← Назад

Acrofacial dysostosis, Rodríguez type

ORPHA:1788Malformation syndromeAutosomal dominant, Autosomal recessiveAntenatal, Neonatal

Ассоциированные гены (1)

SF3B4
splicing factor 3b subunit 4
Disease-causing germline mutation(s) (loss of function) in
OMIM: 605593

Фенотипы (20)

Очень частый (80–99%)5
HP:0000272Malar flattening
HP:0000308Microretrognathia
HP:0000426Prominent nasal bridge
HP:0008551Microtia
HP:0009380Finger aplasia
Частый (30–79%)9
HP:0000912Sprengel anomaly
HP:0002139Arrhinencephaly
HP:0002410Aqueductal stenosis
HP:0002644Abnormality of pelvic girdle bone morphology
HP:0002974Radioulnar synostosis
HP:0003038Fibular hypoplasia
HP:0006495Aplasia/Hypoplasia of the ulna
HP:0006501Aplasia/Hypoplasia of the radius
HP:0030680Abnormal cardiovascular system morphology
Периодический (5–29%)6
HP:0000130Abnormality of the uterus
HP:0001511Intrauterine growth retardation
HP:0001762Talipes equinovarus
HP:0003312Abnormal form of the vertebral bodies
HP:0006101Finger syndactyly
HP:0008678Renal hypoplasia/aplasia

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