LD2A.4

ICD-11 MMS 2026-01

46,XY disorder of sex development due to androgen resistance

Description

Androgen insensitivity syndrome (AIS) is a disorder of sex development (DSD) characterised by the presence of female external genitalia, ambiguous genitalia or variable defects in virilization in a 46,XY individual with absent or partial responsiveness to age-appropriate levels of androgens. It comprises two clinical subgroups: complete AIS (CAIS) and partial AIS (PAIS).

Index terms

  • 46,XY disorder of sex development due to androgen resistance
  • Androgen insensitivity syndrome
  • Androgen resistance syndrome
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  • Goldberg-Maxwell syndrome
  • Goldberg-Morris syndrome
  • Male pseudohermaphroditism with androgen resistance
  • Morris syndrome
  • TFM - [testicular feminisation syndrome]
  • Testicular feminization syndrome
  • male pseudohermaphroditism with feminizing testis
  • peripheral androgen receptor disorder
  • syndrome of feminising testes
  • syndrome of feminizing testes
  • testicular feminisation
  • testicular feminisation syndrome
  • Complete peripheral androgen resistance syndrome
  • 46,XY DSD due to complete androgen insensitivity
  • 46,XY disorder of sex development due to complete androgen insensitivity
  • Complete androgen insensitivity syndrome
  • complete androgen resistance syndrome
  • Partial peripheral androgen resistance syndrome
  • 46,XY DSD due to partial androgen insensitivity
  • 46,XY disorder of sex development due to partial androgen insensitivity
  • Partial androgen insensitivity
  • Reifenstein syndrome
  • partial androgen resistance syndrome

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.3 – 46,XY disorder of sex development due to a defect in testosterone metabolism
  • 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 androgen resistance – http://id.who.int/icd/entity/1688293388

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