autosomal recessive osteopetrosis 6

MeSH: C566931ORPHA: 210110

Overview

osteopetrosis characterized by autosomal recessive inheritance of that has material basis in mutation in the PLEKHM1 gene on chromosome 17q21.31

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 autosomal recessive osteopetrosis 6, sourced from HPO and Orphanet clinical annotations.

Abnormality of bone mineral densityIncreased susceptibility to fracturesRecurrent fracturesBack painSandwich appearance of vertebral bodiesErlenmeyer flask deformity of the femursCortical sclerosisOsteosclerosis of the base of the skullGeneralized osteosclerosisAbnormality of the nervous systemAnemiaElevated circulating alkaline phosphatase concentrationAbnormality of the dentitionVisual impairmentDental malocclusionCranial nerve compressionHepatosplenomegalyOsteomyelitisAbnormal dental morphologyOptic atrophy from cranial nerve compressionChronic infection

Classification & Codes

MeSH Code

C566931

Orphanet Code

ORPHA:210110
autosomal recessive osteopetrosis 6
MeSHC566931
OrphanetORPHA:210110
Treatments0 drug(s)
Symptoms on record21 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
autosomal recessive osteopetrosis 6 | OrphanDrug