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Odontochondrodysplasia

ORPHA:166272Malformation syndromeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

TRIP11
thyroid hormone receptor interactor 11
Disease-causing germline mutation(s) in
OMIM: 604505

Фенотипы (22)

Очень частый (80–99%)9
HP:0000703Dentinogenesis imperfecta
HP:0000774Narrow chest
HP:0000926Platyspondyly
HP:0000944Abnormal metaphysis morphology
HP:0002983Micromelia
HP:0004279Short palm
HP:0004322Short stature
HP:0010579Cone-shaped epiphysis
HP:0001382Joint hypermobility
Частый (30–79%)4
HP:0000684Delayed eruption of teeth
HP:0002650Scoliosis
HP:0002673Coxa valga
HP:0003278Square pelvis
Периодический (5–29%)9
HP:0000278Retrognathia
HP:0000486Strabismus
HP:0001522Death in infancy
HP:0001643Patent ductus arteriosus
HP:0002007Frontal bossing
HP:0002098Respiratory distress
HP:0003196Short nose
HP:0005280Depressed nasal bridge
HP:0006487Bowing of the long bones

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