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Langer mesomelic dysplasia

ORPHA:2632Malformation syndromeAutosomal recessiveAntenatal

Ассоциированные гены (1)

SHOX
SHOX homeobox
Disease-causing germline mutation(s) (loss of function) in
OMIM: 312865

Фенотипы (13)

Очень частый (80–99%)13
HP:0000218High palate
HP:0001191Abnormal carpal morphology
HP:0002983Micromelia
HP:0003067Madelung deformity
HP:0003510Severe short stature
HP:0005026Mesomelic/rhizomelic limb shortening
HP:0005930Abnormality of epiphysis morphology
HP:0006487Bowing of the long bones
HP:0006492Aplasia/Hypoplasia of the fibula
HP:0008873Disproportionate short-limb short stature
HP:0009465Ulnar deviation of finger
HP:0040071Abnormal morphology of ulna
HP:0100864Short femoral neck

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