| The long-term outcome of patients with glycogen storage diseases. | |
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MedLine Citation:
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PMID: 2122109 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In this retrospective study from five centres, 139 patients over 10 years of age with glycogen storage disease types I, III, VI and IX are described. Almost half of the patients with glycogen storage disease type Ia had retarded growth and most had hyperlipidaemia. One-third of the patients had adenomas, although none of these showed malignant transformations. With increasing age the growth, liver size and hyperlipidaemia of patients with glycogen storage disease type III improve. However, there was a high incidence of myopathy and cardiomyopathy. Patients with glycogen storage disease types VI and IX had a normal growth pattern after childhood. Hepatomegaly and hypercholesterolaemia, however, were still present in half of the patients. |
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Authors:
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G P Smit; J Fernandes; J V Leonard; E E Matthews; S W Moses; M Odievre; K Ullrich |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of inherited metabolic disease Volume: 13 ISSN: 0141-8955 ISO Abbreviation: J. Inherit. Metab. Dis. Publication Date: 1990 |
Date Detail:
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Created Date: 1990-12-07 Completed Date: 1990-12-07 Revised Date: 2007-03-21 |
Medline Journal Info:
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Nlm Unique ID: 7910918 Medline TA: J Inherit Metab Dis Country: NETHERLANDS |
Other Details:
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Languages: eng Pagination: 411-8 Citation Subset: IM |
Affiliation:
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Department of Paediatrics, University of Groningen, The Netherlands. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Child Female Glycogen Storage Disease / diagnosis* Humans Incidence Longitudinal Studies Male Prognosis Prospective Studies |
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