本文選題：垂體性巨人癥　切入點(diǎn)：垂體生長(zhǎng)激素腺瘤　出處：《吉林大學(xué)》2017年碩士論文

【摘要】：目的:通過(guò)收集總結(jié)垂體性巨人癥的臨床資料明確中國(guó)地區(qū)垂體性巨人癥患者的臨床特點(diǎn)。方法:收集1990年1月-2016年12月就診于北京協(xié)和醫(yī)院內(nèi)分泌科及神經(jīng)外科診斷為“垂體性巨人癥”的患者的病例資料,總結(jié)所有納入患者的臨床表現(xiàn)、實(shí)驗(yàn)室檢查、影像學(xué)檢查、治療情況以及隨訪資料,以此明確垂體性巨人癥患者的臨床特征。結(jié)果:本研究共納入48例垂體性巨人癥患者,其中43例為病理證實(shí)的垂體生長(zhǎng)激素腺瘤,包括1例診斷為Mc Cune-Albright綜合征的垂體性巨人癥,剩余5例患者的臨床表現(xiàn)、生化、影像學(xué)均提示垂體生長(zhǎng)激素腺瘤,包括3例已行手術(shù)治療但術(shù)后病理未提示垂體腺瘤的患者和2例未行手術(shù)治療的患者。所有患者就診時(shí)中位身高標(biāo)準(zhǔn)差分?jǐn)?shù)(Standard Deviation Score,SDS)為4.05[3.14,5.35],起病即表現(xiàn)為身高增長(zhǎng)過(guò)快的為30例(62.5%),而起病表現(xiàn)為肢端肥大癥的為27例(56.3%)。初始癥狀出現(xiàn)的中位年齡為11.5歲[7,14],身高開(kāi)始增長(zhǎng)中位年齡為11歲[4.5,14],中位診斷年齡為18歲[14,21],中位病程年數(shù)為5年[2,11.5]。診斷時(shí)的葡萄糖生長(zhǎng)激素抑制試驗(yàn)中位GH谷值為17.45 ng/ml[8.92,34.73],中位IGF-1(測(cè)量)/IGF-1(正常參考值上限)(Upper limit of normal age-referenced norms for IGF-1 concentration,ULN)水平為1.82[1.28,2.64],均明顯高于正常值。經(jīng)影像學(xué)證實(shí)為垂體微腺瘤的有13例(27.1%),大腺瘤有35例(72.9%),其中鞍外生長(zhǎng)的有20例(41.7%),而具有侵襲性的為16例(33.3%)。共有46例患者接受了手術(shù),其中33例接受了一次手術(shù),11例接受了兩次手術(shù),1例接受了三次手術(shù)治療,10例患者行生長(zhǎng)抑素類似物治療,包括2例行單純藥物治療的患者,另有7例行放射治療。結(jié)論:本研究回顧了48例垂體性巨人癥患者的臨床資料,巨人癥患者總體上具有起病早、診斷晚、病程長(zhǎng)、男性患者居多的特點(diǎn)。目前仍將手術(shù)治療作為其一線治療,盡管治療后術(shù)后即刻GH值明顯低于術(shù)前,但無(wú)論是短期隨訪還是長(zhǎng)期隨訪顯示其緩解率不理想;必要時(shí)仍需聯(lián)合生長(zhǎng)抑素類似物的藥物治療和放射治療以此提高其緩解率。綜上所述,垂體性巨人癥在臨床上的治療仍舊是一個(gè)難題,容易復(fù)發(fā)且治療效果不佳,下一步應(yīng)該聯(lián)合多中心研究探究更精確的治療方法。
[Abstract]:Objective: to summarize the clinical data of pituitary giant disease in China.Methods: from January 1990 to December 2016, we collected the data of all the patients diagnosed as "pituitary giant disease" in Department of Endocrinology, Department of Endocrinology and Neurosurgery, and summarized the clinical manifestation, laboratory examination and imaging examination of all the patients involved.Treatment and follow-up data were used to identify the clinical features of patients with pituitary giant disease.Results: a total of 48 patients with pituitary giant's disease were included in this study, 43 of whom were pathologically confirmed pituitary growth hormone adenomas, including one pituitary giant diagnosed as MC Cune-Albright syndrome, and the remaining 5 patients with clinical manifestations, biochemistry, and biochemistry.All the imaging findings showed that pituitary growth hormone adenoma including 3 patients who had undergone surgical treatment but whose pathology did not indicate pituitary adenoma and 2 patients who had not received surgical treatment.The median standard deviation of height (Standard Deviation Scoreen SDSs) of all patients was 4.05 [3.145.35]. The onset of the disease was 62.5% in 30 patients with rapid height growth, and 56.3% in 27 cases with acromegaly.璇婃柇鏃剁殑钁¤悇緋栫敓闀挎縺绱犳姂鍒惰瘯楠屼腑浣岹H璋峰，

本文編號(hào)：1710264