← Назад

Meacham syndrome

ORPHA:3097Malformation syndromeAutosomal dominantInfancy, Neonatal

Ассоциированные гены (1)

WT1
WT1 transcription factor
Disease-causing germline mutation(s) in
OMIM: 607102

Фенотипы (25)

Очень частый (80–99%)6
HP:0000062Ambiguous genitalia
HP:0000142Abnormality of the vagina
HP:0000148Vaginal atresia
HP:0000776Congenital diaphragmatic hernia
HP:0006703Aplasia/Hypoplasia of the lungs
HP:0011027Abnormality of the fallopian tube
Частый (30–79%)6
HP:0000028Cryptorchidism
HP:0002101Abnormal lung lobation
HP:0004383Hypoplastic left heart
HP:0008736Hypoplasia of penis
HP:0030010Hydrometrocolpos
HP:0100632Pulmonary sequestration
Периодический (5–29%)13
HP:0000085Horseshoe kidney
HP:0001629Ventricular septal defect
HP:0001631Atrial septal defect
HP:0001636Tetralogy of Fallot
HP:0001643Patent ductus arteriosus
HP:0001650Aortic valve stenosis
HP:0001669Transposition of the great arteries
HP:0001680Coarctation of aorta
HP:0001696Situs inversus totalis
HP:0001710Conotruncal defect
HP:0001743Abnormality of the spleen
HP:0004736Crossed fused renal ectopia
HP:0010772Anomalous pulmonary venous return

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