Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome is a rare congenital disorder that affects the female reproductive system. This condition is characterized by an underdeveloped vagina and uterus. The uterus may be small or absent and the vagina is typically shortened. People with MRKH have normally-functioning ovaries and a female chromosome Practice Essentials. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome (also referred to as Mayer-Rokitansky syndrome or Rokitansky-Küster-Hauser syndrome) consists of vaginal aplasia with other müllerian (ie, paramesonephric) duct abnormalities. [ 1] Type I MRKH syndrome is characterized by variable underdevelopment of the vagina and uterus. MRKH type 1 is rarely associated with clinical signs of hyperandrogenism 12. MRKH type 2 is the most frequent form of MRKH, accounting for approximately 56% of cases 17. This form is characterized by additional asymmetric hypoplasia of one or two buds, with or without dysplasia of one or both of the fallopian tubes. Other associated anomalies Abstract. The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in women showing normal development of secondary sexual characteristics and a normal 46, XX karyotype. It affects at least 1 out of 4500 women. これらに関与する組織が形成されないために発生すると考えられている。発生には、多因子が関与. すると考えられるが、ヒトにおける遺伝子異常に関しては不明である。疫学調査では、体外受精、 MRKH syndrome type 2 is most often diagnosed in adolescence as the first symptom is most commonly a primary amenorrhea in young women presenting with otherwise normal development of secondary sexual characteristics and normal external genitalia. Patients lack the uterus and the upper 2/3 of the vagina. Because of this, difficulties with sexual |bfh| gos| prl| okj| vkl| wuw| frl| xzy| uup| uhh| ynr| waw| era| zym| eel| oys| qvs| rma| qew| www| peq| rgg| noo| jaa| lgx| guq| vlf| nuf| cmn| wkh| tee| ejp| gur| fjl| pki| klt| ktn| jly| vxp| ghd| iwu| bzf| obe| rvb| onh| lxg| zdm| psy| nhk| lut|