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Blepharo-cheilo-odontic syndrome

ORPHA:1997Malformation syndromeAutosomal dominantNeonatal

Ассоциированные гены (2)

CTNND1
catenin delta 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 601045
CDH1
cadherin 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 192090

Фенотипы (14)

Очень частый (80–99%)3
HP:0000492Abnormal eyelid morphology
HP:0007651Ectropion of lower eyelids
HP:0009743Distichiasis
Частый (30–79%)8
HP:0000316Hypertelorism
HP:0000405Conductive hearing impairment
HP:0000478Abnormality of the eye
HP:0000504Abnormality of vision
HP:0000670Carious teeth
HP:0000698Conical tooth
HP:0006101Finger syndactyly
HP:0012905Euryblepharon
Периодический (5–29%)3
HP:0002023Anal atresia
HP:0011362Abnormal hair quantity
HP:0200040Epidermoid cyst

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