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Phocomelia, Schinzel type

ORPHA:2879Malformation syndromeAutosomal recessiveAntenatal, Infancy, Neonatal

Ассоциированные гены (1)

WNT7A
Wnt family member 7A
Disease-causing germline mutation(s) (loss of function) in
OMIM: 601570

Фенотипы (31)

Очень частый (80–99%)12
HP:0009380Finger aplasia
HP:0001849Foot oligodactyly
HP:0002164Nail dysplasia
HP:0002983Micromelia
HP:0002990Fibular aplasia
HP:0002992Abnormality of tibia morphology
HP:0003498Disproportionate short stature
HP:0003982Absent ulna
HP:0006487Bowing of the long bones
HP:0008517Aplasia/Hypoplasia of the sacrum
HP:0009103Aplasia/Hypoplasia involving the pelvis
HP:0100257Ectrodactyly
Частый (30–79%)6
HP:0000151Aplasia of the uterus
HP:0000347Micrognathia
HP:0000470Short neck
HP:0001511Intrauterine growth retardation
HP:0002984Hypoplasia of the radius
HP:0002986Radial bowing
Периодический (5–29%)13
HP:0000028Cryptorchidism
HP:0000175Cleft palate
HP:0000411Protruding ear
HP:0001362Skull defect
HP:0001789Hydrops fetalis
HP:0001883Talipes
HP:0002023Anal atresia
HP:0002435Meningocele
HP:0002575Tracheoesophageal fistula
HP:0002705High, narrow palate
HP:0003041Humeroradial synostosis
HP:0003196Short nose
HP:0008736Hypoplasia of penis

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