Document Detail


Efficacy of cornstarch therapy in type III glycogen-storage disease.
MedLine Citation:
PMID:  2403059     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Type III glycogen-storage disease (GSD-III), due to decreased activity of the glycogen debranching enzyme amylo-1,6 glucosidase, may cause hepatic dysfunction, growth failure, and myopathy. The prevention of hypoglycemia by nocturnal intragastric formula infusion has been shown to enhance growth and improve the metabolic abnormalities associated with GSD-III. Cornstarch therapy was effective in preventing hypoglycemia in a few patients with GSD-III who were previously treated with nocturnal enteral formula infusion, but oral cornstarch had not been evaluated as an initial treatment. We studied three patients with GSD-III who exhibited growth failure, elevated serum aminotransferase concentrations, and asymptomatic hypoglycemia. Cornstarch therapy was associated with maintenance of normoglycemia, increased growth velocity, and decreased serum aminotransferase concentrations in all patients. Our experience suggests that cornstarch therapy can be effective as an initial treatment for patients with GSD-III.
Authors:
D A Gremse; J C Bucuvalas; W F Balistreri
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The American journal of clinical nutrition     Volume:  52     ISSN:  0002-9165     ISO Abbreviation:  Am. J. Clin. Nutr.     Publication Date:  1990 Oct 
Date Detail:
Created Date:  1990-10-19     Completed Date:  1990-10-19     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0376027     Medline TA:  Am J Clin Nutr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  671-4     Citation Subset:  AIM; IM    
Affiliation:
Division of Pediatric Gastroenterology and Nutrition, Children's Hospital Research Foundation, Cincinnati.
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MeSH Terms
Descriptor/Qualifier:
Blood Glucose / analysis
Child
Child Development
Child, Preschool
Female
Glycogen Storage Disease / drug therapy*
Glycogen Storage Disease Type III / blood,  drug therapy*,  physiopathology
Humans
Liver / pathology
Male
Starch / therapeutic use*
Transaminases / blood
Weight Gain
Zea mays*
Chemical
Reg. No./Substance:
0/Blood Glucose; 9005-25-8/Starch; EC 2.6.1.-/Transaminases

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


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