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Spondylometaphyseal dysplasia, Schmidt type

ORPHA:93316DiseaseAutosomal dominantAdolescent, Childhood, Infancy

Ассоциированные гены (1)

COL2A1
collagen type II alpha 1 chain
Disease-causing germline mutation(s) in
OMIM: 120140

Фенотипы (30)

Очень частый (80–99%)3
HP:0000925Abnormality of the vertebral column
HP:0000944Abnormal metaphysis morphology
HP:0100255Metaphyseal dysplasia
Частый (30–79%)12
HP:0000926Platyspondyly
HP:0001385Hip dysplasia
HP:0002650Scoliosis
HP:0002812Coxa vara
HP:0002815Abnormality of the knee
HP:0002857Genu valgum
HP:0002867Abnormality of the ilium
HP:0003375Narrow greater sciatic notch
HP:0003510Severe short stature
HP:0003521Disproportionate short-trunk short stature
HP:0010574Abnormality of the epiphysis of the femoral head
HP:0100866Short iliac bones
Периодический (5–29%)15
HP:0000185Cleft soft palate
HP:0000347Micrognathia
HP:0000402Stenosis of the external auditory canal
HP:0000545Myopia
HP:0001561Polyhydramnios
HP:0002020Gastroesophageal reflux
HP:0002033Poor suck
HP:0002751Kyphoscoliosis
HP:0003019Abnormality of the wrist
HP:0003026Short long bone
HP:0003796Irregular iliac crest
HP:0008833Irregular acetabular roof
HP:0011470Nasogastric tube feeding in infancy
HP:0011471Gastrostomy tube feeding in infancy
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