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Focal facial dermal dysplasia type III

ORPHA:1807Clinical subtypeAutosomal dominant, Autosomal recessiveAntenatal, Neonatal

Ассоциированные гены (1)

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

Фенотипы (23)

Очень частый (80–99%)9
HP:0000457Depressed nasal ridge
HP:0001582Redundant skin
HP:0002714Downturned corners of mouth
HP:0007495Prematurely aged appearance
HP:0008065Aplasia/Hypoplasia of the skin
HP:0008070Sparse hair
HP:0010720Abnormal hair pattern
HP:0010751Chin dimple
HP:0100781Abnormality of the sacroiliac joint
Частый (30–79%)9
HP:0000286Epicanthus
HP:0000322Short philtrum
HP:0000431Wide nasal bridge
HP:0002023Anal atresia
HP:0002553Highly arched eyebrow
HP:0005338Sparse lateral eyebrow
HP:0007776Sparse lower eyelashes
HP:0009743Distichiasis
HP:0010935Abnormality of the upper urinary tract
Периодический (5–29%)5
HP:0000486Strabismus
HP:0000494Downslanted palpebral fissures
HP:0000632Lacrimation abnormality
HP:0001053Hypopigmented skin patches
HP:0007565Multiple cafe-au-lait spots

Эпидемиология (2)

Point prevalence
Unknown
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы