syndromic X-linked intellectual disability Shashi type

MeSH: C537135ORPHA: 85286

Overview

syndromic X-linked intellectual disability characterized by moderate intellectual deficit, obesity, macroorchidism and a characteristic facies that has material basis in mutation in the RBMX gene on chromosome Xq26

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 syndromic X-linked intellectual disability Shashi type, sourced from HPO and Orphanet clinical annotations.

MacroorchidismEverted lower lip vermilionCoarse facial featuresProminent supraorbital ridgesMacrotiaBulbous noseBlepharophimosisDelayed speech and language developmentSeizureObesityModerate intellectual disabilityPalpebral edema

Classification & Codes

MeSH Code

C537135

Orphanet Code

ORPHA:85286
syndromic X-linked intellectual disability Shashi type
MeSHC537135
OrphanetORPHA:85286
Treatments0 drug(s)
Symptoms on record12 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
syndromic X-linked intellectual disability Shashi type | OrphanDrug