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Posterior urethral valve

ORPHA:93110Morphological anomalyAutosomal recessive, Not applicable, X-linked recessiveAll ages

Ассоциированные гены (1)

BNC2
basonuclin zinc finger protein 2
Disease-causing germline mutation(s) in
OMIM: 608669

Фенотипы (27)

Очень частый (80–99%)3
HP:0000010Recurrent urinary tract infections
HP:0010957Congenital posterior urethral valve
HP:0012622Chronic kidney disease
Частый (30–79%)3
HP:0000076Vesicoureteral reflux
HP:0000126Hydronephrosis
HP:0041047Bladder outlet obstruction
Периодический (5–29%)16
HP:0006703Aplasia/Hypoplasia of the lungs
HP:0010945Fetal pyelectasis
HP:0010955Dilatation of the bladder
HP:0010956Fetal megacystis
HP:0012330Pyelonephritis
HP:0000015Bladder diverticulum
HP:0000020Urinary incontinence
HP:0000083Renal insufficiency
HP:0000093Proteinuria
HP:0000110Renal dysplasia
HP:0000790Hematuria
HP:0000822Hypertension
HP:0002009Potter facies
HP:0002027Abdominal pain
HP:0003419Low back pain
HP:0003774Stage 5 chronic kidney disease
Очень редкий (1–4%)5
HP:0000016Urinary retention
HP:0001254Lethargy
HP:0001562Oligohydramnios
HP:0008661Urethral stenosis
HP:0100518Dysuria

Эпидемиология (3)

Point prevalence
1-9 / 100 000
Europe
Prevalence at birth
1-9 / 100 000
Europe
Prevalence at birth
1-9 / 100 000
Australia

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы