← Назад

Osteoglosphonic dysplasia

ORPHA:2645Malformation syndromeAutosomal dominantNeonatal

Ассоциированные гены (1)

FGFR1
fibroblast growth factor receptor 1
Disease-causing germline mutation(s) (gain of function) in
OMIM: 136350

Фенотипы (20)

Очень частый (80–99%)6
HP:0000316Hypertelorism
HP:0001363Craniosynostosis
HP:0003312Abnormal form of the vertebral bodies
HP:0003510Severe short stature
HP:0006283Multiple unerupted teeth
HP:0009804Tooth agenesis
Частый (30–79%)7
HP:0000347Micrognathia
HP:0000411Protruding ear
HP:0000463Anteverted nares
HP:0000889Abnormality of the clavicle
HP:0001531Failure to thrive in infancy
HP:0002750Delayed skeletal maturation
HP:0008905Rhizomelia
Периодический (5–29%)7
HP:0000023Inguinal hernia
HP:0000028Cryptorchidism
HP:0000453Choanal atresia
HP:0001156Brachydactyly
HP:0001249Intellectual disability
HP:0002650Scoliosis
HP:0011849Abnormal bone ossification

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