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Tibial aplasia-ectrodactyly syndrome

ORPHA:3329Malformation syndromeAutosomal dominantAntenatal, Infancy, Neonatal

Ассоциированные гены (1)

BHLHA9
basic helix-loop-helix family member a9
Major susceptibility factor in
OMIM: 615416

Фенотипы (18)

Очень частый (80–99%)1
HP:0001171Split hand
Частый (30–79%)3
HP:0001376Limitation of joint mobility
HP:0005772Aplasia/Hypoplasia of the tibia
HP:0100257Ectrodactyly
Периодический (5–29%)14
HP:0000396Overfolded helix
HP:0001156Brachydactyly
HP:0001162Postaxial hand polydactyly
HP:0001177Preaxial hand polydactyly
HP:0001539Omphalocele
HP:0002823Abnormality of femur morphology
HP:0002980Femoral bowing
HP:0002991Abnormal fibula morphology
HP:0003038Fibular hypoplasia
HP:0003097Short femur
HP:0006101Finger syndactyly
HP:0006443Patellar aplasia
HP:0006495Aplasia/Hypoplasia of the ulna
HP:0009756Popliteal pterygium

Эпидемиология (1)

Point prevalence
1-9 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы