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Congenital contractural arachnodactyly

ORPHA:115Malformation syndromeAutosomal dominantNeonatal

Ассоциированные гены (1)

FBN2
fibrillin 2
Disease-causing germline mutation(s) in
OMIM: 612570

Фенотипы (21)

Очень частый (80–99%)13
HP:0000218High palate
HP:0001166Arachnodactyly
HP:0001371Flexion contracture
HP:0001387Joint stiffness
HP:0001533Slender build
HP:0002650Scoliosis
HP:0002803Congenital contracture
HP:0002804Arthrogryposis multiplex congenita
HP:0003011Abnormality of the musculature
HP:0008453Congenital kyphoscoliosis
HP:0008544Abnormally folded helix
HP:0009901Crumpled ear
HP:0100490Camptodactyly of finger
Частый (30–79%)1
HP:0001519Disproportionate tall stature
Периодический (5–29%)7
HP:0001083Ectopia lentis
HP:0001634Mitral valve prolapse
HP:0002247Duodenal atresia
HP:0002566Intestinal malrotation
HP:0002575Tracheoesophageal fistula
HP:0004942Aortic aneurysm
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