Pathogenesis of maple-syrup-urine disease: observations during dietary management and treatment of coma by peritoneal dialysis

Automatic Recording Apparatus for Use in Chromatography of Amino Acids

1958 • 9,612 citations

PYRUVIC ACID

1943 • 1,944 citations

The Metabolic Basis of Inherited Disease.

1966 • 547 citations

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

1954 • 408 citations

ION EXCHANGE CHROMATOGRAPHY OF THE FREE AMINO ACIDS IN THE PLASMA OF THE NEWBORN INFANT

1965 • 248 citations

MAPLE SYRUP URINE DISEASE, WITH PARTICULAR REFERENCE TO DIETOTHERAPY

1964 • 229 citations

"Maple Syrup Urine Disease"

1959 • 227 citations

Origins and metabolism of the intracellular amino acid pools in rat liver and muscle

1967 • 204 citations

KINETICS OF PERITONEAL DIALYSIS

1961 • 204 citations

Quantitative Displacement of Acid-Base Equilibrium in Metabolic Acidosis

1967 • 187 citations

"Maple Syrup Urine Disease"

1959 • 121 citations

Intermittent Branched-Chain Ketonuria

1967 • 113 citations

Metabolism of the white blood cells in maple-syrup-urine disease

1960 • 103 citations

Peritoneal Dialysis

1962 • 101 citations

BRAIN LIPIDS, PROTEOLIPIDS, AND FREE AMINO ACIDS IN MAPLE SYRUP URINE DISEASE*

1966 • 76 citations

Dietary Treatment of a Child with Maple Syrup Urine Disease (Branched-chain Ketoaciduria)

1963 • 69 citations

ADDITION OF AMINOACIDS TO PERITONEAL-DIALYSIS FLUID

1968 • 65 citations

Hemodialysis and Plasma Amino Acid Composition in Chronic Renal Failure

1968 • 63 citations

Maple Syrup Urine Disease

1961 • 60 citations

CLINICAL AND BIOCHEMICAL OBSERVATIONS ON AN APPARENTLY NONFATAL VARIANT OF BRANCHEDCHAIN KETOACIDURIA (MAPLE SYRUP URINE DISEASE)

1961 • 59 citations

Studies in Maple Syrup Urine Disease

1961 • 58 citations

Late Manifesting Variant of Branched‐Chain Ketoaciduria (Maple Syrup Urine Disease)

1964 • 50 citations

Cerebral lipids in maple syrup disease

1965 • 46 citations

The use of [14C]amino acids to study sites and rates of antibody synthesis in living hyperimmune rabbits

1958 • 42 citations

Maple Syrup Urine Disease

1963 • 33 citations

Maple Syrup Disease

1967 • 31 citations

Biochemical studies on branched-chain oxoacid oxidases

1967 • 21 citations

EAR COMPLICATIONS OF INFLUENZA

1932 • 20 citations

Brain damage by inborn errors of metabolism

1969 • 8 citations