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Ghosal hematodiaphyseal dysplasia

ORPHA:1802Malformation syndromeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

TBXAS1
thromboxane A synthase 1
Disease-causing germline mutation(s) in
OMIM: 274180

Фенотипы (13)

Очень частый (80–99%)11
HP:0000944Abnormal metaphysis morphology
HP:0001903Anemia
HP:0002644Abnormality of pelvic girdle bone morphology
HP:0002823Abnormality of femur morphology
HP:0002992Abnormality of tibia morphology
HP:0003103Abnormal cortical bone morphology
HP:0003312Abnormal form of the vertebral bodies
HP:0004493Craniofacial hyperostosis
HP:0005019Diaphyseal thickening
HP:0006487Bowing of the long bones
HP:0010978Abnormality of immune system physiology
Периодический (5–29%)2
HP:0001744Splenomegaly
HP:0002167Abnormality of speech or vocalization

Эпидемиология (1)

Point prevalence
<1 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы