| Extraction of glyceric and glycolic acids from urine with tetrahydrofuran: utility in detection of primary hyperoxaluria. | |
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MedLine Citation:
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PMID: 9267307 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Primary hyperoxaluria (PH) is an autosomal recessive metabolic abnormality characterized by excessive oxalate excretion leading to nephrocalcinosis and progressive renal dysfunction. Type I primary hyperoxaluria (PH I) results from a deficiency of alanine:glyoxylate aminotransferase, whereas type II disease has been traced to a deficiency of D-glycerate dehydrogenase. The two syndromes are often distinguished on the basis of organic acids that are coexcreted with oxalate: glycolate and L-glycerate in type I and type II disease, respectively. Routine organic acid analysis with diethyl ether extraction followed by gas chromatographic analysis failed to detect normal and increased concentrations of these diagnostic metabolites. Subsequent extraction of urine with tetrahydrofuran (THF), however, extracted 75% of added glycerate, 42% of added glycolate, and 75% of added ethylphosphonic acid (internal calibrator). THF extraction was analytically sensitive enough to allow determination of normal excretion of glycolate (14-72 micrograms/mg creatinine) and glycerate (0-5 years, 12-177 micrograms/mg creatinine and > 5 years, 19-115 micrograms/mg creatinine). Four of five patients with PH I and both patients with type II disease were correctly identified. Thus, THF extraction is a convenient adjunct to routine organic acid analysis and facilitates the detection of PH. |
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Authors:
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D J Dietzen; T R Wilhite; D N Kenagy; D S Milliner; C H Smith; M Landt |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Clinical chemistry Volume: 43 ISSN: 0009-9147 ISO Abbreviation: Clin. Chem. Publication Date: 1997 Aug |
Date Detail:
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Created Date: 1997-09-11 Completed Date: 1997-09-11 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 9421549 Medline TA: Clin Chem Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1315-20 Citation Subset: IM |
Affiliation:
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Department of Pathology, Washington University School of Medicine, St. Louis, MO 63110, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Child Child, Preschool Chromatography, Gas Creatinine / urine Furans* / isolation & purification, urine* Glyceric Acids / urine* Glycolates / isolation & purification, urine* Humans Hyperoxaluria, Primary / diagnosis* Infant Infant, Newborn Oxalates / urine Reference Values |
| Chemical | |
Reg. No./Substance:
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0/Furans; 0/Glyceric Acids; 0/Glycolates; 0/Oxalates; 109-99-9/tetrahydrofuran; 473-81-4/glyceric acid; 60-27-5/Creatinine; 79-14-1/glycolic acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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