DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E72 Other disorders of amino-acid metabolism

  • Header · not billable

E72 is not billable. The category heads 31 billable codes in FY2027, grouped under 8 subdivisions.

Index → code

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E72 Other disorders of amino-acid metabolism
Billable codes
31
Subdivisions
8
CC / MCC codes
27 / 0
HCC V28 codes
3
New FY2027
0
Index entries
182

Codes in E72

  1. E72.0
    Disorders of amino-acid transport
    Header
    Excludes1 1

    Excludes1

    • disorders of tryptophan metabolism (E70.5) E70.5
  2. E72.00
    Disorders of amino-acid transport, unspecified
    BillableCC
  3. E72.01
    Cystinuria
    BillableCC
  4. E72.02
    Hartnup's disease
    BillableCC
  5. E72.03
    Lowe's syndrome
    BillableCC
    Use additional code 1

    Use additional code

    • code for associated glaucoma (H42) H42
  6. E72.04
    Cystinosis
    BillableCC
    Inclusion terms 1, Excludes1 1

    Inclusion terms

    • Fanconi (-de Toni) (-Debré) syndrome with cystinosis

    Excludes1

    • Fanconi (-de Toni) (-Debré) syndrome without cystinosis (E72.09) E72.09
  7. E72.09
    Other disorders of amino-acid transport
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Fanconi (-de Toni) (-Debré) syndrome, unspecified
  8. E72.1
    Disorders of sulfur-bearing amino-acid metabolism
    Header
    Excludes1 3

    Excludes1

    • cystinosis (E72.04) E72.04
    • cystinuria (E72.01) E72.01
    • transcobalamin II deficiency (D51.2) D51.2
  9. E72.10
    Disorders of sulfur-bearing amino-acid metabolism, unspecified
    BillableCC
  10. E72.11
    Homocystinuria
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Cystathionine synthase deficiency
  11. E72.12
    Methylenetetrahydrofolate reductase deficiency
    BillableCC
  12. E72.19
    Other disorders of sulfur-bearing amino-acid metabolism
    BillableCC
    Inclusion terms 3

    Inclusion terms

    • Cystathioninuria
    • Methioninemia
    • Sulfite oxidase deficiency
  13. E72.2
    Disorders of urea cycle metabolism
    Header
    Excludes1 1

    Excludes1

    • disorders of ornithine metabolism (E72.4) E72.4
  14. E72.20
    Disorder of urea cycle metabolism, unspecified
    BillableCC
    Inclusion terms 1, Excludes1 2

    Inclusion terms

    • Hyperammonemia

    Excludes1

    • hyperammonemia-hyperornithinemia-homocitrullinemia syndrome E72.4 E72.4
    • transient hyperammonemia of newborn (P74.6) P74.6
  15. E72.21
    Argininemia
    BillableCC
  16. E72.22
    Arginosuccinic aciduria
    BillableCC
  17. E72.23
    Citrullinemia
    BillableCC
  18. E72.29
    Other disorders of urea cycle metabolism
    BillableCC
  19. E72.3
    Disorders of lysine and hydroxylysine metabolism
    BillableCC
    Inclusion terms 4, Excludes1 3

    Inclusion terms

    • Glutaric aciduria NOS
    • Glutaric aciduria (type I)
    • Hydroxylysinemia
    • Hyperlysinemia

    Excludes1

    • glutaric aciduria type II (E71.313) E71.313
    • Refsum's disease (G60.1) G60.1
    • Zellweger syndrome (E71.510) E71.510
  20. E72.4
    Disorders of ornithine metabolism
    BillableCC
    Inclusion terms 3, Excludes1 1

    Inclusion terms

    • Hyperammonemia-Hyperornithinemia-Homocitrullinemia syndrome
    • Ornithinemia (types I, II)
    • Ornithine transcarbamylase deficiency

    Excludes1

    • hereditary choroidal dystrophy (H31.2-) H31.2
  21. E72.5
    Disorders of glycine metabolism
    Header
  22. E72.50
    Disorder of glycine metabolism, unspecified
    BillableCC
  23. E72.51
    Non-ketotic hyperglycinemia
    BillableCC
  24. E72.52
    Trimethylaminuria
    BillableCC
  25. E72.53
    Primary hyperoxaluria
    Header
    Inclusion terms 2, Excludes1 1

