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Mayer-Rokitansky-Küster-Hauser syndrome type 2

ORPHA:2578Clinical subtypeAutosomal dominant, Not applicableAdolescent, Antenatal, Neonatal

Ассоциированные гены (2)

HNF1B
HNF1 homeobox B
Disease-causing germline mutation(s) (loss of function) in
OMIM: 189907
WNT4
Wnt family member 4
Disease-causing germline mutation(s) in
OMIM: 603490

Фенотипы (12)

Очень частый (80–99%)9
HP:0000027Azoospermia
HP:0000086Ectopic kidney
HP:0000104Renal agenesis
HP:0000110Renal dysplasia
HP:0000470Short neck
HP:0000813Bicornuate uterus
HP:0002162Low posterior hairline
HP:0004322Short stature
HP:0008684Aplasia/hypoplasia of the uterus
Частый (30–79%)2
HP:0000772Abnormal rib morphology
HP:0003422Vertebral segmentation defect
Периодический (5–29%)1
HP:0000365Hearing impairment

Эпидемиология (2)

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

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы