机构地区:[1] 复旦大学附属华山医院内分泌科,上海200040 [2] 复旦大学附属华山医院神经外科,上海200040 [3]复旦大学附属华山医院放射科,上海200040 [4]复旦大学附属华山医院神经病理科,上海200040
出 处:《中华神经外科杂志》2015年第6期601-604,共4页Chinese Journal of Neurosurgery
基 金:上海市科学技术委员会2011年度科技创新重点项目(11411951900)
摘 要:目的 分析临床多学科综合治疗团队(MDT)诊治库欣病的疗效及经验.方法 回顾性纳入2013年1月至12月复旦大学附属华山医院在MDT诊治流程模式下诊治的45例库欣综合征患者.其中22例行双侧岩下窦采血,确诊库欣病40例(初诊断32例,首次治疗后复发或未缓解8例).大腺瘤、微腺瘤和MRI阴性分别为8、26、6例,3例为侵袭性;33例接受神经外科手术(经鼻手术32例,开颅手术1例).总结其诊断、治疗情况.结果 33例接受手术的患者中,术后1周缓解者22例(66.7%),其术前、术后1周内血皮质醇最低值差异有统计学意义[分别为(869±361)、(60±41)nmol/L,P<0.01].术后3个月总体缓解25例(76%);大腺瘤、微腺瘤和MRI阴性及侵袭性垂体瘤缓解的比例分别为6/6、14/18、3/6、2/3.病理学显示,27例促肾上腺皮质激素瘤、6例增生或腺组织者分别有25例(92.6%)和0例缓解.手术后未缓解的8例中,2例纳入帕瑞肽新药研究、3例行伽玛刀治疗、1例开始甲吡酮治疗、1例待再次手术治疗、1例失访.7例患者出现手术并发症.结论 MDT模式下多学科参与的病例诊治有助于患者及时选择最佳的治疗方案,提高治疗效果.Objective To analyze the efficacy and experiences of the diagnosis and treatment of Cushing' s disease with a multidisciplinary team.Methods Forty-five patients with Cushing's syndrome treated under the MDT diagnosis and treatment process mode at Shanghai Huashan Hospital,Fudan University from January to December 2013 were analyzed retrospectively.Among the 45 patients,22 underwent bilateral inferior petrosal sinus blood sampling,and 40 were diagnosed as Cushing' s syndrome.Macroadenomas,microadenomas,and MRI negative were 8,26,and 6,respectively,3 of them were invasive.Thirty-two patients were newly diagnosed,8 relapsed or did not relieve after the first treatment.Their diagnosis,management,efficacy,and room for improvement were summarized.Results Thirty-three patients with Cushing's disease received neurosurgery,32 had transnasal surgery and 1 underwent craniotomy.There was significant difference in the lowest values of serum cortisol at 1 week before and after surgery (869±361 and 60±41 nmol/L respectively; P 〈 0.01).Twenty-two patients had early remission,and 25 had overall remission (76%) at 3 months after surgery; the remission proportions of macroadenoma,microadenoma,MRI negative,and invasive pituitary adenoma were 6/6,14/18,3/6,and 2/3,respectively.Of the patients who relapsed or did not relieve after treatment,6 were reoperated,and 4 were relieved.Of the 27 patients with adrenocorticotropic hormone (ACTH) tumor and 6 with hyperplasia or glandular tissue,25 and 0 were relieved.Of the 8 patients who were not relieved after surgery,2 were enrolled into the new drug research of pasireotide,3 were treated with gamma knife,1 began to be treated with metyrapone,1 was waiting for reoperation,and 1 was lost to follow-up.Seven patients had surgical complications.Conclusions The factors affecting surgical treatment of Cushing' s disease include the pituitary imaging and pathological features,etc.Under the MDT mode,the diagnosis and treatment help patients to timely choose the optim
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