XXXY syndrome

ORPHA: 96263

Overview

chromosomal anomaly of the aneuploidic type characterized by the presence of two extra X chromosomes in males

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 XXXY syndrome, sourced from HPO and Orphanet clinical annotations.

Joint hypermobilityAggressive behaviorAzoospermiaHypogonadismInfertilityMild intellectual disabilityGlobal developmental delayLanguage impairmentDecreased testicular sizeCryptorchidismSmall scrotumTall statureEpicanthusHypertelorismChronic otitis mediaDepressed nasal ridgeStrabismusUpslanted palpebral fissureCarious teethTaurodontiaAbnormal dental enamel morphologyDelayed eruption of teethAutismGynecomastiaHypotoniaPes planusConstipationAsthmaRecurrent respiratory infectionsRadioulnar synostosisElbow dislocationClinodactyly of the 5th fingerAbnormal epiphysis morphologyAttention deficit hyperactivity disorderHypoplasia of penisOpen biteDown-sloping shouldersInguinal herniaRenal dysplasiaCleft palateBrachycephalyMandibular prognathiaFacial asymmetryShort neckBlepharophimosisIrritabilityAnxietyDelayed speech and language developmentSeizureTremorObesityTalipes equinovarusGastroesophageal refluxPulmonary embolismScoliosisCoxa valgaHip dislocationVenous thrombosisType II diabetes mellitusAbnormal social behaviorAbnormal cardiovascular system morphologySchizophrenia

Classification & Codes

Orphanet Code

ORPHA:96263
XXXY syndrome
OrphanetORPHA:96263
Treatments0 drug(s)
Symptoms on record62 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
XXXY syndrome | OrphanDrug