Literature DB >> 14212461

MAPLE SYRUP URINE DISEASE, WITH PARTICULAR REFERENCE TO DIETOTHERAPY.

S E SNYDERMAN, P M NORTON, E ROITMAN, L E HOLT.   

Abstract

Keywords:  AMINO ACID METABOLISM, INBORN ERRORS; ANTHROPOMETRY; BLOOD PROTEINS; CEREBROSPINAL FLUID PROTEINS; DIET THERAPY; ERYTHROCYTES; EXCHANGE TRANSFUSION; GROWTH; INFANT, NEWBORN; ISOLEUCINE; LEUCINE; MAPLE SYRUP URINE DISEASE; METHIONINE; VALINE

Mesh：

Substances：

Year:  1964        PMID： 14212461

Source DB:  PubMed          Journal:  Pediatrics        ISSN： 0031-4005            Impact factor:   7.124


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  55 in total

1.  Successful Pregnancy in a Woman with Maple Syrup Urine Disease: Case Report.

Authors:  Stefanie Heiber; Henryk Zulewski; Marianne Zaugg; Caroline Kiss; Matthias Baumgartner
Journal:  JIMD Rep       Date:  2015-02-27

2.  In response to R.E. Roux et al (2006) the longest surviving patient with classical maple syrup urine disease. J Inherit Metab Dis 29:190-194.

Authors:  Selma E Snyderman
Journal:  J Inherit Metab Dis       Date:  2006-08-18       Impact factor: 4.982

3.  Coadministration of branched-chain amino acids and lipopolysaccharide causes matrix metalloproteinase activation and blood-brain barrier breakdown.

Authors:  Giselli Scaini; Meline O S Morais; Leticia S Galant; Francieli Vuolo; Dhébora M Dall'Igna; Matheus A B Pasquali; Vitor M Ramos; Daniel P Gelain; Jose Claudio F Moreira; Patrícia F Schuck; Gustavo C Ferreira; Francisco G Soriano; Felipe Dal-Pizzol; Emilio L Streck
Journal:  Mol Neurobiol       Date:  2014-01-05       Impact factor: 5.590

4.  Neuroprotective Effect of Creatine and Pyruvate on Enzyme Activities of Phosphoryl Transfer Network and Oxidative Stress Alterations Caused by Leucine Administration in Wistar Rats.

Authors:  Elenara Rieger; Itiane Diehl de Franceschi; Thales Preissler; Clovis Milton Duval Wannmacher
Journal:  Neurotox Res       Date:  2017-06-13       Impact factor: 3.911

5.  Developmental profile of patients with maple syrup urine disease.

Authors:  A Nord; W J van Doorninck; C Greene
Journal:  J Inherit Metab Dis       Date:  1991       Impact factor: 4.982

Review 6.  Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: insight from animal studies.

Authors:  R Pessoa-Pureur; M Wajner
Journal:  J Inherit Metab Dis       Date:  2007-06-14       Impact factor: 4.982

7.  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

8.  Hudson memorial lecture. Neonatal management of organic acidurias. Clinical update.

Authors:  J M Saudubray; H Ogier; C Charpentier; E Depondt; F X Coudé; A Munnich; G Mitchell; F Rey; J Rey; J Frézal
Journal:  J Inherit Metab Dis       Date:  1984       Impact factor: 4.982

9.  Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain.

Authors:  M S Patel
Journal:  Biochem J       Date:  1974-10       Impact factor: 3.857

10.  Study on established lymphoid cells in maple syrup urine disease. Correlation with clinical heterogeneity.

Authors:  Y Jinno; I Akaboshi; T Katsuki; I Matsuda
Journal:  Hum Genet       Date:  1984       Impact factor: 4.132
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