Document Detail


Respiratory insufficiency in adult-type acid maltase deficiency.
MedLine Citation:
PMID:  8488406     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
AMD is a glycogen storage disease that affects all age groups. In both childhood and adult forms, the classic clinical picture is that of a progressive myopathy that may resemble polymyositis or limb girdle muscular dystrophy. Respiratory muscle involvement is common, may occur early in the course of the disease, and is the most frequent cause of mortality from AMD. These findings underscore the need to consider AMD in the differential diagnosis of unexplained respiratory insufficiency. We have described the case of one patient in whom respiratory failure was the initial symptom and two other cases in which the patient had progressive muscular weakness and subsequent respiratory failure. A review of the literature revealed that respiratory muscle weakness has been reported in 21 of 43 cases of AMD in adults. In 10 cases respiratory insufficiency was preceded by only mild or moderate weakness.
Authors:
N A Moufarrej; T E Bertorini
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Southern medical journal     Volume:  86     ISSN:  0038-4348     ISO Abbreviation:  South. Med. J.     Publication Date:  1993 May 
Date Detail:
Created Date:  1993-06-08     Completed Date:  1993-06-08     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0404522     Medline TA:  South Med J     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  560-7     Citation Subset:  AIM; IM    
Affiliation:
University of Tennessee, Departments of Neurology and Pathology, Memphis 38163.
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MeSH Terms
Descriptor/Qualifier:
Adult
Female
Glucan 1,4-alpha-Glucosidase / deficiency
Glycogen Storage Disease Type II / complications*,  pathology
Humans
Muscles / ultrastructure
Respiratory Insufficiency / etiology*
alpha-Glucosidases
Chemical
Reg. No./Substance:
EC 3.2.1.20/alpha-Glucosidases; EC 3.2.1.3/Glucan 1,4-alpha-Glucosidase

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


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