DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism

  • Header · not billable

E71 is not billable. The category heads 38 billable codes in FY2027, grouped under 6 subdivisions.

Index → code

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E71 Disorders of branched-chain amino-acid metabolism and fatty-acid metabolism
Billable codes
38
Subdivisions
6
CC / MCC codes
31 / 0
HCC V28 codes
0
New FY2027
0
Index entries
101

Codes in E71

  1. E71.0
    Maple-syrup-urine disease
    BillableCC
  2. E71.1
    Other disorders of branched-chain amino-acid metabolism
    Header
  3. E71.11
    Branched-chain organic acidurias
    Header
  4. E71.110
    Isovaleric acidemia
    BillableCC
  5. E71.111
    3-methylglutaconic aciduria
    BillableCC
  6. E71.118
    Other branched-chain organic acidurias
    BillableCC
  7. E71.12
    Disorders of propionate metabolism
    Header
  8. E71.120
    Methylmalonic acidemia
    BillableCC
  9. E71.121
    Propionic acidemia
    BillableCC
  10. E71.128
    Other disorders of propionate metabolism
    BillableCC
  11. E71.19
    Other disorders of branched-chain amino-acid metabolism
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • Hyperleucine-isoleucinemia
    • Hypervalinemia
  12. E71.2
    Disorder of branched-chain amino-acid metabolism, unspecified
    BillableCC
  13. E71.3
    Disorders of fatty-acid metabolism
    Header
    Excludes1 3, Excludes2 1

    Excludes1

    • peroxisomal disorders (E71.5) E71.5
    • Refsum's disease (G60.1) G60.1
    • Schilder's disease (G37.0) G37.0

    Excludes2

    • carnitine deficiency due to inborn error of metabolism (E71.42) E71.42
  14. E71.30
    Disorder of fatty-acid metabolism, unspecified
    Billable
  15. E71.31
    Disorders of fatty-acid oxidation
    Header
  16. E71.310
    Long chain/very long chain acyl CoA dehydrogenase deficiency
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • LCAD deficiency
    • VLCAD deficiency
  17. E71.311
    Medium chain acyl CoA dehydrogenase deficiency
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • MCAD deficiency
  18. E71.312
    Short chain acyl CoA dehydrogenase deficiency
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • SCAD deficiency
  19. E71.313
    Glutaric aciduria type II
    BillableCC
    Inclusion terms 3, Excludes1 1

    Inclusion terms

    • Glutaric aciduria type II A
    • Glutaric aciduria type II B
    • Glutaric aciduria type II C

    Excludes1

    • glutaric aciduria (type 1) NOS (E72.3) E72.3
  20. E71.314
    Muscle carnitine palmitoyltransferase deficiency
    BillableCC
  21. E71.318
    Other disorders of fatty-acid oxidation
    BillableCC
  22. E71.32
    Disorders of ketone metabolism
    BillableCC
  23. E71.39
    Other disorders of fatty-acid metabolism
    BillableCC
  24. E71.4
    Disorders of carnitine metabolism
    Header
    Excludes1 1

    Excludes1

    • Muscle carnitine palmitoyltransferase deficiency (E71.314) E71.314
  25. E71.40
    Disorder of carnitine metabolism, unspecified
    Billable
  26. E71.41
    Primary carnitine deficiency
    Billable
  27. E71.42
    Carnitine deficiency due to inborn errors of metabolism
    Billable
    Code also 1

    Code also

    • associated inborn error or metabolism
  28. E71.43
    Iatrogenic carnitine deficiency
    Billable
    Inclusion terms 2

    Inclusion terms

    • Carnitine deficiency due to hemodialysis
    • Carnitine deficiency due to Valproic acid therapy
  29. E71.44
    Other secondary carnitine deficiency
    Header
  30. E71.440
    Ruvalcaba-Myhre-Smith syndrome
    Billable
  31. E71.448
    Other secondary carnitine deficiency
    Billable
  32. E71.5
    Peroxisomal disorders
    Header
    Excludes1 1

    Excludes1

    • Schilder's disease (G37.0) G37.0
  33. E71.50
    Peroxisomal disorder, unspecified
    BillableCC
  34. E71.51
    Disorders of peroxisome biogenesis
    Header
    Inclusion terms 1, Excludes1 1

