ICD-10-CM Code E71: Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism and Related Diagnosis Codes.

The ICD-10-CM (the International Classification of Diseases, Tenth Revision, Clinical Modification) is a standardized coding system used when classifying diseases and medical conditions. Healthcare providers report these codes on Medical claims when diagnosing patient care.

Explore ICD-10-CM codes, organized by chapters based on body systems and medical conditions, as outlined in the International Classification of Diseases, Tenth Revision, Clinical Modification.

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Eclaims - ICD-10-CM Tabular Code List v1.0.0

A categorized list of ICD-10-CM codes organized by chapters based on body systems or medical conditions, according to the International Classification of Diseases, Tenth Revision, Clinical Modification.

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  • E71 - Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
    • E71.0 - Maple-syrup-urine disease
    • E71.1 - Other disorders of branched-chain amino-acid metabolism
      • E71.11 - Branched-chain organic acidurias
        • E71.110 - Isovaleric acidemia
        • E71.111 - 3-methylglutaconic aciduria
        • E71.118 - Other branched-chain organic acidurias
      • E71.12 - Disorders of propionate metabolism
        • E71.120 - Methylmalonic acidemia
        • E71.121 - Propionic acidemia
        • E71.128 - Other disorders of propionate metabolism
      • E71.19 - Other disorders of branched-chain amino-acid metabolism
    • E71.2 - Disorder of branched-chain amino-acid metabolism, unspecified
    • E71.3 - Disorders of fatty-acid metabolism
      • E71.30 - Disorder of fatty-acid metabolism, unspecified
      • E71.31 - Disorders of fatty-acid oxidation
        • E71.310 - Long chain/very long chain acyl CoA dehydrogenase deficiency
        • E71.311 - Medium chain acyl CoA dehydrogenase deficiency
        • E71.312 - Short chain acyl CoA dehydrogenase deficiency
        • E71.313 - Glutaric aciduria type II
        • E71.314 - Muscle carnitine palmitoyltransferase deficiency
        • E71.318 - Other disorders of fatty-acid oxidation
      • E71.32 - Disorders of ketone metabolism
      • E71.39 - Other disorders of fatty-acid metabolism
    • E71.4 - Disorders of carnitine metabolism
      • E71.40 - Disorder of carnitine metabolism, unspecified
      • E71.41 - Primary carnitine deficiency
      • E71.42 - Carnitine deficiency due to inborn errors of metabolism
      • E71.43 - Iatrogenic carnitine deficiency
      • E71.44 - Other secondary carnitine deficiency
        • E71.440 - Ruvalcaba-Myhre-Smith syndrome
        • E71.448 - Other secondary carnitine deficiency
    • E71.5 - Peroxisomal disorders
      • E71.50 - Peroxisomal disorder, unspecified
      • E71.51 - Disorders of peroxisome biogenesis
        • E71.510 - Zellweger syndrome
        • E71.511 - Neonatal adrenoleukodystrophy
        • E71.518 - Other disorders of peroxisome biogenesis
      • E71.52 - X-linked adrenoleukodystrophy
        • E71.520 - Childhood cerebral X-linked adrenoleukodystrophy
        • E71.521 - Adolescent X-linked adrenoleukodystrophy
        • E71.522 - Adrenomyeloneuropathy
        • E71.528 - Other X-linked adrenoleukodystrophy
        • E71.529 - X-linked adrenoleukodystrophy, unspecified type
      • E71.53 - Other group 2 peroxisomal disorders
      • E71.54 - Other peroxisomal disorders
        • E71.540 - Rhizomelic chondrodysplasia punctata
        • E71.541 - Zellweger-like syndrome
        • E71.542 - Other group 3 peroxisomal disorders
        • E71.548 - Other peroxisomal disorders
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Note: The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) codes are developed and maintained by the Centers for Disease Control and Prevention (CDC). CDC's National Center for Health Statistics (NCHS) is responsible for the clinical modification of ICD-10 for use in the United States. The World Health Organization (WHO), which owns and publishes ICD-10, authorized NCHS to develop ICD-10-CM. All modifications to ICD-10 must conform to WHO's established ICD structure and conventions. All rights are reserved by the respective entities.