| Natural history of hepatocellular adenoma formation in glycogen storage disease type I. | |
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MedLine Citation:
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PMID: 21481415 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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David Q Wang; Laurie M Fiske; Caroline T Carreras; David A Weinstein |
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Publication Detail:
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Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2011-04-09 |
Journal Detail:
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Title: The Journal of pediatrics Volume: 159 ISSN: 1097-6833 ISO Abbreviation: J. Pediatr. Publication Date: 2011 Sep |
Date Detail:
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Created Date: 2011-08-17 Completed Date: 2011-11-08 Revised Date: 2012-09-28 |
Medline Journal Info:
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Nlm Unique ID: 0375410 Medline TA: J Pediatr Country: United States |
Other Details:
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Languages: eng Pagination: 442-6 Citation Subset: AIM; IM |
Copyright Information:
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Copyright © 2011 Mosby, Inc. All rights reserved. |
Affiliation:
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Glycogen Storage Disease Program and Division of Pediatric Endocrinology, Department of Pediatrics, University of Florida, Gainesville, FL 32610-0296, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adenoma, Liver Cell
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etiology*,
metabolism Adolescent Adult Body Height Body Mass Index Case-Control Studies Child Child, Preschool Disease Progression Female Follow-Up Studies Glycogen Storage Disease Type I / complications*, metabolism Humans Liver Neoplasms / etiology*, metabolism Male Retrospective Studies Triglycerides / blood |
| Grant Support | |
ID/Acronym/Agency:
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1UL1RR029890/RR/NCRR NIH HHS; UL1 RR029890-01/RR/NCRR NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Triglycerides |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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