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22. [Studies on "branched chain oxoacid aciduria" (maple syrup diseases)]. Goedde HW, Blume KG, Möhlenbeck F, Rotthauwe HW, Simon HA, Lang K. Med Klin; 1966 Dec 30; 61(52):2063-7. PubMed ID: 4385693 [No Abstract] [Full Text] [Related]
23. Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease. Schönberger S, Schweiger B, Schwahn B, Schwarz M, Wendel U. Mol Genet Metab; 2004 May 30; 82(1):69-75. PubMed ID: 15110325 [Abstract] [Full Text] [Related]
24. Intracellular levels and metabolism of leucine and alpha-ketoisocaproate in normal and maple syrup urine disease fibroblasts. Wendel U, Langenbeck U. Biochem Med; 1984 Jun 30; 31(3):294-302. PubMed ID: 6477534 [Abstract] [Full Text] [Related]
25. Synaptic plasma membrane Na(+), K (+)-ATPase activity is significantly reduced by the alpha-keto acids accumulating in maple syrup urine disease in rat cerebral cortex. Wajner A, Bürger C, Dutra-Filho CS, Wajner M, de Souza Wyse AT, Wannmacher CM. Metab Brain Dis; 2007 Mar 30; 22(1):77-88. PubMed ID: 17295076 [Abstract] [Full Text] [Related]
26. Plasma amino acid analyses in two cases of maple syrup urine disease. Surarit R, Srisomsap C, Wasant P, Svasti J, Suthatvoravut U, Chokchaichamnankit D, Liammongkolkul S. Southeast Asian J Trop Med Public Health; 1999 Mar 30; 30 Suppl 2():138-9. PubMed ID: 11400750 [Abstract] [Full Text] [Related]
27. Exfoliative erythroderma resulting from inadequate intake of branched-chain amino acids in infants with maple syrup urine disease. Northrup H, Sigman ES, Hebert AA. Arch Dermatol; 1993 Mar 30; 129(3):384-5. PubMed ID: 8447687 [No Abstract] [Full Text] [Related]
28. Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N. J Inherit Metab Dis; 1983 Mar 30; 6(4):183-9. PubMed ID: 6422161 [Abstract] [Full Text] [Related]
29. [Hereditary metabolic diseases: maple syrup disease]. Berger R, Broyer M. Presse Med (1893); 1968 Jul 13; 76(30):1531-2. PubMed ID: 5686194 [No Abstract] [Full Text] [Related]
30. Nasogastric drip feeding as the only treatment of neonatal maple syrup urine disease. Parini R, Sereni LP, Bagozzi DC, Corbetta C, Rabier D, Narcy C, Hubert P, Saudubray JM. Pediatrics; 1993 Aug 13; 92(2):280-3. PubMed ID: 8337030 [No Abstract] [Full Text] [Related]
31. ENU mutagenesis identifies mice with mitochondrial branched-chain aminotransferase deficiency resembling human maple syrup urine disease. Wu JY, Kao HJ, Li SC, Stevens R, Hillman S, Millington D, Chen YT. J Clin Invest; 2004 Feb 13; 113(3):434-40. PubMed ID: 14755340 [Abstract] [Full Text] [Related]
32. [Biochemical, clinical and genetic analysis of various aminoacidopathies (non-ketotic hyperglycemia, maple syrup urine disease, histidinemia, tyrosinemia)]. László A, Nagy I, Szücs L, Havass Z, Sztriha L, Svékus A, Veres E. Orv Hetil; 1992 Nov 29; 133(48):3075-80. PubMed ID: 1448273 [Abstract] [Full Text] [Related]
33. Correlations between branched-chain amino acids and branched-chain alpha-keto acids in blood in maple syrup urine disease. Langenbeck U, Wendel U, Mench-Hoinowski A, Kuschel D, Becker K, Przyrembel H, Bremer HJ. Clin Chim Acta; 1978 Sep 01; 88(2):283-91. PubMed ID: 699323 [Abstract] [Full Text] [Related]
35. Branched-chain amino acid-free parenteral nutrition in the treatment of acute metabolic decompensation in patients with maple syrup urine disease. Berry GT, Heidenreich R, Kaplan P, Levine F, Mazur A, Palmieri MJ, Yudkoff M, Segal S. N Engl J Med; 1991 Jan 17; 324(3):175-9. PubMed ID: 1898534 [No Abstract] [Full Text] [Related]
36. [Biochemical-genetic studies in maple syrup disease]. Goedde HW, Blume KG. Med Welt; 1968 Dec 17; 49():2699-703. PubMed ID: 5730703 [No Abstract] [Full Text] [Related]
38. Intermediate maple syrup urine disease: neuroimaging observations in 3 patients from South India. Bindu PS, Shehanaz KE, Christopher R, Pal PK, Ravishankar S. J Child Neurol; 2007 Jul 17; 22(7):911-3. PubMed ID: 17715290 [Abstract] [Full Text] [Related]
39. Domino hepatic transplantation in maple syrup urine disease. Barshop BA, Khanna A. N Engl J Med; 2005 Dec 01; 353(22):2410-1. PubMed ID: 16319396 [No Abstract] [Full Text] [Related]
40. Maple syrup urine disease in Thai infants. Pangkanon S, Charoensiriwatana W, Sangtawesin V. J Med Assoc Thai; 2008 Oct 01; 91 Suppl 3():S41-4. PubMed ID: 19255991 [Abstract] [Full Text] [Related] Page: [Previous] [Next] [New Search]