Free Diagnosis Codes Lookup > 2008 > Endocrine, Nutritional And Metabolic Diseases, And Immunity Disorders 240-279 > Other Metabolic Disorders And Immunity Disorders 270-279 > Disorders of amino-acid transport and metabolism 270.* >
2008 ICD-9-CM Diagnosis Code 270.3
Disturbances of branched-chain amino-acid metabolism
autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids; metabolites accumulate in body fluids and render a "maple syrup" odor; divided into classic, intermediate, intermittent, and thiamine responsive subtypes; classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia; the intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting.
Also applicable to/known as
Disturbances of metabolism of leucine, isoleucine, and valine
Hypervalinemia
Intermittent branched-chain ketonuria
Leucine-induced hypoglycemia
Leucinosis
Maple syrup urine disease
ICD-9-CM Volume 2 Index Entries That Refer To 270.3
2008 ICD-9-CM Diagnosis Code 270.3
270.3
