KBG syndrome

MeSH: C537015ORPHA: 2332

Overview

syndrome that is characterized by short stature, moderate to severe degrees of mental retardation, developmental abnormalities of the limbs, bones of the spine (vertebrae), extremities, and/or underdevelopment of the bones of the skeleton

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 KBG syndrome, sourced from HPO and Orphanet clinical annotations.

Long philtrumMacrotiaProminent nasal bridgeUnderdeveloped nasal alaeAnteverted naresWebbed neckShort neckStrabismusTelecanthusThick eyebrowLong palpebral fissureSynophrysOligodontiaCervical ribsSingle transverse palmar creaseGlobal developmental delayWidely-spaced maxillary central incisorsMacrodontiaScoliosisCryptorchidismCleft palateThin upper lip vermilionMicrocephalyHypertelorismTriangular faceDelayed skeletal maturationThoracic kyphosisVertebral fusionShort statureBilateral conductive hearing impairmentAbnormal hair patternAbnormal skeletal morphologyFeeding difficultiesCutaneous syndactylyFinger clinodactylyRound faceSeizureEEG abnormalityPersistent open anterior fontanelleCongenital malformation of the left heart

Classification & Codes

MeSH Code

C537015

Orphanet Code

ORPHA:2332
KBG syndrome
MeSHC537015
OrphanetORPHA:2332
Treatments0 drug(s)
Symptoms on record40 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO