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Wilson's Disease: Experience at a Tertiary Care Hospital.
MedLine Citation:
PMID:  23823966     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Wilson's disease (WD) is a rare autosomal recessive disorder of copper metabolism. Data regarding WD is not available from Pakistan. A cross-sectional study was conducted at The Aga Khan University Hospital, Karachi, and all patients admitted with primary and secondary diagnosis of Wilson's disease were added. A total of 47 patients were seen; 68% (n = 32) were male. The mean age was 26.6 ± 9.97 years. Most of the patients presented with hepatic, (n = 22, 46.8%), neurological, (n = 17, 36.2%) and psychiatric (n = 8, 17%) symptoms. Mean ceruloplasmin level was 0.17 ± 0.13 g/dl; it was < 0.25 g/dl in 39 (86.6%) patients. Serum copper (Cu) was reduced in 32 (68.1%) patients and 24-hr-urinary Cu was raised in 22 (47.6%) patients. Slit lamp examination for Kayser-Fleischer (KF) rings was done on 15 (31.9%) patients and 9 (60%) of them had KF rings. Mean serum aspartate transaminase (AST) / alanine transaminases (ALT) ratio was 1.92 and median alkaline phosphatase / total bilirubin ratio was 79.30 (IQR 35.05; 166.50).
Authors:
Om Parkash; Adil Ayub; Wasim Jafri; Syed Hasnain Alishah; Saeed Hamid
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of the College of Physicians and Surgeons--Pakistan : JCPSP     Volume:  23     ISSN:  1681-7168     ISO Abbreviation:  J Coll Physicians Surg Pak     Publication Date:  2013 Jul 
Date Detail:
Created Date:  2013-07-04     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9606447     Medline TA:  J Coll Physicians Surg Pak     Country:  Pakistan    
Other Details:
Languages:  eng     Pagination:  525-6     Citation Subset:  IM    
Affiliation:
Department of Medicine, The Aga Khan University, Karachi.
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