Literature DB >> 8295418

Postoperative metabolic decompensation in maple syrup urine disease is completely prevented by insulin.

B Biggemann1, R Zass, U Wendel.   

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Year:  1993        PMID： 8295418     DOI： 10.1007/BF00714298

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN： 0141-8955            Impact factor:   4.982


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  4 in total

1.  Branched-chain amino acid-free parenteral nutrition in the treatment of acute metabolic decompensation in patients with maple syrup urine disease.

Authors:  G T Berry; R Heidenreich; P Kaplan; F Levine; A Mazur; M J Palmieri; M Yudkoff; S Segal
Journal:  N Engl J Med       Date:  1991-01-17       Impact factor: 91.245

2.  Effect of insulin and plasma amino acid concentrations on leucine metabolism in man. Role of substrate availability on estimates of whole body protein synthesis.

Authors:  P Castellino; L Luzi; D C Simonson; M Haymond; R A DeFronzo
Journal:  J Clin Invest       Date:  1987-12       Impact factor: 14.808

3.  Dose-response curves of effects of insulin on leucine kinetics in humans.

Authors:  P Tessari; R Trevisan; S Inchiostro; G Biolo; R Nosadini; S V De Kreutzenberg; E Duner; A Tiengo; G Crepaldi
Journal:  Am J Physiol       Date:  1986-09

4.  Phenylalanine levels in PKU following minor surgery.

Authors:  A E Fiedler; M J Miller; H Bickel; S D Cederbaum
Journal:  Am J Med Genet       Date:  1982-04
  4 in total
  3 in total

Review 1.  Acute management of propionic acidemia.

Authors:  Kimberly A Chapman; Andrea Gropman; Erin MacLeod; Kathy Stagni; Marshall L Summar; Keiko Ueda; Nicholas Ah Mew; Jill Franks; Eddie Island; Dietrich Matern; Loren Pena; Brittany Smith; V Reid Sutton; Tiina Urv; Charles Venditti; Anupam Chakrapani
Journal:  Mol Genet Metab       Date:  2011-09-24       Impact factor: 4.797

2.  Pregnancy in a woman with maple syrup urine disease.

Authors:  S Grünewald; F Hinrichs; U Wendel
Journal:  J Inherit Metab Dis       Date:  1998-04       Impact factor: 4.982

Review 3.  Emergency management of inherited metabolic diseases.

Authors:  V Prietsch; M Lindner; J Zschocke; W L Nyhan; G F Hoffmann
Journal:  J Inherit Metab Dis       Date:  2002-11       Impact factor: 4.982
  3 in total

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