| Urinary excretion of lactate, 2-oxoglutarate, citrate, and glycerol in patients with glycogenosis type I. | |
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MedLine Citation:
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PMID: 3470705 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Growth retardation and lactic aciduria are well-known abnormalities in patients with a deficiency of either glucose-6-phosphatase or glucose-6-phosphate translocase. In 19 patients with glucose-6-phosphatase and two patients with glucose-6-phosphate translocase, growth retardation was quantified by calculating the height standard deviation score. The urinary excretion of lactate and some other metabolites was quantified by calculating the lactate/creatinine, 2-oxoglutarate/creatinine, citrate/creatinine, and glycerol/creatinine ratios in urine. Significant correlations were found between the lactate/creatinine ratio, the 2-oxoglutarate/creatinine ratio, and height SD score. Urinary lactate appeared to respond promptly to changes of the diet, while urinary 2-oxoglutarate responded only slowly, as did growth itself. The citrate/creatinine ratio and the glycerol/creatinine ratio were within the normal range and varied little. It was concluded that the urinary 2-oxoglutarate excretion primarily reflects the severity of the disease as expressed in stunted growth. Thus, while urinary lactate levels are more suitable for monitoring the diet, urinary 2-oxoglutarate levels can be used as an indication for intensive treatment with hyperalimentation. |
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Authors:
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J Fernandes; R Berger |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Pediatric research Volume: 21 ISSN: 0031-3998 ISO Abbreviation: Pediatr. Res. Publication Date: 1987 Mar |
Date Detail:
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Created Date: 1987-05-18 Completed Date: 1987-05-18 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0100714 Medline TA: Pediatr Res Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 279-82 Citation Subset: IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Body Height Child Child, Preschool Citrates / urine* Citric Acid Creatinine / urine Glycerol / urine* Glycogen Storage Disease Type I / complications, pathology, urine* Growth Disorders / etiology Humans Ketoglutaric Acids / urine* Lactates / urine* Lactic Acid Reference Values |
| Chemical | |
Reg. No./Substance:
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0/Citrates; 0/Ketoglutaric Acids; 0/Lactates; 328-50-7/alpha-ketoglutaric acid; 50-21-5/Lactic Acid; 56-81-5/Glycerol; 60-27-5/Creatinine; 77-92-9/Citric Acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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