X-linked diffuse leiomyomatosis-Alport syndrome

MeSH: C537113ORPHA: 1018

Overview

The association of X-linked Alport syndrome with leiomyomatosis of the esophagus, tracheobronchial tree or female genitals has been reported in more than 30 families

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 X-linked diffuse leiomyomatosis-Alport syndrome, sourced from HPO and Orphanet clinical annotations.

DysphagiaAbnormal esophagus morphologyAbnormal gastrointestinal tract morphologyEsophageal neoplasmProteinuriaNephropathySensorineural hearing impairmentAbnormality of the eyeCataractHematuriaFailure to thriveVomitingGastroesophageal refluxAchalasiaAnti-smooth muscle antibody positivityDiffuse leiomyomatosisAbnormality of the female genitaliaChronic kidney diseaseChest painHypoperistalsisDyspepsiaKeratitisMyopiaWeight lossDyspneaRecurrent respiratory infectionsStage 5 chronic kidney diseaseTracheobronchial leiomyomatosisEsophageal stenosisFibromaUterine neoplasmAnterior lenticonusAspiration pneumoniaAbnormal respiratory system morphologyCoughVulvar neoplasmPrecordial painNasogastric tube feedingVaginal neoplasm

Classification & Codes

MeSH Code

C537113

Orphanet Code

ORPHA:1018
X-linked diffuse leiomyomatosis-Alport syndrome
MeSHC537113
OrphanetORPHA:1018
Treatments0 drug(s)
Symptoms on record39 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO