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Fibular aplasia-complex brachydactyly syndrome

ORPHA:2639Malformation syndromeAutosomal recessiveAntenatal, Neonatal

Ассоциированные гены (2)

BMPR1B
bone morphogenetic protein receptor type 1B
Disease-causing germline mutation(s) in
OMIM: 603248
GDF5
growth differentiation factor 5
Disease-causing germline mutation(s) (loss of function) in
OMIM: 601146

Фенотипы (15)

Очень частый (80–99%)15
HP:0000446Narrow nasal bridge
HP:0001156Brachydactyly
HP:0001172Abnormal thumb morphology
HP:0001376Limitation of joint mobility
HP:0002818Abnormal morphology of the radius
HP:0002983Micromelia
HP:0002992Abnormality of tibia morphology
HP:0003272Abnormality of the hip bone
HP:0004322Short stature
HP:0005048Synostosis of carpal bones
HP:0005930Abnormality of epiphysis morphology
HP:0006492Aplasia/Hypoplasia of the fibula
HP:0007598Bilateral single transverse palmar creases
HP:0008368Tarsal synostosis
HP:0040071Abnormal morphology of ulna

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