Синдром Майєра-Рокітанського-Кюстера-Хаузера
ORPHA:3109· ICD-10 Q51.8· Mayer-Rokitansky-Küster-Hauser syndrome
Визначення(English summary)
A rare spectrum of Mullerian duct anomalies characterized by congenital aplasia of the uterus and upper two-thirds of the vagina in otherwise phenotypically normal females. It can be classified as either Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome type 1 (corresponding to isolated utero-vaginal aplasia) or MRKH syndrome type 2 (utero-vaginal aplasia associated with other malformations).
- Поширеність
- 1-5 / 10 000
- Успадкування
- Autosomal dominant, Not applicable
- Вік початку
- Adolescent, Antenatal