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Matthew-Wood syndrome

ORPHA:2470Malformation syndromeAutosomal dominant, Autosomal recessiveAntenatal, Neonatal

Ассоциированные гены (2)

STRA6
signaling receptor and transporter of retinol STRA6
Disease-causing germline mutation(s) in
OMIM: 610745
WNT7B
Wnt family member 7B
Disease-causing germline mutation(s) in
OMIM: 601967

Фенотипы (20)

Очень частый (80–99%)3
HP:0000528Anophthalmia
HP:0000568Microphthalmia
HP:0001249Intellectual disability
Частый (30–79%)4
HP:0000776Congenital diaphragmatic hernia
HP:0002088Abnormal lung morphology
HP:0002089Pulmonary hypoplasia
HP:0030680Abnormal cardiovascular system morphology
Периодический (5–29%)13
HP:0000028Cryptorchidism
HP:0000076Vesicoureteral reflux
HP:0000085Horseshoe kidney
HP:0000089Renal hypoplasia
HP:0000130Abnormality of the uterus
HP:0000369Low-set ears
HP:0001252Hypotonia
HP:0001508Failure to thrive
HP:0001511Intrauterine growth retardation
HP:0001734Annular pancreas
HP:0025408Abnormal spleen morphology
HP:0100800Aplasia/Hypoplasia of the pancreas
HP:0100867Duodenal stenosis

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