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Ramon syndrome

ORPHA:3019Malformation syndromeAutosomal recessiveInfancy

Ассоциированные гены (1)

ELMO2
engulfment and cell motility 2
Disease-causing germline mutation(s) in
OMIM: 606421

Фенотипы (17)

Очень частый (80–99%)7
HP:0000169Gingival fibromatosis
HP:0000189Narrow palate
HP:0000293Full cheeks
HP:0001249Intellectual disability
HP:0001250Seizure
HP:0001508Failure to thrive
HP:0002797Osteolysis
Частый (30–79%)3
HP:0000684Delayed eruption of teeth
HP:0002230Generalized hirsutism
HP:0007703Abnormality of retinal pigmentation
Периодический (5–29%)7
HP:0000405Conductive hearing impairment
HP:0000407Sensorineural hearing impairment
HP:0000593Abnormal anterior chamber morphology
HP:0000682Abnormality of dental enamel
HP:0000819Diabetes mellitus
HP:0000962Hyperkeratosis
HP:0100585Telangiectasia of the skin

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