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Metaphyseal chondrodysplasia, Spahr type

ORPHA:2501DiseaseAutosomal recessiveChildhood

Ассоциированные гены (1)

MMP13
matrix metallopeptidase 13
Disease-causing germline mutation(s) (loss of function) in
OMIM: 600108

Фенотипы (17)

Очень частый (80–99%)13
HP:0000944Abnormal metaphysis morphology
HP:0001288Gait disturbance
HP:0001385Hip dysplasia
HP:0002750Delayed skeletal maturation
HP:0002970Genu varum
HP:0003307Hyperlordosis
HP:0003498Disproportionate short stature
HP:0004349Reduced bone mineral density
HP:0005871Metaphyseal chondrodysplasia
HP:0006385Short lower limbs
HP:0006409Progressive leg bowing
HP:0006487Bowing of the long bones
HP:0100255Metaphyseal dysplasia
Частый (30–79%)4
HP:0000164Abnormality of the dentition
HP:0000670Carious teeth
HP:0002650Scoliosis
HP:0005930Abnormality of epiphysis morphology

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