Maple syrup urine disease icd 1012/2/2023 The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. ICD-10 E71.0 - Maple-syrup-urine disease Chapter 4 Section E70-E88 E71.0 ICD-10 Billable. No ads, no spam, and its free for everybody. ![]() The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. This site is dedicated exclusively to helping you look up ICD-10 codes, quickly access the codes you use most, and become more comfortable with the new code set in general. These metabolites accumulate in body fluids and render a "maple syrup" odor. Diseases » Nervous System Diseases » Central Nervous System Diseases » Brain Diseases » Brain Diseases, Metabolic » Brain Diseases, Metabolic, Inborn » Maple Syrup Urine Diseaseĭiseases » Congenital, Hereditary, and Neonatal Diseases and Abnormalities » Genetic Diseases, Inborn » Metabolism, Inborn Errors » Amino Acid Metabolism, Inborn Errors » Maple Syrup Urine Diseaseĭiseases » Congenital, Hereditary, and Neonatal Diseases and Abnormalities » Genetic Diseases, Inborn » Metabolism, Inborn Errors » Brain Diseases, Metabolic, Inborn » Maple Syrup Urine Diseaseĭiseases » Nutritional and Metabolic Diseases » Metabolic Diseases » Brain Diseases, Metabolic » Brain Diseases, Metabolic, Inborn » Maple Syrup Urine Diseaseĭiseases » Nutritional and Metabolic Diseases » Metabolic Diseases » Metabolism, Inborn Errors » Amino Acid Metabolism, Inborn Errors » Maple Syrup Urine Diseaseĭiseases » Nutritional and Metabolic Diseases » Metabolic Diseases » Metabolism, Inborn Errors » Brain Diseases, Metabolic, Inborn » Maple Syrup Urine DiseaseĪn 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).
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