49,XXXYY syndrome

ORPHA: 261534

Overview

chromosomal disorder

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 49,XXXYY syndrome, sourced from HPO and Orphanet clinical annotations.

Male hypogonadismMicropenisAmbiguous genitaliaEpicanthusMandibular prognathiaHypertelorismMicrognathiaWide nasal bridgeAtypical behaviorAutistic behaviorLow frustration toleranceDelayed speech and language developmentGlobal developmental delayGynecomastiaNarrow chestExternal genital hypoplasiaIncreased circulating gonadotropin levelDelayed skeletal maturationIntellectual disabilityBilateral talipes equinovarusAbnormal facial shapeVentriculomegalyAbnormal cerebral white matter morphologyRecurrent upper respiratory tract infectionsEunuchoid habitusPrimary gonadal insufficiencyDecreased testicular sizeAbnormal plantar dermatoglyphicsProminent foreheadModerate global developmental delayFinger clinodactylyDecreased serum testosterone concentrationAbnormality of the testis sizePosteriorly rotated ears

Classification & Codes

Orphanet Code

ORPHA:261534
49,XXXYY syndrome
OrphanetORPHA:261534
Treatments0 drug(s)
Symptoms on record34 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO