AJCN Tufts Nutrition Symposium, Boston Sept 24-26
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
QUICK SEARCH:   [advanced]


     

This Article
Right arrow Full Text (PDF)
Right arrow Purchase Article
Right arrow View Shopping Cart
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Gremse, D. A.
Right arrow Articles by Balistreri, W. F.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Gremse, D. A.
Right arrow Articles by Balistreri, W. F.
Agricola
Right arrow Articles by Gremse, D. A.
Right arrow Articles by Balistreri, W. F.

American Journal of Clinical Nutrition, Vol 52, 671-674, Copyright © 1990 by The American Society for Clinical Nutrition, Inc

ORIGINAL RESEARCH COMMUNICATIONS

Efficacy of cornstarch therapy in type III glycogen-storage disease

DA Gremse, JC Bucuvalas and WF Balistreri
Division of Pediatric Gastroenterology and Nutrition, Children's Hospital Research Foundation, Cincinnati.

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.




HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Copyright © 1990 by The American Society for Nutrition