Document Detail


The failure of parenteral copper therapy in Menkes Kinky Hair syndrome.
MedLine Citation:
PMID:  6468439     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
In the untreated infant with Menkes Kinky Hair Syndrome, copper concentrations in brain and liver are deficient, while excessive copper accumulates in other tissues. The observed serum ceruloplasmin response after parenteral copper administration is suggestive of an impairment in the incorporation of copper into this metalloprotein. These findings, together with increased urinary copper excretion and the absence of clinical improvement, are compatible with a generalized defect in copper metabolism, transport or storage. The excessive accumulation of copper in many tissues illustrates the potential danger of parenteral copper therapy.
Authors:
A D Garnica
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  European journal of pediatrics     Volume:  142     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  1984 Jun 
Date Detail:
Created Date:  1984-10-09     Completed Date:  1984-10-09     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  98-102     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Brain Diseases, Metabolic / drug therapy*
Ceruloplasmin / metabolism
Copper / administration & dosage,  adverse effects*,  metabolism
Humans
Infant
Infant, Newborn
Liver / metabolism
Male
Menkes Kinky Hair Syndrome / drug therapy*,  metabolism
Grant Support
ID/Acronym/Agency:
3 R01 NS 14927/NS/NINDS NIH HHS; 5 KO4 NS 00409/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
7440-50-8/Copper; EC 1.16.3.1/Ceruloplasmin

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


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