Document Detail

Extraction of glyceric and glycolic acids from urine with tetrahydrofuran: utility in detection of primary hyperoxaluria.
MedLine Citation:
PMID:  9267307     Owner:  NLM     Status:  MEDLINE    
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.
D J Dietzen; T R Wilhite; D N Kenagy; D S Milliner; C H Smith; M Landt
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Clinical chemistry     Volume:  43     ISSN:  0009-9147     ISO Abbreviation:  Clin. Chem.     Publication Date:  1997 Aug 
Date Detail:
Created Date:  1997-09-11     Completed Date:  1997-09-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  9421549     Medline TA:  Clin Chem     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1315-20     Citation Subset:  IM    
Department of Pathology, Washington University School of Medicine, St. Louis, MO 63110, USA.
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MeSH Terms
Child, Preschool
Chromatography, Gas
Creatinine / urine
Furans* / isolation & purification,  urine*
Glyceric Acids / urine*
Glycolates / isolation & purification,  urine*
Hyperoxaluria, Primary / diagnosis*
Infant, Newborn
Oxalates / urine
Reference Values
Reg. No./Substance:
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

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