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Infantile osteopetrosis with neuroaxonal dysplasia

ORPHA:85179Malformation syndromeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

OSTM1
osteoclastogenesis associated transmembrane protein 1
Disease-causing germline mutation(s) in
OMIM: 607649

Фенотипы (15)

Частый (30–79%)12
HP:0001250Seizure
HP:0001263Global developmental delay
HP:0001274Agenesis of corpus callosum
HP:0001338Partial agenesis of the corpus callosum
HP:0002059Cerebral atrophy
HP:0002119Ventriculomegaly
HP:0004330Increased skull ossification
HP:0006824Cranial nerve paralysis
HP:0009830Peripheral neuropathy
HP:0012444Brain atrophy
HP:0012447Abnormal myelination
HP:0025517Hypoplastic hippocampus
Периодический (5–29%)3
HP:0000405Conductive hearing impairment
HP:0002090Pneumonia
HP:0025116Fetal distress

Эпидемиология (1)

Point prevalence
<1 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы