monosomy X

ORPHA: 99226

Overview

human disease

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 monosomy X, sourced from HPO and Orphanet clinical annotations.

Abnormal pinna morphologyLymphedemaVitiligoAbnormal fingernail morphologyHip dysplasiaHepatic fibrosisAlopeciaAtrial septal defectBicuspid aortic valveProlonged QT intervalMyocardial infarctionCoarctation of aortaPes planusHyperconvex fingernailsShort toeCeliac diseaseCholestatic liver diseaseScoliosisAutoimmunityMadelung deformityInverted nipplesNevusReduced bone mineral densityNumerous congenital melanocytic neviType II diabetes mellitusAttention deficit hyperactivity disorderAplasia/Hypoplasia of the mandibleSplayed toesDelayed early-childhood social milestone developmentThyroiditisGonadoblastomaGastrointestinal angiodysplasiaCirrhosisInflammation of the large intestineBiliary cirrhosisAortic dissectionMelanomaHypoplastic left ventricleGastrointestinal inflammationArterial dissectionRenal hypoplasia/aplasiaNeurodevelopmental delayAbnormality of the ovaryShort neckDelayed pubertyIncreased circulating gonadotropin levelShort sternumOsteopeniaOsteoporosisGrowth delayIntrauterine growth retardationDelayed skeletal maturationCubitus valgusHigh urinary gonadotropin levelShort statureWide intermamillary distanceAplasia/Hypoplasia of the nipplesPremature ovarian insufficiencyFemale infertilityPostnatal growth retardationIncreased upper to lower segment ratioAbnormal forearm bone morphologyEnlarged thoraxHigh palateRetrognathiaMicrognathiaHearing impairmentLow-set earsRecurrent otitis mediaWebbed neckThickened nuchal skin foldBroad neckAtypical behaviorAnxietyAbnormal nonverbal communicative behaviorPrimary amenorrheaHypertensionSecondary amenorrheaHashimoto thyroiditisShield chestSpecific learning disabilityHepatic steatosisObesityFailure to thrive in infancyHypoplastic toenailsGlucose intoleranceLow posterior hairlineHigh, narrow palateKyphosisGenu valgumElevated circulating hepatic transaminase concentrationAortic arch aneurysmDermatoglyphic ridges abnormalEnlargement of the distal femoral epiphysisIrregular proximal tibial epiphysesAbnormal dermatoglyphicsNeck pterygiaShort 4th metacarpalShort 5th metacarpalHypermobility of toe jointsHorseshoe kidneyEctopic kidneyAbnormality of the dentitionEpicanthusCystic hygromaStrabismusPtosisMyopiaDepressionPectus excavatumHyperinsulinemiaAtypical scarring of skinMelanocytic nevus

Classification & Codes

Orphanet Code

ORPHA:99226
monosomy X
OrphanetORPHA:99226
Treatments0 drug(s)
Symptoms on record113 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO