Document Detail

Urinary excretion of lactate, 2-oxoglutarate, citrate, and glycerol in patients with glycogenosis type I.
MedLine Citation:
PMID:  3470705     Owner:  NLM     Status:  MEDLINE    
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.
J Fernandes; R Berger
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Pediatric research     Volume:  21     ISSN:  0031-3998     ISO Abbreviation:  Pediatr. Res.     Publication Date:  1987 Mar 
Date Detail:
Created Date:  1987-05-18     Completed Date:  1987-05-18     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0100714     Medline TA:  Pediatr Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  279-82     Citation Subset:  IM    
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MeSH Terms
Body Height
Child, Preschool
Citrates / urine*
Citric Acid
Creatinine / urine
Glycerol / urine*
Glycogen Storage Disease Type I / complications,  pathology,  urine*
Growth Disorders / etiology
Ketoglutaric Acids / urine*
Lactates / urine*
Lactic Acid
Reference Values
Reg. No./Substance:
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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