Excludes1

  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan's syndrome (Q87.4)
  • 5-alpha-reductase deficiency (E29.1)

Excludes2

  • Ehlers-Danlos syndromes (Q79.6-)
  1. E74 Other disorders of carbohydrate metabolism

    Excludes1

    1. E74.0 Glycogen storage disease

      1. E74.00 Glycogen storage disease, unspecified

      2. E74.01 von Gierke disease

        Inclusion term(s):

        • Type I glycogen storage disease
      3. E74.02 Pompe disease

        Inclusion term(s):

        • Cardiac glycogenosis
        • Type II glycogen storage disease
      4. E74.03 Cori disease

        Inclusion term(s):

        • Forbes disease
        • Type III glycogen storage disease
      5. E74.04 McArdle disease

        Inclusion term(s):

        • Type V glycogen storage disease
      6. E74.09 Other glycogen storage disease

        Inclusion term(s):

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

      1. E74.10 Disorder of fructose metabolism, unspecified

      2. E74.11 Essential fructosuria

        Inclusion term(s):

        • Fructokinase deficiency
      3. E74.12 Hereditary fructose intolerance

        Inclusion term(s):

        • Fructosemia
      4. E74.19 Other disorders of fructose metabolism

        Inclusion term(s):

        • Fructose-1, 6-diphosphatase deficiency
    3. E74.2 Disorders of galactose metabolism

      1. E74.20 Disorders of galactose metabolism, unspecified

      2. E74.21 Galactosemia

      3. E74.29 Other disorders of galactose metabolism

        Inclusion term(s):

        • Galactokinase deficiency
    4. E74.3 Other disorders of intestinal carbohydrate absorption

      1. E74.31 Sucrase-isomaltase deficiency

      2. E74.39 Other disorders of intestinal carbohydrate absorption

        Inclusion term(s):

        • Disorder of intestinal carbohydrate absorption NOS
        • Glucose-galactose malabsorption
        • Sucrase deficiency
    5. E74.4 Disorders of pyruvate metabolism and gluconeogenesis

      Inclusion term(s):

      • Deficiency of phosphoenolpyruvate carboxykinase
      • Deficiency of pyruvate carboxylase
      • Deficiency of pyruvate dehydrogenase
    6. E74.8 Other specified disorders of carbohydrate metabolism

      1. E74.81 Disorders of glucose transport, not elsewhere classified

        • E74.810 Glucose transporter protein type 1 deficiency

          Inclusion term(s):

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

          Inclusion term(s):

          • (Familial) renal glycosuria
        • E74.819 Disorders of glucose transport, unspecified
      2. E74.89 Other specified disorders of carbohydrate metabolism

        Inclusion term(s):

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

Abbreviations used here:

NEC Not elsewhere classifiable
This abbreviation in the Tabular List represents “other specified”. When a specific code is not available for a condition, the Tabular List includes an NEC entry under a code to identify the code as the “other specified” code.

NOS Not otherwise specified
This abbreviation is the equivalent of unspecified.