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Barber-Say syndrome

ORPHA:1231Malformation syndromeAutosomal dominant, Autosomal recessive, Not applicableNeonatal

Ассоциированные гены (1)

TWIST2
twist family bHLH transcription factor 2
Disease-causing germline mutation(s) in
OMIM: 607556

Фенотипы (23)

Очень частый (80–99%)16
HP:0000154Wide mouth
HP:0000271Abnormality of the face
HP:0000316Hypertelorism
HP:0000365Hearing impairment
HP:0000414Bulbous nose
HP:0000431Wide nasal bridge
HP:0000463Anteverted nares
HP:0000506Telecanthus
HP:0000656Ectropion
HP:0000684Delayed eruption of teeth
HP:0001508Failure to thrive
HP:0001582Redundant skin
HP:0002230Generalized hirsutism
HP:0008065Aplasia/Hypoplasia of the skin
HP:0100840Aplasia/Hypoplasia of the eyebrow
HP:0200102Sparse or absent eyelashes
Частый (30–79%)3
HP:0000974Hyperextensible skin
HP:0002557Hypoplastic nipples
HP:0100783Breast aplasia
Периодический (5–29%)4
HP:0000049Shawl scrotum
HP:0000377Abnormal pinna morphology
HP:0000413Atresia of the external auditory canal
HP:0011224Ablepharon

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