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Autosomal dominant palmoplantar keratoderma and congenital alopecia

ORPHA:1010DiseaseAutosomal dominantInfancy, Neonatal

Ассоциированные гены (1)

GJA1
gap junction protein alpha 1
Disease-causing germline mutation(s) (gain of function) in
OMIM: 121014

Фенотипы (25)

Очень частый (80–99%)6
HP:0000653Sparse eyelashes
HP:0000982Palmoplantar keratoderma
HP:0001597Abnormality of the nail
HP:0002209Sparse scalp hair
HP:0008404Nail dystrophy
HP:0045075Sparse eyebrow
Частый (30–79%)7
HP:0000958Dry skin
HP:0001807Ridged nail
HP:0004528Generalized hypotrichosis
HP:0010491Digital constriction ring
HP:0011838Sclerodactyly
HP:0012785Flexion contracture of finger
HP:0032152Keratosis pilaris
Периодический (5–29%)10
HP:0001041Facial erythema
HP:0001058Poor wound healing
HP:0002223Absent eyebrow
HP:0002298Absent hair
HP:0002435Meningocele
HP:0009886Trichorrhexis nodosa
HP:0009900Unilateral deafness
HP:0031057Skin fissure
HP:0040189Scaling skin
HP:0100018Nuclear cataract
Очень редкий (1–4%)2
HP:0000613Photophobia
HP:0001250Seizure

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