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Hidrotic ectodermal dysplasia

ORPHA:189DiseaseAutosomal dominantChildhood

Ассоциированные гены (1)

GJB6
gap junction protein beta 6
Disease-causing germline mutation(s) in
OMIM: 604418

Фенотипы (27)

Частый (30–79%)7
HP:0000653Sparse eyelashes
HP:0000972Palmoplantar hyperkeratosis
HP:0000982Palmoplantar keratoderma
HP:0001596Alopecia
HP:0008070Sparse hair
HP:0008404Nail dystrophy
HP:0045075Sparse eyebrow
Периодический (5–29%)20
HP:0000365Hearing impairment
HP:0000953Hyperpigmentation of the skin
HP:0001072Thickened skin
HP:0001792Small nail
HP:0001798Anonychia
HP:0002209Sparse scalp hair
HP:0002213Fine hair
HP:0002215Sparse axillary hair
HP:0002221Absent axillary hair
HP:0002223Absent eyebrow
HP:0002225Sparse pubic hair
HP:0002555Absent pubic hair
HP:0004528Generalized hypotrichosis
HP:0004779Brittle scalp hair
HP:0005599Hypopigmentation of hair
HP:0008383Slow-growing nails
HP:0011911Abnormality of metacarpophalangeal joint
HP:0031288Cobblestone-like hyperkeratosis
HP:0045059Hyperkeratotic papule
HP:0100759Clubbing of fingers

Эпидемиология (1)

Point prevalence
1-9 / 100 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы