Thiamine Response in Maple Syrup Urine Disease

A NEW SYNDROME: PROGRESSIVE FAMILIAL INFANTILE CEREBRAL DYSFUNCTION ASSOCIATED WITH AN UNUSUAL URINARY SUBSTANCE

John H. Menkes, Peter L. Hurst, John M. Craig · PEDIATRICS · 1954 · 411 citations

Medicine, Neurology, Pediatrics +8

THIAMINE-RESPONSIVE MAPLE-SYRUP-URINE DISEASE

C. R. Scriver, C Clow, Susan A. MacKenzie et al. · The Lancet · 1971 · 171 citations

Maple Syrup Urine Disease: Coenzyme function and prenatal monitoring

Louis J. Elsas, Jean H. Priest, Frances B. Wheeler et al. · Metabolism · 1974 · 48 citations

Medicine, Maternal Health, Obstetrics +12

Classical Maple Syrup Urine Disease: Cofactor resistance

Louis J. Elsas, Beatrice A. Pask, Frances B. Wheeler et al. · Metabolism · 1972 · 24 citations

Veterinary Medicine, Infectious Diseases, Pathobiology +6

EFFECTS OF THIAMINE IN A PATIENT WITH A VARIANT FORM OF BRANCHED‐CHAIN KETOACIDURIA

M. Durán, Aloysius G. M. Tielens, S.K. Wadman et al. · Acta Paediatrica · 1978 · 21 citations

Pharmacy, Pediatrics, Pharmacotherapy +12