LB45.1

ICD-11 MMS 2026-01

46,XX gonadal dysgenesis

Description

Karyotype 46 XX; Gonads: gonadal dysgenesis (streak gonads); Phenotype female with symptoms like primary amenorrhea, hypergonadotropic hypogonadism, normal stature and no other abnormalities.

Index terms

  • 46,XX gonadal dysgenesis
  • 46,XX complete gonadal dysgenesis
  • 46,XX pure gonadal dysgenesis
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  • Dysgenetic ovaries
  • FSH-RO - [follicular stimulating hormone-resistant ovaries]
  • Follicular stimulating hormone-resistant ovaries
  • Hypergonadotropic ovarian dysgenesis
  • Resistant ovary syndrome

Exclusions from above levels

  • LD2A – Disorders of sex development leading to sexual ambiguity
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LB45.0 – Congenital absence of ovary
  • LB45.2 – Developmental ovarian cyst
  • LB45.3 – Congenital torsion of ovary
  • LB45.4 – Accessory ovary
  • LB45.5 – Congenital absence of fallopian tube
  • LB45.6 – Atresia of fallopian tube
  • LB45.7 – Accessory fallopian tube
  • LB45.8 – Embryonic cyst of fallopian tube
  • LB45.9 – Embryonic cyst of broad ligament
  • LB45.Y – Other specified structural developmental anomalies of ovaries, fallopian tubes or broad ligaments
  • LB45.Z – Structural developmental anomalies of ovaries, fallopian tubes or broad ligaments, unspecified

Foundation entity

46,XX gonadal dysgenesis – http://id.who.int/icd/entity/1742528605

Allowed postcoordination axes

Part of: LB45: Structural developmental anomalies of ovaries, fallopian tubes or broad ligaments › 20: Developmental anomalies