    Inclusion terms

    • Oxalosis
    • Oxaluria

    Excludes1

    • secondary hyperoxaluria (E72.54-) E72.54
  26. E72.530
    Primary hyperoxaluria, type 1
    BillableCC50
  27. E72.538
    Other specified primary hyperoxaluria
    BillableCC50
    Inclusion terms 2

    Inclusion terms

    • Primary hyperoxaluria, type 2
    • Primary hyperoxaluria, type 3
  28. E72.539
    Primary hyperoxaluria, unspecified
    BillableCC50
  29. E72.54
    Secondary hyperoxaluria
    Header
    Excludes1 1

    Excludes1

    • primary hyperoxaluria (E72.53-) E72.53
  30. E72.540
    Dietary hyperoxaluria
    Billable
  31. E72.541
    Enteric hyperoxaluria
    Billable
  32. E72.548
    Other secondary hyperoxaluria
    Billable
  33. E72.549
    Secondary hyperoxaluria, unspecified
    Billable
  34. E72.59
    Other disorders of glycine metabolism
    BillableCC
    Inclusion terms 4

    Inclusion terms

    • D-glycericacidemia
    • Hyperhydroxyprolinemia
    • Hyperprolinemia (types I, II)
    • Sarcosinemia
  35. E72.8
    Other specified disorders of amino-acid metabolism
    Header
  36. E72.81
    Disorders of gamma aminobutyric acid metabolism
    BillableCC
    Inclusion terms 8

    Inclusion terms

    • 4-hydroxybutyric aciduria
    • Disorders of GABA metabolism
    • GABA metabolic defect
    • GABA transaminase deficiency
    • GABA-T deficiency
    • Gamma-hydroxybutyric aciduria
    • SSADHD
    • Succinic semialdehyde dehydrogenase deficiency
  37. E72.89
    Other specified disorders of amino-acid metabolism
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • Disorders of beta-amino-acid metabolism
    • Disorders of gamma-glutamyl cycle
  38. E72.9
    Disorder of amino-acid metabolism, unspecified
    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

At E72

Excludes1

  • disorders of:
  • aromatic amino-acid metabolism (E70.-) E70
  • branched-chain amino-acid metabolism (E71.0-E71.2) E71.0 E71.2
  • fatty-acid metabolism (E71.3) E71.3
  • purine and pyrimidine metabolism (E79.-) E79
  • gout (M1A.-, M10.-) M1A M10

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

  • Cystinuria → E72.01
  • H (Hartnup's) disease → E72.02
  • Hartnup's disease → E72.02
  • Pellagra-cerebellar-ataxia-renal aminoaciduria syndrome → E72.02
  • Lowe's syndrome → E72.03
  • Abderhalden-Kaufmann-Lignac syndrome (cystinosis) → E72.04
  • Cystinosis (malignant) → E72.04
  • Beta-mercaptolactate-cysteine disulfiduria → E72.09
  • De Toni-Fanconi (-Debré) syndrome → E72.09
  • Fanconi (-de Toni)(-Debré) syndrome → E72.09
  • Glycinuria (renal) (with ketosis) → E72.09
  • Lignac (-de Toni) (-Fanconi) (-Debré) disease or syndrome → E72.09
  • Toni-Fanconi syndrome (cystinosis) → E72.09
  • Cystathionine synthase deficiency → E72.11
  • Homocystinuria → E72.11
  • Hyperhomocysteinemia → E72.11
  • Cystathioninemia → E72.19
  • Cystathioninuria → E72.19
  • Hypermethioninemia → E72.19
  • Methioninemia → E72.19
  • Hyperammonemia (congenital) → E72.20
  • Argininemia → E72.21
  • Arginosuccinic aciduria → E72.22
  • Citrullinemia → E72.23
  • Citrullinuria → E72.23
  • Glutaric aciduria → E72.3
  • Hydroxylysinemia → E72.3
  • Hyperlysinemia → E72.3
  • Lysine and hydroxylysine metabolism disorder → E72.3
  • Saccharopinuria → E72.3
  • Hyperornithinemia → E72.4
  • Ornithine metabolism disorder → E72.4
  • Ornithinemia (Type I) (Type II) → E72.4
  • Glucoglycinuria → E72.51
  • Glycinemia → E72.51
  • Hyperglycinemia (non-ketotic) → E72.51
  • Non-ketotic hyperglycinemia → E72.51
  • Trimethylaminuria → E72.52
  • Oxalosis → E72.53
  • Oxaluria → E72.53

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

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