垂体性巨人症患者的临床资料分析

发布时间：2018-04-04 14:02

  本文选题：垂体性巨人症　切入点：垂体生长激素腺瘤　出处：《吉林大学》2017年硕士论文

【摘要】：目的:通过收集总结垂体性巨人症的临床资料明确中国地区垂体性巨人症患者的临床特点。方法:收集1990年1月-2016年12月就诊于北京协和医院内分泌科及神经外科诊断为“垂体性巨人症”的患者的病例资料,总结所有纳入患者的临床表现、实验室检查、影像学检查、治疗情况以及随访资料,以此明确垂体性巨人症患者的临床特征。结果:本研究共纳入48例垂体性巨人症患者,其中43例为病理证实的垂体生长激素腺瘤,包括1例诊断为Mc Cune-Albright综合征的垂体性巨人症,剩余5例患者的临床表现、生化、影像学均提示垂体生长激素腺瘤,包括3例已行手术治疗但术后病理未提示垂体腺瘤的患者和2例未行手术治疗的患者。所有患者就诊时中位身高标准差分数(Standard Deviation Score,SDS)为4.05[3.14,5.35],起病即表现为身高增长过快的为30例(62.5%),而起病表现为肢端肥大症的为27例(56.3%)。初始症状出现的中位年龄为11.5岁[7,14],身高开始增长中位年龄为11岁[4.5,14],中位诊断年龄为18岁[14,21],中位病程年数为5年[2,11.5]。诊断时的葡萄糖生长激素抑制试验中位GH谷值为17.45 ng/ml[8.92,34.73],中位IGF-1(测量)/IGF-1(正常参考值上限)(Upper limit of normal age-referenced norms for IGF-1 concentration,ULN)水平为1.82[1.28,2.64],均明显高于正常值。经影像学证实为垂体微腺瘤的有13例(27.1%),大腺瘤有35例(72.9%),其中鞍外生长的有20例(41.7%),而具有侵袭性的为16例(33.3%)。共有46例患者接受了手术,其中33例接受了一次手术,11例接受了两次手术,1例接受了三次手术治疗,10例患者行生长抑素类似物治疗,包括2例行单纯药物治疗的患者,另有7例行放射治疗。结论:本研究回顾了48例垂体性巨人症患者的临床资料,巨人症患者总体上具有起病早、诊断晚、病程长、男性患者居多的特点。目前仍将手术治疗作为其一线治疗,尽管治疗后术后即刻GH值明显低于术前,但无论是短期随访还是长期随访显示其缓解率不理想;必要时仍需联合生长抑素类似物的药物治疗和放射治疗以此提高其缓解率。综上所述,垂体性巨人症在临床上的治疗仍旧是一个难题,容易复发且治疗效果不佳,下一步应该联合多中心研究探究更精确的治疗方法。
[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¤悇绯栫敓闀挎縺绱犳姂鍒惰瘯楠屼腑浣岹H璋峰，

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