Literature DB >> 699323

Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease.

U Langenbeck, U Wendel, A Mench-Hoinowski, D Kuschel, K Becker, H Przyrembel, H J Bremer.   

Abstract

In 62 blood samples from 3 patients with classical maple syrup urine disease and from one patient with a variant form, a close linear correlation was found between levels of branched chain amino acids and their corresponding alpha-keto acids. Keto acids were determined as O-trimethylsilyl quinoxalinols by gas chromatography with a nitrogen-selective detector.

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Year:  1978        PMID： 699323     DOI： 10.1016/0009-8981(78)90433-3

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


  13 in total

1.  Maple syrup urine disease: interrelations between branched-chain amino-, oxo- and hydroxyacids; implications for treatment; associations with CNS dysmyelination.

Authors:  E Treacy; C L Clow; T R Reade; D Chitayat; O A Mamer; C R Scriver
Journal:  J Inherit Metab Dis       Date:  1992       Impact factor: 4.982

2.  Removal of branched-chain amino acids and alpha-ketoisocaproate by haemofiltration and haemodiafiltration.

Authors:  J B Gouyon; D Semama; A Prévot; J Desgres
Journal:  J Inherit Metab Dis       Date:  1996       Impact factor: 4.982

3.  The management and long term outcome of organic acidaemias.

Authors:  J V Leonard; P Daish; E R Naughten; K Bartlett
Journal:  J Inherit Metab Dis       Date:  1984       Impact factor: 4.982

4.  Peritoneal dialysis in maple-syrup-urine disease: studies on branched-chain amino and keto acids.

Authors:  U Wendel; K Becker; H Przyrembel; M Bulla; C Manegold; A Mench-Hoinowski; U Langenbeck
Journal:  Eur J Pediatr       Date:  1980-06       Impact factor: 3.183

5.  Blood levels of branched-chain alpha-keto acids in uremia: Therapeutic implications.

Authors:  P Schauder; D Matthaei; F Scheler; A Mench-Hoinowski; U Langenbeck
Journal:  Klin Wochenschr       Date:  1979-08-15

6.  Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease.

Authors:  Y Shigematsu; K Kikuchi; T Momoi; M Sudo; Y Kikawa; K Nosaka; M Kuriyama; S Haruki; K Sanada; N Hamano
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

7.  Structural white matter changes in adolescents and young adults with maple syrup urine disease.

Authors:  D Klee; E Thimm; H J Wittsack; D Schubert; R Primke; G Pentang; J Schaper; U Mödder; A Antoch; U Wendel; M Cohnen
Journal:  J Inherit Metab Dis       Date:  2013-01-25       Impact factor: 4.982

8.  Maple syrup urine disease--therapeutic use of insulin in catabolic states.

Authors:  U Wendel; U Langenbeck; I Lombeck; H J Bremer
Journal:  Eur J Pediatr       Date:  1982-11       Impact factor: 3.183

9.  Intellectual performance of children with maple syrup urine disease.

Authors:  C Hilliges; D Awiszus; U Wendel
Journal:  Eur J Pediatr       Date:  1993-02       Impact factor: 3.183

10.  A familial progressive neurodegenerative disease with 2-oxoglutaric aciduria.

Authors:  A Kohlschütter; A Behbehani; U Langenbeck; M Albani; P Heidemann; G Hoffmann; J Kleineke; W Lehnert; U Wendel
Journal:  Eur J Pediatr       Date:  1982-02       Impact factor: 3.183
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