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Autosomal dominant popliteal pterygium syndrome

ORPHA:1300Malformation syndromeAutosomal dominantAntenatal, Neonatal

Ассоциированные гены (1)

IRF6
interferon regulatory factor 6
Disease-causing germline mutation(s) in
OMIM: 607199

Фенотипы (24)

Очень частый (80–99%)6
HP:0000175Cleft palate
HP:0000219Thin upper lip vermilion
HP:0000347Micrognathia
HP:0001387Joint stiffness
HP:0001770Toe syndactyly
HP:0002230Generalized hirsutism
Частый (30–79%)14
HP:0000028Cryptorchidism
HP:0000046Small scrotum
HP:0000048Bifid scrotum
HP:0000059Hypoplastic labia majora
HP:0000772Abnormal rib morphology
HP:0001597Abnormality of the nail
HP:0002650Scoliosis
HP:0006101Finger syndactyly
HP:0008288Nonketotic hyperglycinemia
HP:0009754Fibrous syngnathia
HP:0009755Ankyloblepharon
HP:0009756Popliteal pterygium
HP:0100267Lip pit
HP:0100335Non-midline cleft of the upper lip
Периодический (5–29%)4
HP:0000062Ambiguous genitalia
HP:0000453Choanal atresia
HP:0001171Split hand
HP:0001328Specific learning disability

Эпидемиология (2)

Point prevalence
1-9 / 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