Literature DB >> 6839506

The determination of pipecolic acid: method and results of hospital survey.

J Hutzler, J Dancis.   

Abstract

A method is described for the quantitative assay of pipecolic acid in biological fluids using column chromatography, with acid ninhydrin for color development. The technique permits measurement of plasma pipecolic acid in normal individuals with blood samples of 1 ml. The mean value in 29 pediatric patients, aged 4 months to 17 years, hospitalized with a variety of diagnoses was 2.1 mumol/1 +/- 1.6. Newborn infants had slightly elevated plasma levels with a mean of 12 mumol/1 +/- 5.6. Among the diagnostic categories, patients with liver disease were distinctive in commonly having levels at the upper limits of normal. In some cases, the levels were exceedingly high, reaching 78 mumol/1 in one fatal case.

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Year:  1983        PMID: 6839506     DOI: 10.1016/0009-8981(83)90057-8

Source DB:  PubMed          Journal:  Clin Chim Acta        ISSN: 0009-8981            Impact factor:   3.786


  5 in total

1.  Non-invasive urinary metabolomic profiles discriminate biliary atresia from infantile hepatitis syndrome.

Authors:  Wei-Wei Li; Yan Yang; Qi-Gang Dai; Li-Li Lin; Tong Xie; Li-Li He; Jia-Lei Tao; Jin-Jun Shan; Shou-Chuan Wang
Journal:  Metabolomics       Date:  2018-06-21       Impact factor: 4.290

2.  The significance of hyperpipecolatemia in Zellweger syndrome.

Authors:  J Dancis; J Hutzler
Journal:  Am J Hum Genet       Date:  1986-05       Impact factor: 11.025

3.  Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical and morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy.

Authors:  J Vamecq; J P Draye; F Van Hoof; J P Misson; P Evrard; G Verellen; H J Eyssen; J Van Eldere; R B Schutgens; R J Wanders
Journal:  Am J Pathol       Date:  1986-12       Impact factor: 4.307

4.  Pipecolic acid levels in serum and urine from neonates and normal infants: comparison with values reported in Zellweger syndrome.

Authors:  L Govaerts; F Trijbels; L Monnens; A van Raay-Selten
Journal:  J Inherit Metab Dis       Date:  1985       Impact factor: 4.982

5.  Laboratory diagnosis of disorders of peroxisomal biogenesis and function: a technical standard of the American College of Medical Genetics and Genomics (ACMG).

Authors:  Irene De Biase; Silvia Tortorelli; Lisa Kratz; Steven J Steinberg; Kristina Cusmano-Ozog; Nancy Braverman
Journal:  Genet Med       Date:  2019-12-11       Impact factor: 8.822

  5 in total

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