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Journal Abstract Search

202 related items for PubMed ID: 10947205

  • 21. Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease.
    Zinnanti WJ, Lazovic J, Griffin K, Skvorak KJ, Paul HS, Homanics GE, Bewley MC, Cheng KC, Lanoue KF, Flanagan JM.
    Brain; 2009 Apr; 132(Pt 4):903-18. PubMed ID: 19293241
    [Abstract] [Full Text] [Related]

  • 22. Amino acid clearance during acute metabolic decompensation in maple syrup urine disease treated with continuous venovenous hemodialysis with filtration.
    Hmiel SP, Martin RA, Landt M, Levy FH, Grange DK.
    Pediatr Crit Care Med; 2004 May; 5(3):278-81. PubMed ID: 15115568
    [Abstract] [Full Text] [Related]

  • 23. Quantification of branched-chain amino acids in blood spots and plasma by liquid chromatography tandem mass spectrometry for the diagnosis of maple syrup urine disease.
    Sowell J, Pollard L, Wood T.
    J Sep Sci; 2011 Mar; 34(6):631-9. PubMed ID: 21328532
    [Abstract] [Full Text] [Related]

  • 24. Docosahexaenoic acid status in females of reproductive age with maple syrup urine disease.
    Mazer LM, Yi SH, Singh RH.
    J Inherit Metab Dis; 2010 Apr; 33(2):121-7. PubMed ID: 20217236
    [Abstract] [Full Text] [Related]

  • 25. Cerebrospinal fluid concentrations of large neutral and basic amino acids in Macaca mulatta: diurnal variations and responses to chronic changes in dietary protein intake.
    Grimes MA, Cameron JL, Fernstrom JD.
    Metabolism; 2009 Jan; 58(1):129-40. PubMed ID: 19059540
    [Abstract] [Full Text] [Related]

  • 26. Diagnosis of maple syrup urine disease by determination of L-valine, L-isoleucine, L-leucine and L-phenylalanine in neonatal blood spots by gas chromatography-mass spectrometry.
    Deng C, Deng Y.
    J Chromatogr B Analyt Technol Biomed Life Sci; 2003 Jul 25; 792(2):261-8. PubMed ID: 12860033
    [Abstract] [Full Text] [Related]

  • 27. Living related versus deceased donor liver transplantation for maple syrup urine disease.
    Feier F, Schwartz IV, Benkert AR, Seda Neto J, Miura I, Chapchap P, da Fonseca EA, Vieira S, Zanotelli ML, Pinto e Vairo F, Camelo JS, Margutti AV, Mazariegos GV, Puffenberger EG, Strauss KA.
    Mol Genet Metab; 2016 Mar 25; 117(3):336-43. PubMed ID: 26786177
    [Abstract] [Full Text] [Related]

  • 28. Maple syrup urine disease: clinical, EEG, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity.
    Korein J, Sansaricq C, Kalmijn M, Honig J, Lange B.
    Int J Neurosci; 1994 Nov 25; 79(1-2):21-45. PubMed ID: 7744549
    [Abstract] [Full Text] [Related]

  • 29. Investigation of inflammatory profile in MSUD patients: benefit of L-carnitine supplementation.
    Mescka CP, Guerreiro G, Donida B, Marchetti D, Wayhs CA, Ribas GS, Coitinho AS, Wajner M, Dutra-Filho CS, Vargas CR.
    Metab Brain Dis; 2015 Oct 25; 30(5):1167-74. PubMed ID: 26002427
    [Abstract] [Full Text] [Related]

  • 30. Second-tier test for quantification of alloisoleucine and branched-chain amino acids in dried blood spots to improve newborn screening for maple syrup urine disease (MSUD).
    Oglesbee D, Sanders KA, Lacey JM, Magera MJ, Casetta B, Strauss KA, Tortorelli S, Rinaldo P, Matern D.
    Clin Chem; 2008 Mar 25; 54(3):542-9. PubMed ID: 18178665
    [Abstract] [Full Text] [Related]

  • 31. [Clinical characteristics and analysis of mass spectrometric data in 33 patients with maple syrup urine disease].
    Yang N, Han LS, Ye J, Qiu WJ, Zhang HW, Gao XL, Wang Y, Li XY, Xu H, Gu XF.
    Zhonghua Yi Xue Za Zhi; 2012 Oct 30; 92(40):2839-42. PubMed ID: 23290213
    [Abstract] [Full Text] [Related]

  • 32. Acute illness in maple syrup urine disease: dynamics of protein metabolism and implications for management.
    Thompson GN, Francis DE, Halliday D.
    J Pediatr; 1991 Jul 30; 119(1 Pt 1):35-41. PubMed ID: 2066856
    [Abstract] [Full Text] [Related]

  • 33. Oral enzyme therapy for maple syrup urine disease (MSUD) suppresses plasma leucine levels in intermediate MSUD mice and healthy nonhuman primates.
    Skvorak K, Liu J, Kruse N, Mehmood R, Das S, Jenne S, Chng C, Lao UL, Duan D, Asfaha J, Du F, Teadt L, Sero A, Ching C, Riggins J, Pope L, Yan P, Mashiana H, Ismaili MHA, McCluskie K, Huisman G, Silverman AP.
    J Inherit Metab Dis; 2023 Nov 30; 46(6):1089-1103. PubMed ID: 37494004
    [Abstract] [Full Text] [Related]

  • 34. Treatment of the acute crisis in maple syrup urine disease.
    Nyhan WL, Rice-Kelts M, Klein J, Barshop BA.
    Arch Pediatr Adolesc Med; 1998 Jun 30; 152(6):593-8. PubMed ID: 9641714
    [Abstract] [Full Text] [Related]

  • 35. Reduction of plasma concentrations of large neutral amino acids in patients with maple syrup urine disease during crises.
    Wajner M, Vargas CR.
    Arch Dis Child; 1999 Jun 30; 80(6):579. PubMed ID: 10523253
    [No Abstract] [Full Text] [Related]

  • 36. Glucose and alanine metabolism in children with maple syrup urine disease.
    Haymond MW, Ben-Galim E, Strobel KE.
    J Clin Invest; 1978 Aug 30; 62(2):398-405. PubMed ID: 670400
    [Abstract] [Full Text] [Related]

  • 37. An early diagnosis leads to a good prognosis: a patient with maple syrup urine disease--screened by tandem mass spectrometry.
    Lin JF, Chiu PC, Hsu HY, Lin SM, Chen YY, Hsieh KS.
    Acta Paediatr Taiwan; 2004 Aug 30; 45(5):287-9. PubMed ID: 15868812
    [Abstract] [Full Text] [Related]

  • 38. Total branched-chain amino acids requirement in patients with maple syrup urine disease by use of indicator amino acid oxidation with L-[1-13C]phenylalanine.
    Riazi R, Rafii M, Clarke JT, Wykes LJ, Ball RO, Pencharz PB.
    Am J Physiol Endocrinol Metab; 2004 Jul 30; 287(1):E142-9. PubMed ID: 14970005
    [Abstract] [Full Text] [Related]

  • 39. Development of micellar electro kinetic chromatography for the separation and quantitation of L-valine, L-leucine, L-isoleucin and L-phenylalanine in human plasma and comparison with HPLC.
    Darvish M, Ebrahimi SA, Ghadam P.
    Pak J Biol Sci; 2007 Aug 01; 10(15):2436-41. PubMed ID: 19070110
    [Abstract] [Full Text] [Related]

  • 40. Electroencephalograms in a case of maple syrup urine disease: their relation to serum levels of branched-chain amino acids.
    Iinuma K, Saito T, Wada Y, Onuma A, Takamatsu N.
    Tohoku J Exp Med; 1976 Oct 01; 120(2):191-5. PubMed ID: 982436
    [Abstract] [Full Text] [Related]

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