← Назад

Osteosclerosis-developmental delay-craniosynostosis syndrome

ORPHA:178377Malformation syndromeAutosomal dominantInfancy, Neonatal

Ассоциированные гены (1)

LRP5
LDL receptor related protein 5
Disease-causing germline mutation(s) (gain of function) in
OMIM: 603506

Фенотипы (16)

Очень частый (80–99%)8
HP:0000248Brachycephaly
HP:0000256Macrocephaly
HP:0000316Hypertelorism
HP:0000337Broad forehead
HP:0000348High forehead
HP:0002684Thickened calvaria
HP:0011001Increased bone mineral density
HP:0012802Broad jaw
Частый (30–79%)1
HP:0001363Craniosynostosis
Периодический (5–29%)7
HP:0000365Hearing impairment
HP:0000505Visual impairment
HP:0000648Optic atrophy
HP:0002315Headache
HP:0002516Increased intracranial pressure
HP:0010628Facial palsy
HP:0011342Mild global developmental delay

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