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Acrofacial dysostosis, Weyers type

ORPHA:952Malformation syndromeAutosomal dominantNeonatal

Ассоциированные гены (3)

EVC
EvC ciliary complex subunit 1
Disease-causing germline mutation(s) in
OMIM: 604831
EVC2
EvC ciliary complex subunit 2
Disease-causing germline mutation(s) in
OMIM: 607261
CTNNB1
catenin beta 1
Disease-causing germline mutation(s) (gain of function) in
OMIM: 116806

Фенотипы (19)

Очень частый (80–99%)14
HP:0000164Abnormality of the dentition
HP:0000190Abnormal oral frenulum morphology
HP:0000668Hypodontia
HP:0000698Conical tooth
HP:0001162Postaxial hand polydactyly
HP:0001231Abnormal fingernail morphology
HP:0001792Small nail
HP:0001800Hypoplastic toenails
HP:0003502Mild short stature
HP:0006288Advanced eruption of teeth
HP:0006315Single median maxillary incisor
HP:0008388Abnormal toenail morphology
HP:0008404Nail dystrophy
HP:0100797Toenail dysplasia
Частый (30–79%)5
HP:0002006Facial cleft
HP:0004209Clinodactyly of the 5th finger
HP:0009738Abnormality of the antihelix
HP:0010557Overlapping fingers
HP:0200055Small hand

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