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Cole-Carpenter syndrome

ORPHA:2050Malformation syndromeAutosomal dominant, Autosomal recessive, Not applicableNeonatal

Ассоциированные гены (3)

P4HB
prolyl 4-hydroxylase subunit beta
Disease-causing germline mutation(s) in
OMIM: 176790
SEC24D
SEC24 homolog D, COPII component
Disease-causing germline mutation(s) in
OMIM: 607186
CRTAP
cartilage associated protein
Disease-causing germline mutation(s) in
OMIM: 605497

Фенотипы (26)

Очень частый (80–99%)14
HP:0000347Micrognathia
HP:0000520Proptosis
HP:0000592Blue sclerae
HP:0000684Delayed eruption of teeth
HP:0000772Abnormal rib morphology
HP:0000944Abnormal metaphysis morphology
HP:0001608Abnormality of the voice
HP:0002007Frontal bossing
HP:0002652Skeletal dysplasia
HP:0003312Abnormal form of the vertebral bodies
HP:0004322Short stature
HP:0006367Crumpled long bones
HP:0006487Bowing of the long bones
HP:0011800Midface retrusion
Частый (30–79%)9
HP:0000262Turricephaly
HP:0000682Abnormality of dental enamel
HP:0001252Hypotonia
HP:0001334Communicating hydrocephalus
HP:0001511Intrauterine growth retardation
HP:0002645Wormian bones
HP:0002650Scoliosis
HP:0002757Recurrent fractures
HP:0002808Kyphosis
Периодический (5–29%)3
HP:0000494Downslanted palpebral fissures
HP:0001263Global developmental delay
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