child, Preschool
DNA Repair
Humans
Mental Retardation / complications
Syndrome
Xeroderma Pigmentosum / genetics,  pathology*

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


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