本文關(guān)鍵詞： MRKH綜合征 子宮內(nèi)膜異位癥 子宮腺肌病 上皮化生學(xué)說(shuō) MRKH綜合征 子宮腺肌病　出處：《浙江大學(xué)》2014年博士論文　論文類型：學(xué)位論文

【摘要】：目的：通過(guò)對(duì)MRKH綜合征(Mayer-Rokitansky-Kuster-Hauser Syndrome)患者中子宮內(nèi)膜異位癥和子官腺肌病的發(fā)病調(diào)查,探討MRKH綜合征合并子宮內(nèi)膜異位性疾病的可能機(jī)制。 方法：回顧性分析浙江大學(xué)醫(yī)學(xué)院附屬婦產(chǎn)科醫(yī)院從1999年1月1日至2013年12月31日收治的219例MRKH綜合征患者的病例資料,分析其臨床特征,并比較有正常內(nèi)膜組和無(wú)正常內(nèi)膜組的患者中子宮內(nèi)膜異位癥和子宮腺肌病的發(fā)病率差異。 結(jié)果：219例MRKH綜合征患者中,171例(78.08%)無(wú)正常內(nèi)膜,其中2例合并子宮腺肌病,1例合并子宮內(nèi)膜異位癥；48例(21.92%)有正常內(nèi)膜,其中7例合并子宮腺肌病,6例合并子宮內(nèi)膜異位癥。有正常內(nèi)膜組與無(wú)正常內(nèi)膜組的MRKH綜合征患者中子宮內(nèi)膜異位癥的發(fā)病率有顯著差異(p0.05)；有正常內(nèi)膜與有始基子宮但無(wú)正常內(nèi)膜的MRKH綜合征患者中子宮腺肌病的發(fā)病率亦有顯著差異(p0.05)。 結(jié)論：子宮內(nèi)膜異位癥和子宮腺肌病在有正常內(nèi)膜的MRKH綜合征患者中的發(fā)病率顯著高于無(wú)正常內(nèi)膜的MRKH綜合征患者,提示正常內(nèi)膜在子宮內(nèi)膜異位性疾病的發(fā)生發(fā)展中有重要作用；同時(shí)無(wú)正常內(nèi)膜的MRKH綜合征患者仍可發(fā)生子宮內(nèi)膜異位癥和子宮腺肌病,推測(cè)體腔上皮化生學(xué)說(shuō)與子宮內(nèi)膜異位性疾病的發(fā)病有關(guān)。 第二部分無(wú)正常內(nèi)膜的MRKH綜合征合并子宮腺肌病2例中文摘要 子宮腺肌病被認(rèn)為是子宮內(nèi)膜的腺體和間質(zhì)侵入子宮肌層內(nèi)生長(zhǎng)所致的一種良性子宮病變,似乎不可能發(fā)生在沒(méi)有正常內(nèi)膜的患者上。在此我們報(bào)道兩例無(wú)正常內(nèi)膜的Mayer-Rokitansky-Kuster-Hauser (MRKH)綜合征患者發(fā)生子宮腺肌病的例子。兩名患者均因原發(fā)性閉經(jīng)及周期性下腹痛就診；婦科檢查提示正常外陰發(fā)育、陰道盲端及盆腔腫塊,超聲檢查提示“不均質(zhì)低回聲包塊,無(wú)宮腔線”；腹腔鏡手術(shù)中發(fā)現(xiàn)增大的始基子宮,雙側(cè)卵巢正常；術(shù)前CA125均升高,始基子宮切除術(shù)后恢復(fù)至正常水平。術(shù)后病理報(bào)告示子宮腺肌病,未見(jiàn)正常內(nèi)膜。無(wú)正常內(nèi)膜的MRKH綜合征患者可以發(fā)生子宮腺肌病,提示子宮腺肌病的發(fā)生可能與苗勒管的殘余化生有關(guān)。
[Abstract]:Objective: to investigate the pathogenesis of endometriosis and adenomyosis in patients with MRKH syndrome (Mayer-Rokitansky-Kuster-Hauser Syndrome) and to explore the possible mechanism of MRKH syndrome complicated with endometriosis. Methods: the data of 219 patients with MRKH syndrome admitted from January 1st 1999 to December 31st 2013 in the affiliated Obstetrics and Gynecology Hospital of Zhejiang University Medical College were retrospectively analyzed. The incidence of endometriosis and adenomyosis were compared between normal endometrium group and non-normal endometrium group. Results there was no normal endometrium in 171 of the 219 MRKH syndrome patients (including 2 cases with adenomyosis and 1 case with endometriosis, 48 cases with endometriosis) with normal endometrium. The incidence of endometriosis in 7 cases with adenomyosis and 6 cases with endometriosis was significantly different between those with normal endometrium and those without normal endometrium (p 0.05), normal endometrium with normal endometrium and primary base with normal endometrium. The incidence of adenomyosis in patients with MRKH syndrome without normal endometrium was also significantly different (P 0.05). Conclusion: the incidence of endometriosis and adenomyosis in patients with MRKH syndrome with normal endometrium is significantly higher than that with MRKH syndrome without normal endometrium. The results suggest that normal endometrium plays an important role in the occurrence and development of endometriosis and that endometriosis and adenomyosis can still occur in patients with MRKH syndrome without normal endometrium. It is inferred that the supraluminal metaplasia theory is related to the pathogenesis of endometriosis. Part two MRKH syndrome without normal endometrium with adenomyosis: a report of 2 cases. Adenomyosis is thought to be a benign uterine lesion caused by the invasion of the endometrial glands and stroma into the myometrium of the uterus. It seems unlikely to occur in patients without normal endometrium. Here we report two cases of adenomyosis in patients with Mayer-Rokitansky-Kuster-Hauser 's syndrome without normal endometrium. Both patients were treated for primary amenorrhea and periodic lower abdominal pain. Gynecological examination showed normal vulva development, vaginal mass at the blind end and pelvic mass, ultrasonic examination showed "uneven hypoechoic mass without uterine cavity line", enlarged primary uterus and normal bilateral ovaries were found during laparoscopic surgery, and CA125 was increased before operation. After primary hysterectomy, it returned to normal level. The postoperative pathological report showed that adenomyosis was not seen in normal endometrium. Adenomyosis could occur in patients with MRKH syndrome without normal endometrium. The results suggest that the occurrence of adenomyosis may be related to the residual metaplasia of Mullerian canal.
【學(xué)位授予單位】：浙江大學(xué)
【學(xué)位級(jí)別】：博士
【學(xué)位授予年份】：2014
【分類號(hào)】：R711.71

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