104 related articles for article (PubMed ID: 3088326)
1. Thiamine non-responsive intermittent branched-chain ketoaciduria in a Laotian child.
Pueschel SM
J Inherit Metab Dis; 1986; 9(1):72. PubMed ID: 3088326
[No Abstract] [Full Text] [Related]
2. Effects of thiamine in a patient with a variant form of branched-chian ketoaciduria.
Duran M; Tielens AG; Wadman SK; Stigter JC; Kleijer WJ
Acta Paediatr Scand; 1978 May; 67(3):367-72. PubMed ID: 654914
[TBL] [Abstract][Full Text] [Related]
3. 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]
4. Thiamine-responsive intermittent branched-chain ketoaciduria.
Pueschel SM; Bresnan MJ; Shih VE; Levy HL
J Pediatr; 1979 Apr; 94(4):628-31. PubMed ID: 430308
[No Abstract] [Full Text] [Related]
5. The role of thiamin in maple syrup urine disease.
Elsas LJ; Danner DJ
Ann N Y Acad Sci; 1982; 378():404-21. PubMed ID: 7044230
[No Abstract] [Full Text] [Related]
6. The gas-chromatographic diagnosis of intermittent maple syrup urine disease (branch-chain ketoaciduria).
Gompertz D; Draffan GH
Clin Chim Acta; 1972 Aug; 40(1):5-11. PubMed ID: 5056643
[No Abstract] [Full Text] [Related]
7. Thiamin-responsive maple syrup urine disease: seizures after 7 years of satisfactory metabolic control.
Delis D; Michelakakis H; Katsarou E; Bartsocas CS
J Inherit Metab Dis; 2001 Nov; 24(6):683-4. PubMed ID: 11768588
[No Abstract] [Full Text] [Related]
8. Maple syrup urine disease. Thiamin responsive branched chain aminoaciduria: a unique case in an adult: (case report).
Kannan V; Subramanyam K
J Assoc Physicians India; 1977 Feb; 25(2):163-6. PubMed ID: 914742
[No Abstract] [Full Text] [Related]
9. Neuropathological observations in maple syrup urine disease: branched-chain ketoaciduria.
SILBERMAN J; DANCIS J; FEIGIN I
Arch Neurol; 1961 Oct; 5():351-63. PubMed ID: 13912814
[No Abstract] [Full Text] [Related]
10. Clinical and biochemical observations on an apparently nonfatal variant of branched-chain ketoaciduria (maple syrup urine disease).
MORRIS MD; LEWIS BD; DOOLAN PD; HARPER HA
Pediatrics; 1961 Dec; 28():918-23. PubMed ID: 14476272
[No Abstract] [Full Text] [Related]
11. Branched-chain alpha-keto acids isolated as oxime derivatives: relationship to the corresponding hydroxy acids and amino acids in maple syrup urine disease.
Lancaster G; Mamer OA; Scriver CR
Metabolism; 1974 Mar; 23(3):257-65. PubMed ID: 4813956
[No Abstract] [Full Text] [Related]
12. So-called thiamin-responsive maple syrup urine disease: 15-year follow-up of the original patient.
Scriver CR; Clow CL; George H
J Pediatr; 1985 Nov; 107(5):763-5. PubMed ID: 4056978
[No Abstract] [Full Text] [Related]
13. Thiamine-responsive inborn errors of metabolism.
Duran M; Wadman SK
J Inherit Metab Dis; 1985; 8 Suppl 1():70-5. PubMed ID: 3930844
[TBL] [Abstract][Full Text] [Related]
14. Late-onset branched-chain ketoaciduria: (maple syrup urine disease).
Morris MD; Fisher DA; Fiser R
J Lancet; 1966 Mar; 86(3):149-52. PubMed ID: 5904649
[No Abstract] [Full Text] [Related]
15. Clinical and biochemical-genetic aspects of intermittent branched-chain ketoaciduria. Report of two Scandinavian families.
Goedde HW; Langenbeck U; Brackertz D; Keller W; Rokkones T; Halvorsen S; Kiil R; Merton B
Acta Paediatr Scand; 1970 Jan; 59(1):83-7. PubMed ID: 5452306
[No Abstract] [Full Text] [Related]
16. 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]
17. Thiamin-responsive maple-syrup-urine disease: decreased affinity of the mutant branched-chain alpha-keto acid dehydrogenase for alpha-ketoisovalerate and thiamin pyrophosphate.
Chuang DT; Ku LS; Cox RP
Proc Natl Acad Sci U S A; 1982 May; 79(10):3300-4. PubMed ID: 6954481
[TBL] [Abstract][Full Text] [Related]
18. A case of classical maple syrup urine disease "thiamine non-responsive".
Wong PW; Justice P; Smith GF; Hsia DY
Clin Genet; 1972; 3(1):27-33. PubMed ID: 5066975
[No Abstract] [Full Text] [Related]
19. Family with intermittent maple syrup urine disease.
Valman HB; Patrick AD; Seakins JW; Platt JW; Gompertz D
Arch Dis Child; 1973 Mar; 48(3):225-8. PubMed ID: 4693464
[TBL] [Abstract][Full Text] [Related]
20. [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]
[Next] [New Search]