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


  • Ehlers-Danlos syndromes (Q79.6-)
  1. E77 Disorders of glycoprotein metabolism

    1. E77.0 Defects in post-translational modification of lysosomal enzymes

      Inclusion term(s):

      • Mucolipidosis II [I-cell disease]
      • Mucolipidosis III [pseudo-Hurler polydystrophy]
    2. E77.1 Defects in glycoprotein degradation

      Inclusion term(s):

      • Aspartylglucosaminuria
      • Fucosidosis
      • Mannosidosis
      • Sialidosis [mucolipidosis I]
    3. E77.8 Other disorders of glycoprotein metabolism

    4. E77.9 Disorder of glycoprotein 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.