Document Detail

Natural history of hepatocellular adenoma formation in glycogen storage disease type I.
MedLine Citation:
PMID:  21481415     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: To characterize the natural history and factors related to hepatocellular adenoma (HCA) development in glycogen storage disease type Ia (GSD Ia).
STUDY DESIGN: Retrospective chart review was performed for 117 patients with GSD Ia. Kaplan-Meier analysis of HCA progression among two groups of patients with GSD Ia (5-year mean triglyceride concentration ≤ 500 mg/dL and >500 mg/dL); analysis of serum triglyceride concentration, body mass index SDS, and height SDS between cases at time of HCA diagnosis and age- and sex-matched control subjects.
RESULTS: Logrank analysis of Kaplan-Meier survival curve demonstrated a significant difference in progression to HCA between the 5-year mean triglyceride groups (P = .008). No significant difference was detected in progression to adenoma event between sexes. Serum triglyceride concentration was significantly different at time of diagnosis of adenoma (737 ± 422 mg/dL) compared with control subjects (335 ± 195 mg/dL) (P = .009). Differences in height SDS (P = .051) and body mass index SDS (P = .066) approached significance in our case-control analysis.
CONCLUSION: Metabolic control may be related to HCA formation in patients with GSD Ia. Optimizing metabolic control remains critical, and further studies are warranted to understand the pathogenesis of adenoma development.
David Q Wang; Laurie M Fiske; Caroline T Carreras; David A Weinstein
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2011-04-09
Journal Detail:
Title:  The Journal of pediatrics     Volume:  159     ISSN:  1097-6833     ISO Abbreviation:  J. Pediatr.     Publication Date:  2011 Sep 
Date Detail:
Created Date:  2011-08-17     Completed Date:  2011-11-08     Revised Date:  2013-06-30    
Medline Journal Info:
Nlm Unique ID:  0375410     Medline TA:  J Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  442-6     Citation Subset:  AIM; IM    
Copyright Information:
Copyright © 2011 Mosby, Inc. All rights reserved.
Glycogen Storage Disease Program and Division of Pediatric Endocrinology, Department of Pediatrics, University of Florida, Gainesville, FL 32610-0296, USA.
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MeSH Terms
Adenoma, Liver Cell / etiology*,  metabolism
Body Height
Body Mass Index
Case-Control Studies
Child, Preschool
Disease Progression
Follow-Up Studies
Glycogen Storage Disease Type I / complications*,  metabolism
Liver Neoplasms / etiology*,  metabolism
Retrospective Studies
Triglycerides / blood
Grant Support
Reg. No./Substance:

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