Kleefstra syndrome due to 9q34 microdeletion

ORPHA: 96147

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 Kleefstra syndrome due to 9q34 microdeletion, sourced from HPO and Orphanet clinical annotations.

Everted lower lip vermilionBrachycephalyHypertelorismAnteverted naresIntellectual disabilityHypotoniaGlobal developmental delaySpecific learning disabilityMutismAphasiaHighly arched eyebrowShort noseFlat occiputEcholaliaCryptorchidismAbnormal testis morphologyMacroglossiaMicrocephalySynophrysAutismSeizureObesityAbnormal cardiac septum morphologyGeneralized non-motor (absence) seizureStatus epilepticusDownturned corners of mouthProtruding tongueEpileptic spasmMidface retrusionInguinal herniaVesicoureteral refluxRenal insufficiencyHearing impairmentAtypical behaviorDepressionIrritabilityAnxietyApathyAgenesis of corpus callosumAbsent septum pellucidumFailure to thriveGrowth delayTetralogy of FallotAortic valve stenosisAortic regurgitationCoarctation of aortaConotruncal defectVentriculomegalyCerebral cortical atrophySleep disturbanceHypoplasia of penisFeeding difficultiesSubcortical cerebral atrophyCerebral cortical hemiatrophyFemoral hernia

Classification & Codes

Orphanet Code

ORPHA:96147
Kleefstra syndrome due to 9q34 microdeletion
OrphanetORPHA:96147
Treatments0 drug(s)
Symptoms on record55 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO