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FGFR2-related bent bone dysplasia

ORPHA:313855DiseaseAutosomal dominantAntenatal, Neonatal

Ассоциированные гены (1)

FGFR2
fibroblast growth factor receptor 2
Disease-causing germline mutation(s) in
OMIM: 176943

Фенотипы (27)

Очень частый (80–99%)24
HP:0000212Gingival overgrowth
HP:0000316Hypertelorism
HP:0000347Micrognathia
HP:0000356Abnormality of the outer ear
HP:0000369Low-set ears
HP:0000485Megalocornea
HP:0000695Natal tooth
HP:0000894Short clavicles
HP:0000938Osteopenia
HP:0001007Hirsutism
HP:0001156Brachydactyly
HP:0001591Bell-shaped thorax
HP:0001978Extramedullary hematopoiesis
HP:0003175Hypoplastic ischia
HP:0004440Coronal craniosynostosis
HP:0004453Overfolding of the superior helices
HP:0005474Decreased calvarial ossification
HP:0007642Congenital stationary night blindness
HP:0008665Clitoral hypertrophy
HP:0010455Steep acetabular roof
HP:0011223Metopic depression
HP:0011800Midface retrusion
HP:0030042Incomplete ossification of pubis
HP:0030313Abnormal periosteum morphology
Периодический (5–29%)3
HP:0001433Hepatosplenomegaly
HP:0002814Abnormality of the lower limb
HP:0002979Bowing of the legs

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