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Autosomal dominant spondylocostal dysostosis

ORPHA:1797Malformation syndromeAutosomal dominantNo data available

Ассоциированные гены (1)

TBX6
T-box transcription factor 6
Disease-causing germline mutation(s) in
OMIM: 602427

Фенотипы (22)

Очень частый (80–99%)4
HP:0001511Intrauterine growth retardation
HP:0002650Scoliosis
HP:0003422Vertebral segmentation defect
HP:0003510Severe short stature
Частый (30–79%)7
HP:0000269Prominent occiput
HP:0000431Wide nasal bridge
HP:0000463Anteverted nares
HP:0000470Short neck
HP:0000582Upslanted palpebral fissure
HP:0003307Hyperlordosis
HP:0010306Short thorax
Периодический (5–29%)11
HP:0000008Abnormal morphology of female internal genitalia
HP:0000175Cleft palate
HP:0000252Microcephaly
HP:0000256Macrocephaly
HP:0000772Abnormal rib morphology
HP:0000913Posterior rib fusion
HP:0000921Missing ribs
HP:0002205Recurrent respiratory infections
HP:0003298Spina bifida occulta
HP:0005107Abnormal sacrum morphology
HP:0030680Abnormal cardiovascular system morphology

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы