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Otofaciocervical syndrome

ORPHA:2792Malformation syndromeAutosomal dominant, Autosomal recessiveAntenatal, Neonatal

Ассоциированные гены (2)

EYA1
EYA transcriptional coactivator and phosphatase 1
Disease-causing germline mutation(s) in
OMIM: 601653
PAX1
paired box 1
Disease-causing germline mutation(s) in
OMIM: 167411

Фенотипы (23)

Очень частый (80–99%)18
HP:0000218High palate
HP:0000293Full cheeks
HP:0000400Macrotia
HP:0000405Conductive hearing impairment
HP:0000411Protruding ear
HP:0000463Anteverted nares
HP:0000889Abnormality of the clavicle
HP:0001249Intellectual disability
HP:0001263Global developmental delay
HP:0001276Hypertonia
HP:0001347Hyperreflexia
HP:0002167Abnormality of speech or vocalization
HP:0003691Scapular winging
HP:0004322Short stature
HP:0004467Preauricular pit
HP:0005280Depressed nasal bridge
HP:0007477Abnormal dermatoglyphics
HP:0200021Down-sloping shoulders
Частый (30–79%)2
HP:0002750Delayed skeletal maturation
HP:0009738Abnormality of the antihelix
Периодический (5–29%)3
HP:0000324Facial asymmetry
HP:0000413Atresia of the external auditory canal
HP:0008678Renal hypoplasia/aplasia

Эпидемиология (2)

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

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы