49,XXXXY syndrome

ORPHA: 96264

Overview

chromosomal anomaly of the aneuploidic type characterized by the presence of three 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 49,XXXXY syndrome, sourced from HPO and Orphanet clinical annotations.

Joint hypermobilityAzoospermiaHypogonadismCarious teethAbnormal dental enamel morphologyInfertilityIntellectual disabilityHypotoniaGlobal developmental delayLanguage impairmentDecreased testicular sizeHypoplasia of penisOpen biteCryptorchidismSmall scrotumRenal dysplasiaEpicanthusHypertelorismChronic otitis mediaStrabismusMyopiaBlepharophimosisUpslanted palpebral fissureTaurodontiaDelayed eruption of teethAutismGynecomastiaSeizurePes planusConstipationAsthmaRecurrent respiratory infectionsScoliosisCoxa valgaHip dislocationRadioulnar synostosisElbow dislocationClinodactyly of the 5th fingerAbnormal epiphysis morphologyAttention deficit hyperactivity disorderDown-sloping shouldersCleft palateBrachycephalyMandibular prognathiaWide noseDepressed nasal ridgeShort neckIrritabilityLow frustration toleranceTremorHoloprosencephalyTalipes equinovarusGastroesophageal refluxHypoplasia of the corpus callosumArrhinencephalyPulmonary embolismShort statureVenous thrombosisDepressed nasal bridgeType II diabetes mellitusRenal hypoplasia/aplasiaAbnormal cardiovascular system morphologyOverfriendlinessExcessive shyness

Classification & Codes

Orphanet Code

ORPHA:96264
49,XXXXY syndrome
OrphanetORPHA:96264
Treatments0 drug(s)
Symptoms on record64 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO