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Autosomal dominant myopia-midfacial retrusion-sensorineural hearing loss-rhizomelic dysplasia syndrome

ORPHA:440354Malformation syndromeAutosomal dominantAntenatal, Neonatal

Ассоциированные гены (1)

COL11A1
collagen type XI alpha 1 chain
Disease-causing germline mutation(s) in
OMIM: 120280

Фенотипы (19)

Очень частый (80–99%)6
HP:0000175Cleft palate
HP:0000347Micrognathia
HP:0000774Narrow chest
HP:0002781Upper airway obstruction
HP:0002983Micromelia
HP:0008905Rhizomelia
Частый (30–79%)13
HP:0000162Glossoptosis
HP:0000407Sensorineural hearing impairment
HP:0000520Proptosis
HP:0000882Hypoplastic scapulae
HP:0000947Dumbbell-shaped long bone
HP:0001156Brachydactyly
HP:0001622Premature birth
HP:0002007Frontal bossing
HP:0002980Femoral bowing
HP:0003016Metaphyseal widening
HP:0003097Short femur
HP:0011003High myopia
HP:0011800Midface retrusion

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы