XXYY syndrome

ORPHA: 10

Overview

chromosomal variation of the aneuploidic type characterized by the presence of an extra X and Y chromosome

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 XXYY syndrome, sourced from HPO and Orphanet clinical annotations.

Joint hypermobilityAzoospermiaInfertilityHypergonadotropic hypogonadismIntellectual disabilityGlobal developmental delayAbnormal speech patternDecreased testicular sizeTall statureThick lower lip vermilionEpicanthusHypertelorismChronic otitis mediaStrabismusBlepharophimosisUpslanted palpebral fissureCarious teethTaurodontiaAbnormal dental enamel morphologyDelayed eruption of teethDepressionAnxietyGynecomastiaHypotoniaTremorObesityPes planusConstipationAsthmaRecurrent respiratory infectionsRadioulnar synostosisElbow dislocationAbnormal shoulder morphologyClinodactyly of the 5th fingerFlat occiputAttention deficit hyperactivity disorderFeeding difficulties in infancyOpen biteInguinal herniaCryptorchidismCleft palateLong faceFacial asymmetryNystagmusPsychosisAutismMotor stereotypySeizureAtaxiaDysarthriaHip dysplasiaTalipesGastroesophageal refluxApneaVentriculomegalyScoliosisLymphomaType II diabetes mellitusHypoplasia of penisBroad jawAbnormal cardiovascular system morphology

Classification & Codes

Orphanet Code

ORPHA:10
XXYY syndrome
OrphanetORPHA:10
Treatments0 drug(s)
Symptoms on record61 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO