LD2A.3

ICD-11 MMS 2026-01

46,XY disorder of sex development due to a defect in testosterone metabolism

Index terms

  • 46,XY disorder of sex development due to a defect in testosterone metabolism
  • 46,XY DSD due to a defect in testosterone metabolism
  • 46,XY disorder of sex development due to 17-beta-hydroxysteroid dehydrogenase 3 deficiency
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  • 17-beta-hydroxysteroid dehydrogenase 3 deficiency
  • 17-ketoreductase deficiency
  • 17-ketosteroidreductase deficiency
  • 46,XY DSD due to 17-beta-hydroxysteroid dehydrogenase 3 deficiency
  • Male pseudohermaphroditism due to 17-beta-hydroxysteroid dehydrogenase 3 deficiency
  • 46,XY disorder of sex development due to 5-alpha-reductase 2 deficiency
  • 46,XY DSD due to 5-alpha-reductase 2 deficiency
  • Male pseudohermaphroditism due to 5-alpha-reductase 2 deficiency
  • 46,XY disorder of sex development due to isolated 17, 20 lyase deficiency
  • 46,XY DSD due to isolated 17, 20 lyase deficiency
  • Leydig cell hypoplasia
  • 46,XY DSD due to LH defects
  • 46,XY disorder of sex development due to LH defects
  • LH resistance due to LH receptor inactivation
  • Male pseudohermaphrodism due to LH defects
  • LH resistance due to complete LH receptor inactivation
  • LH resistance due to partial LH receptor inactivation

Excludes

  • 5A71.01 – Congenital adrenal hyperplasia

Exclusions from above levels

  • 5A71 – pseudohermaphroditism: female, with adrenocortical disorder
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LD2A.0 – Ovotesticular disorder of sex development
  • LD2A.1 – 46,XY gonadal dysgenesis
  • LD2A.2 – Testicular agenesis
  • LD2A.4 – 46,XY disorder of sex development due to androgen resistance
  • LD2A.Y – Other specified malformative disorders of sex development
  • LD2A.Z – Malformative disorders of sex development, unspecified

Foundation entity

46,XY disorder of sex development due to a defect in testosterone metabolism – http://id.who.int/icd/entity/749282256

Part of: LD2A: Malformative disorders of sex development › 20: Developmental anomalies