mixed gonadal dysgenesis X0/XY

MeSH: D006060ORPHA: 1772

Overview

intersex variation

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with mixed gonadal dysgenesis X0/XY, sourced from HPO and Orphanet clinical annotations.

CryptorchidismShort statureMuscle hypertrophy of the lower extremitiesUnilateral cryptorchidismAzoospermiaAmbiguous genitalia, maleHypospadiasMicropenisAmbiguous genitalia, femaleAmbiguous genitaliaPenoscrotal hypospadiasAbnormal internal genitaliaIncreased circulating gonadotropin levelMale infertilityBilateral cryptorchidismUrogenital sinus anomalyEpispadiasChordeeAbnormal scrotum morphologyBifid scrotumAbnormality of the kidneyHorseshoe kidneyGonadoblastomaHigh palateEpicanthusMicrognathiaHearing impairmentRecurrent otitis mediaWebbed neckVisual impairmentNystagmusAutistic behaviorPectus excavatumGynecomastiaHypothyroidismDelayed pubertyDevelopmental glaucomaMild intellectual disabilityObesityBicuspid aortic valveTachycardiaProlonged QT intervalCoarctation of aortaHallux valgusLow posterior hairlineNail dysplasiaDyscalculiaScoliosisDelayed skeletal maturationCubitus valgusWide intermamillary distanceShort 4th metacarpalStreak ovaryShort metatarsalOvotestisOvarian serous cystadenomaCervix cancerAbnormal cardiovascular system morphologyDecreased serum testosterone concentrationPosteriorly rotated ears

Classification & Codes

MeSH Code

D006060

Orphanet Code

ORPHA:1772
mixed gonadal dysgenesis X0/XY
MeSHD006060
OrphanetORPHA:1772
Treatments0 drug(s)
Symptoms on record60 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
mixed gonadal dysgenesis X0/XY | OrphanDrug