DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E74 Other disorders of carbohydrate metabolism

  • Header · not billable

E74 is not billable. The category heads 24 billable codes in FY2027, grouped under 7 subdivisions.

Index → code

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E74 Other disorders of carbohydrate metabolism
Billable codes
24
Subdivisions
7
CC / MCC codes
17 / 0
HCC V28 codes
7
New FY2027
0
Index entries
150

Codes in E74

  1. E74.0
    Glycogen storage disease
    Header
  2. E74.00
    Glycogen storage disease, unspecified
    BillableCC50
  3. E74.01
    von Gierke disease
    BillableCC50
    Inclusion terms 1

    Inclusion terms

    • Type I glycogen storage disease
  4. E74.02
    Pompe disease
    BillableCC49
    Inclusion terms 2

    Inclusion terms

    • Cardiac glycogenosis
    • Type II glycogen storage disease
  5. E74.03
    Cori disease
    BillableCC50
    Inclusion terms 2

    Inclusion terms

    • Forbes disease
    • Type III glycogen storage disease
  6. E74.04
    McArdle disease
    BillableCC50
    Inclusion terms 1

    Inclusion terms

    • Type V glycogen storage disease
  7. E74.05
    Lysosome-associated membrane protein 2 [LAMP2] deficiency
    BillableCC50
    Inclusion terms 1, Code also 3

    Inclusion terms

    • Danon disease

    Code also

    • , if applicable, associated manifestations such as:
    • dilated cardiomyopathy (I42.0-) I42.0
    • obstructive hypertrophic cardiomyopathy (I42.1) I42.1
  8. E74.09
    Other glycogen storage disease
    BillableCC50
    Inclusion terms 6

    Inclusion terms

    • Andersen disease
    • Glycogen storage disease, types 0, IV, VI-XI
    • Hers disease
    • Liver phosphorylase deficiency
    • Muscle phosphofructokinase deficiency
    • Tauri disease
  9. E74.1
    Disorders of fructose metabolism
    Header
    Excludes1 1

    Excludes1

    • muscle phosphofructokinase deficiency (E74.09) E74.09
  10. E74.10
    Disorder of fructose metabolism, unspecified
    Billable
  11. E74.11
    Essential fructosuria
    Billable
    Inclusion terms 1

    Inclusion terms

    • Fructokinase deficiency
  12. E74.12
    Hereditary fructose intolerance
    Billable
    Inclusion terms 1

    Inclusion terms

    • Fructosemia
  13. E74.19
    Other disorders of fructose metabolism
    Billable
    Inclusion terms 1

    Inclusion terms

    • Fructose-1, 6-diphosphatase deficiency
  14. E74.2
    Disorders of galactose metabolism
    Header
  15. E74.20
    Disorders of galactose metabolism, unspecified
    BillableCC
  16. E74.21
    Galactosemia
    BillableCC
  17. E74.29
    Other disorders of galactose metabolism
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Galactokinase deficiency
  18. E74.3
    Other disorders of intestinal carbohydrate absorption
    Header
    Excludes2 1

    Excludes2

    • lactose intolerance (E73.-) E73
  19. E74.31
    Sucrase-isomaltase deficiency
    Billable
  20. E74.39
    Other disorders of intestinal carbohydrate absorption
    Billable
    Inclusion terms 3

    Inclusion terms

    • Disorder of intestinal carbohydrate absorption NOS
    • Glucose-galactose malabsorption
    • Sucrase deficiency
  21. E74.4
    Disorders of pyruvate metabolism and gluconeogenesis
    BillableCC
    Inclusion terms 3, Excludes1 2

    Inclusion terms

    • Deficiency of phosphoenolpyruvate carboxykinase
    • Deficiency of pyruvate carboxylase
    • Deficiency of pyruvate dehydrogenase

    Excludes1

    • disorders of pyruvate metabolism and gluconeogenesis with anemia (D55.-) D55
    • Leigh's syndrome (G31.82) G31.82
  22. E74.8
    Other specified disorders of carbohydrate metabolism
    Header
  23. E74.81
    Disorders of glucose transport, not elsewhere classified
    Header
  24. E74.810
    Glucose transporter protein type 1 deficiency
    BillableCC
    Inclusion terms 5

