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E71.118 Other branched-chain organic acidurias
BackE71Disorders of branched-chain amino-acid metabolism and fatty-acid metabolismCategory6 →E71.0Maple-syrup-urine diseaseCategoryE71.1Other disorders of branched-chain amino-acid metabolismCategory3 →E71.11Branched-chain organic aciduriasCategory3 →E71.110Isovaleric acidemiaCategoryE71.1113-methylglutaconic aciduriaCategoryE71.118Other branched-chain organic aciduriasCategoryE71.12Disorders of propionate metabolismCategory3 →E71.120Methylmalonic acidemiaCategoryE71.121Propionic acidemiaCategoryE71.128Other disorders of propionate metabolismCategoryE71.19Other disorders of branched-chain amino-acid metabolismCategoryE71.2Disorder of branched-chain amino-acid metabolism, unspecifiedCategoryE71.3Disorders of fatty-acid metabolismCategory4 →E71.30Disorder of fatty-acid metabolism, unspecifiedCategoryE71.31Disorders of fatty-acid oxidationCategory6 →E71.310Long chain/very long chain acyl CoA dehydrogenase deficiencyCategoryE71.311Medium chain acyl CoA dehydrogenase deficiencyCategoryE71.312Short chain acyl CoA dehydrogenase deficiencyCategoryE71.313Glutaric aciduria type IICategoryE71.314Muscle carnitine palmitoyltransferase deficiencyCategoryE71.318Other disorders of fatty-acid oxidationCategoryE71.32Disorders of ketone metabolismCategoryE71.39Other disorders of fatty-acid metabolismCategoryE71.4Disorders of carnitine metabolismCategory5 →E71.40Disorder of carnitine metabolism, unspecifiedCategoryE71.41Primary carnitine deficiencyCategoryE71.42Carnitine deficiency due to inborn errors of metabolismCategoryE71.43Iatrogenic carnitine deficiencyCategoryE71.44Other secondary carnitine deficiencyCategory2 →E71.440Ruvalcaba-Myhre-Smith syndromeCategoryE71.448Other secondary carnitine deficiencyCategoryE71.5Peroxisomal disordersCategory5 →E71.50Peroxisomal disorder, unspecifiedCategoryE71.51Disorders of peroxisome biogenesisCategory3 →E71.510Zellweger syndromeCategoryE71.511Neonatal adrenoleukodystrophyCategoryE71.518Other disorders of peroxisome biogenesisCategoryE71.52X-linked adrenoleukodystrophyCategory5 →E71.520Childhood cerebral X-linked adrenoleukodystrophyCategoryE71.521Adolescent X-linked adrenoleukodystrophyCategoryE71.522AdrenomyeloneuropathyCategoryE71.528Other X-linked adrenoleukodystrophyCategoryE71.529X-linked adrenoleukodystrophy, unspecified typeCategoryE71.53Other group 2 peroxisomal disordersCategoryE71.54Other peroxisomal disordersCategory4 →E71.540Rhizomelic chondrodysplasia punctataCategoryE71.541Zellweger-like syndromeCategoryE71.542Other group 3 peroxisomal disordersCategoryE71.548Other peroxisomal disordersCategory
CategoryCODABLE
E71.118
Other branched-chain organic acidurias
- Chapter
- IV · Endocrine, nutritional and metabolic diseases
- Section
- E70-E88
- Valid from
Source
CMS ICD-10-CM Tabular List, edition FY2026.