136 related articles for article (PubMed ID: 13912814)
21. Thiamine response in maple syrup urine disease.
Fernhoff PM; Lubitz D; Danner DJ; Dembure PP; Schwartz HP; Hillman R; Bier DM; Elsas LJ
Pediatr Res; 1985 Oct; 19(10):1011-6. PubMed ID: 3903643
[TBL] [Abstract][Full Text] [Related]
22. The enzyme defect in maple syrup urine disease (branched chain ketoaciduria).
Seegmiller JE; Westall RG
J Ment Defic Res; 1967 Dec; 11(4):288-94. PubMed ID: 5582930
[No Abstract] [Full Text] [Related]
23. 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; 30 Suppl 2():138-9. PubMed ID: 11400750
[TBL] [Abstract][Full Text] [Related]
24. A variant form of branched-chain keto aciduria.
van der Horst JL; Wadman SK
Acta Paediatr Scand; 1971 Sep; 60(5):594-9. PubMed ID: 5125167
[No Abstract] [Full Text] [Related]
25. [Maple syrup urine disease: report of a mild variant case and results of dietary treatment].
Stoppoloni G; Santinelli R; Prisco F; Tolone C; D'Ambrosio M
Pediatria (Napoli); 1978 Sep; 86(3):409-20. PubMed ID: 754161
[No Abstract] [Full Text] [Related]
26. Ketotic hypoglycaemia associated with transient branched-chain aminoacidemia.
Hambraeus L; Westphal O; Hagberg B
Acta Paediatr Scand; 1972 Jan; 61(1):81-9. PubMed ID: 5018580
[No Abstract] [Full Text] [Related]
27. Urinary biomarkers of oxidative damage in Maple syrup urine disease: the L-carnitine role.
Guerreiro G; Mescka CP; Sitta A; Donida B; Marchetti D; Hammerschmidt T; Faverzani J; Coelho Dde M; Wajner M; Dutra-Filho CS; Vargas CR
Int J Dev Neurosci; 2015 May; 42():10-4. PubMed ID: 25680940
[TBL] [Abstract][Full Text] [Related]
28. 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; 22(1):77-88. PubMed ID: 17295076
[TBL] [Abstract][Full Text] [Related]
29. The relationship between the branched chain amino acids and their alpha-ketoacids in maple syrup urine disease.
Snyderman SE; Goldstein F; Sansaricq C; Norton PM
Pediatr Res; 1984 Sep; 18(9):851-3. PubMed ID: 6483508
[TBL] [Abstract][Full Text] [Related]
30. [A case of maple syrup urine disease. Biochemical study].
Lamedica GM; Fregonese B; Moroni G; Famularo L
Minerva Pediatr; 1972 Dec; 24(43):2143-9. PubMed ID: 4652219
[No Abstract] [Full Text] [Related]
31. Gas-liquid chromatography of alpha-keto acids: quantification of the branched-chain-alpha-keto acids from physiological sources.
Cree TC; Hutson SM; Harper AE
Anal Biochem; 1979 Jan; 92(1):159-63. PubMed ID: 426274
[No Abstract] [Full Text] [Related]
32. Maple syrup urine disease (branched-chain keto-aciduria) variant type manifesting as hyperkinetic behaviour and mental retardation. Report of two cases.
Kalyanaraman K; Chamukuttan S; Arjundas G; Gajanan N; Ramamurthi B
J Neurol Sci; 1972 Feb; 15(2):209-17. PubMed ID: 5010106
[No Abstract] [Full Text] [Related]
33. DIETARY TREATMENT OF A CHILD WITH MAPLE SYRUP URINE DISEASE (BRANCHED-CHAIN KETOACIDURIA).
WESTALL RG
Arch Dis Child; 1963 Oct; 38(201):485-91. PubMed ID: 14065992
[No Abstract] [Full Text] [Related]
34. Simple method for rapid quantification of branched-chain 2-oxo acids in physiological fluids as quinoxalinol derivatives by high-performance liquid chromatography.
Radeck W; Beck K; Staib W
J Chromatogr; 1988 Nov; 432():297-301. PubMed ID: 3220898
[No Abstract] [Full Text] [Related]
35. Mild variant of maple syrup urine disease.
Kodama S; Seki A; Hanabusa M; Morisita Y; Sakurai T
Eur J Pediatr; 1976 Dec; 124(1):31-6. PubMed ID: 1001326
[TBL] [Abstract][Full Text] [Related]
36. [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; 92(40):2839-42. PubMed ID: 23290213
[TBL] [Abstract][Full Text] [Related]
37. Maple syrup urine disease in Indian baby: branched chain amino and ketoaciduria.
Dastur DK; Manghani DK; Joshi MK; Adavi SV
Indian J Med Res; 1966 Oct; 54(10):915-22. PubMed ID: 5976996
[No Abstract] [Full Text] [Related]
38. [Maple syrup urine disease. Genetic heterogeneity, heterozygote diagnosis and new therapeutic approach (author's transl)].
Velázquez A; Montiel F; Sahw KN; Carnevale A; del Castillo V
Rev Invest Clin; 1981; 33(3):273-9. PubMed ID: 7330498
[No Abstract] [Full Text] [Related]
39. FAMILY SCREENING STUDIES IN MAPLE SYRUP URINE DISEASE (BRANCHED-CHAIN KETOACIDURIA).
WOODY NC; HARRIS JA
J Pediatr; 1965 Jun; 66():1042-8. PubMed ID: 14288457
[No Abstract] [Full Text] [Related]
40. [Biochemical studies in a patient with maple syrup urine disease (author's transl)].
Vaca G; Rivas F; Sánchez-Corona J; Olivares N; Aguirre-Negrete MG; González-Quiroga G; Medina C; Hernández A; Cantú JM
Rev Invest Clin; 1981; 33(4):379-82. PubMed ID: 7330511
[No Abstract] [Full Text] [Related]
[Previous] [Next] [New Search]