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2007 ICD-9-CM Volume 1 Diagnosis Codes Home > 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.* > 2007 ICD-9-CM Diagnosis 270.3 Disturbances of branched-chain amino-acid metabolism  On October 1, 2008 the 2009 ICD-9-CM codes came into effect. Therefore, this code may be out of date. The 2009 version of ICD-9-CM 270.3 can be accessed here. An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a "maple syrup" odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The 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. (From Adams et al., Principles of Neurology, 6th ed, p936) 270.3 is a specific code that can be used to specify a diagnosis 270.3 contains 19 index entries View the ICD-9-CM Volume 1 270.* hierarchy 270.3 also known as: Disturbances of metabolism of leucine, isoleucine, and valine Hypervalinemia Intermittent branched-chain ketonuria Leucine-induced hypoglycemia Leucinosis Maple syrup urine disease Index entries containing 270.3: Aciduria 791.9 methylmalonic 270.3 with glycinemia 270.7 Branched-chain amino-acid disease 270.3 Degradation disorder, branched-chain amino-acid 270.3 Disease, diseased - see also Syndrome maple syrup (urine) 270.3 Disorder - see also Disease amino acid (metabolic) (see also Disturbance, metabolism, amino acid) 270.9 maple syrup (urine) disease 270.3 branched-chain amino-acid degradation 270.3 degradation, branched-chain amino acid 270.3 Disturbance - see also Disease amino acid (metabolic) (see also Disorder, amino acid) 270.9 maple syrup (urine) disease 270.3 metabolism (acquired) (congenital) (see also Disorder, metabolism) 277.9 amino acid (see also Disorder, amino acid) 270.9 branched-chain 270.3 isoleucine 270.3 leucine 270.3 valine 270.3 Hypervalinemia 270.3 Hypoglycemia (spontaneous) 251.2 leucine-induced 270.3 Ketonuria 791.6 branched-chain, intermittent 270.3 Leucinosis 270.3 Maple syrup (urine) disease or syndrome 270.3 Syndrome - see also Disease maple syrup (urine) 270.3 Menkes' 759.89 maple syrup (urine) disease 270.3