    Inclusion terms

    • Group 1 peroxisomal disorders

    Excludes1

    • Refsum's disease (G60.1) G60.1
  35. E71.510
    Zellweger syndrome
    BillableCC
  36. E71.511
    Neonatal adrenoleukodystrophy
    BillableCC
    Excludes1 1

    Excludes1

    • X-linked adrenoleukodystrophy (E71.42-) E71.42
  37. E71.518
    Other disorders of peroxisome biogenesis
    BillableCC
  38. E71.52
    X-linked adrenoleukodystrophy
    Header
  39. E71.520
    Childhood cerebral X-linked adrenoleukodystrophy
    BillableCC
  40. E71.521
    Adolescent X-linked adrenoleukodystrophy
    BillableCC
  41. E71.522
    Adrenomyeloneuropathy
    BillableCC
  42. E71.528
    Other X-linked adrenoleukodystrophy
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • Addison only phenotype adrenoleukodystrophy
    • Addison-Schilder adrenoleukodystrophy
  43. E71.529
    X-linked adrenoleukodystrophy, unspecified type
    BillableCC
  44. E71.53
    Other group 2 peroxisomal disorders
    BillableCC
  45. E71.54
    Other peroxisomal disorders
    Header
  46. E71.540
    Rhizomelic chondrodysplasia punctata
    BillableCC
  47. E71.541
    Zellweger-like syndrome
    BillableCC
  48. E71.542
    Other group 3 peroxisomal disorders
    BillableCC
  49. E71.548
    Other peroxisomal disorders
    BillableCC

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

Notes that also apply

Block E70-E88 instructions (Excludes1 5, Excludes2 1): printed on the block page.

Chapter 4 instructions (Notes 1, Excludes1 1): printed on the chapter 4 page.

Index entries

  • Maple-syrup-urine disease → E71.0
  • Isovaleric acidemia → E71.110
  • Methylmalonic acidemia → E71.120
  • Propionic acidemia → E71.121
  • Hyperleucine-isoleucinemia → E71.19
  • Hypervalinemia → E71.19
  • Isoleucinosis → E71.19
  • Branched-chain amino-acid disorder → E71.2
  • Carnitine insufficiency → E71.40
  • Ruvalcaba-Myhre-Smith syndrome → E71.440
  • Zellweger's syndrome → E71.510
  • Adolescent X-linked adrenoleukodystrophy → E71.521
  • Adrenomyeloneuropathy → E71.522
  • Addison-Schilder complex → E71.528
  • Adrenoleukodystrophy → E71.529
  • Rhizomelic chondrodysplasia punctata → E71.540
  • Disease, diseasedmaple-syrup-urine → E71.0
  • Menkes' disease or syndromemeaning maple-syrup-urine disease → E71.0
  • Syndromemaple-syrup-urine → E71.0
  • Acidemiaisovaleric → E71.110
  • Disorder (of)isovaleric acidemia → E71.110
  • Acidemiamethylmalonic → E71.120
  • Disorder (of)methylmalonic acidemia → E71.120
  • Acidemiapropionic → E71.121
  • Disorder (of)propionic acidemia → E71.121
  • Disorder (of)hyperleucine-isoleucinemia → E71.19
  • Disorder (of)hypervalinemia → E71.19
  • Hypoglycemia (spontaneous)leucine-induced → E71.19
  • Disorder (of)branched-chain amino-acid metabolism → E71.2
  • Deficiency, deficientLCAD (long chain acyl CoA dehydrogenase deficiency) → E71.310
  • Deficiency, deficientVLCAD (very long chain acyl CoA dehydrogenase deficiency) → E71.310
  • Deficiency, deficientMCAD (medium chain acyl CoA dehydrogenase deficiency) → E71.311
  • Deficiency, deficientSCAD (short chain acyl CoA dehydrogenase deficiency) → E71.312
  • Muscle, muscularcarnitine (palmityltransferase) deficiency → E71.314
  • Disorder (of)ketone metabolism → E71.32
  • Deficiency, deficientcarnitine → E71.40
  • Disorder (of)carnitine metabolism → E71.40
  • SyndromeRuvalcaba-Myhre-Smith → E71.440
  • Disorder (of)peroxisomal → E71.50
  • SyndromeZellweger syndrome → E71.510

61 more, longer entries are not listed; the search box finds them by word.

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