    Inclusion terms

    • De Vivo syndrome
    • Glucose transport defect, blood-brain barrier
    • Glut1 deficiency
    • GLUT1 deficiency syndrome 1, infantile onset
    • GLUT1 deficiency syndrome 2, childhood onset
  25. E74.818
    Other disorders of glucose transport
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • (Familial) renal glycosuria
  26. E74.819
    Disorders of glucose transport, unspecified
    BillableCC
  27. E74.82
    Disorders of citrate metabolism
    Header
  28. E74.820
    SLC13A5 Citrate Transporter Disorder
    BillableCC
  29. E74.829
    Other disorders of citrate metabolism
    BillableCC
  30. E74.89
    Other specified disorders of carbohydrate metabolism
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Essential pentosuria
  31. E74.9
    Disorder of carbohydrate metabolism, unspecified
    Billable

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 E74

Excludes1

  • diabetes mellitus (E08-E13) E08 E13
  • hypoglycemia NOS (E16.2) E16.2
  • increased secretion of glucagon (E16.3) E16.3
  • mucopolysaccharidosis (E76.0-E76.3) E76.0 E76.3

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

  • Aglycogenosis → E74.00
  • Gierke's disease (glycogenosis I) → E74.01
  • Van Creveld-von Gierke disease → E74.01
  • Von Gierke's disease → E74.01
  • Cardiomegalia glycogenica diffusa → E74.02 [I43]
  • Pompe's disease (glycogen storage) → E74.02
  • Amylopectinosis (brancher enzyme deficiency) → E74.03
  • Cori's disease (glycogen storage) → E74.03
  • Dextrinosis, limit (debrancher enzyme deficiency) → E74.03
  • Forbes' glycogen storage disease → E74.03
  • McArdle (-Schmid)(-Pearson) disease (glycogen storage) → E74.04
  • Andersen's disease (glycogen storage) → E74.09
  • Hers' disease → E74.09
  • Tauri's disease → E74.09
  • Fructokinase deficiency → E74.11
  • Fructosuria (benign) (essential) → E74.11
  • Fructosemia (benign) (essential) → E74.12
  • Fructose 1,6 diphosphatase deficiency → E74.19
  • Hyperfructosemia → E74.19
  • Galactosemia (classic) (congenital) → E74.21
  • Galactokinase deficiency → E74.29
  • Galactosuria → E74.29
  • Glucose-galactose malabsorption → E74.39
  • GLUT1 deficiency syndrome 1, infantile onset → E74.810
  • GLUT1 deficiency syndrome 2, childhood onset → E74.810
  • Pentosuria (essential) → E74.89
  • Disease, diseasedglycogen storage → E74.00
  • Deficiency, deficientglucose-6-phosphatase → E74.01
  • Disease, diseasedGierke's (glycogenosis I) → E74.01
  • Disease, diseasedvan Creveld-von Gierke (glycogenosis I) → E74.01
  • Disease, diseasedvon Gierke's (glycogenosis I) → E74.01
  • Cardiomegalyglycogen → E74.02 [I43]
  • Cardiomyopathy (familial) (idiopathic)glycogen storage → E74.02 [I43]
  • Deficiency, deficientlysosomal alpha-1, 4 glucosidase → E74.02
  • Disease, diseasedPompe's (glycogenosis II) → E74.02
  • Glycogenosis (diffuse) (generalized)cardiac → E74.02 [I43]
  • Myocardiopathy (congestive) (constrictive) (familial) (hypertrophic nonobstructive) (idiopathic) (infiltrative) (obstructive) (primary) (restrictive) (sporadic)glycogen storage → E74.02 [I43]
  • Deficiency, deficientbrancher enzyme (amylopectinosis) → E74.03
  • Deficiency, deficientdebrancher enzyme (limit dextrinosis) → E74.03
  • Disease, diseasedCori's (glycogenosis III) → E74.03

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

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