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Spondylo-ocular syndrome

ORPHA:85194Malformation syndromeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

XYLT2
xylosyltransferase 2
Disease-causing germline mutation(s) (loss of function) in
OMIM: 608125

Фенотипы (31)

Очень частый (80–99%)11
HP:0000316Hypertelorism
HP:0000470Short neck
HP:0000534Abnormal eyebrow morphology
HP:0000541Retinal detachment
HP:0000572Visual loss
HP:0000926Platyspondyly
HP:0000939Osteoporosis
HP:0002942Thoracic kyphosis
HP:0003521Disproportionate short-trunk short stature
HP:0005108Abnormal intervertebral disk morphology
HP:0007730Iris hypopigmentation
Частый (30–79%)6
HP:0000297Facial hypotonia
HP:0000518Cataract
HP:0000568Microphthalmia
HP:0001629Ventricular septal defect
HP:0001763Pes planus
HP:0008063Aplasia/Hypoplasia of the lens
Периодический (5–29%)14
HP:0000233Thin vermilion border
HP:0000343Long philtrum
HP:0000369Low-set ears
HP:0000391Thickened helices
HP:0000465Webbed neck
HP:0000545Myopia
HP:0000639Nystagmus
HP:0000974Hyperextensible skin
HP:0001249Intellectual disability
HP:0002162Low posterior hairline
HP:0004322Short stature
HP:0004467Preauricular pit
HP:0009738Abnormality of the antihelix
HP:0001382Joint hypermobility

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