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Oculocutaneous albinism type 2

ORPHA:79432DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (2)

OCA2
OCA2 melanosomal transmembrane protein
Disease-causing germline mutation(s) in
OMIM: 611409
MC1R
melanocortin 1 receptor
Modifying germline mutation in
OMIM: 155555

Фенотипы (24)

Частый (30–79%)17
HP:0000539Abnormality of refraction
HP:0000613Photophobia
HP:0000635Blue irides
HP:0000639Nystagmus
HP:0001010Hypopigmentation of the skin
HP:0001100Heterochromia iridis
HP:0001480Freckling
HP:0002226White eyebrow
HP:0005599Hypopigmentation of hair
HP:0007663Reduced visual acuity
HP:0007703Abnormality of retinal pigmentation
HP:0007730Iris hypopigmentation
HP:0007750Hypoplasia of the fovea
HP:0007988Macular hypopigmentation
HP:0011364White hair
HP:0012805Iris transillumination defect
HP:0025551Optic nerve misrouting
Периодический (5–29%)6
HP:0002227White eyelashes
HP:0002671Basal cell carcinoma
HP:0006739Squamous cell carcinoma of the skin
HP:0012056Cutaneous melanoma
HP:0030856Posterior staphyloma
HP:0200098Absent skin pigmentation
Очень редкий (1–4%)1
HP:0007481Hyperpigmented nevi

Эпидемиология (5)

Point prevalence
6-9 / 10 000
Specific population
Point prevalence
1-9 / 100 000
Worldwide
Point prevalence
6-9 / 10 000
Tanzania, United Republic of
Point prevalence
1-9 / 100 000
United States
Point prevalence
1-5 / 10 000
Africa

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы