LD2A.1

ICD-11 MMS 2026-01

46,XY gonadal dysgenesis

Description

This is any congenital developmental disorder of the reproductive system characterised by a progressive loss of primordial germ cells on the developing gonads of an embryo.

Index terms

  • 46,XY gonadal dysgenesis
  • 46,XY pure gonadal dysgenesis
  • Female with 46,XY karyotype
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  • Gonadal dysgenesis, XY female type
  • Swyer syndrome
  • xy females
  • Complete 46,XY gonadal dysgenesis
  • Partial 46,XY gonadal dysgenesis
  • 46,XY gonadal dysgenesis - motor and sensory neuropathy

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.2 – Testicular agenesis
  • LD2A.3 – 46,XY disorder of sex development due to a defect in testosterone metabolism
  • 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 gonadal dysgenesis – http://id.who.int/icd/entity/1844256276

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