[1]张超,王先令,陈予龙,等.136例POEMS综合征患者内分泌代谢异常的临床特点回顾性分析[J].国际内分泌代谢杂志,2021,41(01):38-43.[doi:10.3760/cma.j.cn121383-20200413-04035]
　Zhang Chao,Wang Xianling,Chen Yulong,et al.Retrospective analysis of the clinical characteristics of endocrine-metabolic abnormalities in 136 patients with POEMS syndrome[J].International Journal of Endocrinology and Metabolism,2021,41(01):38-43.[doi:10.3760/cma.j.cn121383-20200413-04035]
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136例POEMS综合征患者内分泌代谢异常的临床特点回顾性分析()
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《国际内分泌代谢杂志》[ISSN:1673-4157/CN:12-1383/R]

卷:
41
期数:
2021年01期
页码:
38-43
栏目:
论著
出版日期:
2021-01-20

文章信息/Info

Title:
Retrospective analysis of the clinical characteristics of endocrine-metabolic abnormalities in 136 patients with POEMS syndrome
作者:
张超1; 2; 王先令1; 陈予龙1; 郭清华1; 裴育1; 杜锦1; 窦京涛1; 巴建明1; 吕朝晖1; 母义明1
1中国人民解放军总医院第一医学中心内分泌科,北京 100089; 2安钢总医院内分泌代谢科,安阳 455000
Author(s):
Zhang Chao1; 2;  Wang Xianling1;  Chen Yulong1;  Guo Qinghua1;  Pei Yu1;  Du Jin1;  Dou Jingtao1;  Ba Jianming1;  Lyu Zhaohui1;  Mu Yiming1.
1Department of Endocrinology, the Chinese PLA General Hospital, Beijing 100853, China; 2Department of Endocrinology, Angang General Hospital, Anyang 455000, China
关键词:
POEMS综合征;  性腺功能减退症;  甲状腺功能减退症;  糖代谢异常;  肾上腺皮质功能减退症
Keywords:
POEMS syndrome;  Hypogonadism;  Hypothyroidism;  Abnormal glucose metabolism;  Adrenal insufficiency
DOI:
10.3760/cma.j.cn121383-20200413-04035
摘要:
目的 对POEMS综合征患者人群内分泌代谢异常的情况和临床特点进行分析和总结。方法 对2000年1月至2020年1月中国人民解放军总医院第一医学中心临床确诊的136例POEMS综合征患者的病例资料进行系统回顾性分析。结果 93.38%(127/136)的患者存在内分泌代谢异常,病变累及性腺、甲状腺、肾上腺、胰岛等多个内分泌器官。最常见的是性腺功能减退症(98/136,72.06%),其次是甲状腺功能减退症(83/136,61.03%)、低钙血症(50/136,36.76%)、高催乳素血症(47/136,34.56%)、糖代谢异常(41/136,30.15%)和肾上腺皮质功能减退症(41/136,30.15%)。单个内分泌器官受累占12.60%(16/127),多个内分泌器官受累占87.40%(111/127)。多个内分泌器官受累患者中, 2、3、4、5、6种内分泌器官受累的情况分别占29.92%(38/127)、30.71%(39/127)、17.32%(22/127)、7.09%(9/127)和2.36%(3/127)。POEMS综合征患者内分泌代谢异常复杂多变,发病至就诊的平均时间为412 d(198～1 055 d),住院确诊的平均时间为8 d(4～15 d)。结论 POEMS综合征患者内分泌代谢异常表现复杂多样,容易造成误诊、误治。因此,对临床确诊的POEMS综合征患者应当进行详尽的内分泌代谢异常评估并且适时进行激素补充替代治疗。
Abstract:
Objective To analyze and summarize the prevalence and clinical characteristics of endocrine-metabolic abnormalities in a large cohort with POEMS syndrome, and to improve the understanding of endocrine-metabolic abnormalities in this special disease.Methods The retrospective review was performed in patients with a diagnosis of POEMS syndrome at the First Medical Center of Chinese PLA General Hospital between January 2000 and January 2020. The clinical data about endocrine-metabolic abnormalities were extracted from their medical records.Results The prevalence of endocrine-metabolic abnormalities was 93.38%(127/136)including gonads, thyroid, adrenal, islets and other endocrine organs involved. Hypogonadism was the most common endocrine-metabolic abnormality(98/136, 72.06%), followed by hypothyroidism(83/136, 61.03%), hypocalcemia(50/136, 36.76%), hyperprolactinemia(47/136, 34.56%), abnormal glucose metabolism(41/136, 30.15%), and adrenal insufficiency(41/136, 30.15%). The prevalence of single endocrinopathy and multiple endocrinopathies were 12.60%(16/127)and 87.40%(111/127)respectively. In patients with multiple endocrinopathies, the percentage of 2, 3, 4, 5 and 6 kinds of endocrine axes involved were 29.92%(38/127), 30.71%(39/127), 17.32%(22/127), 7.09%(9/127), and 2.36%(3/127), respectively. The endocrine-metabolic abnormalities in patients with POEMS syndrome were complex and variable. The median time from symptoms onset to confirmed diagnosis of POEMS was 412 d(198-1 055 d), and the median time for clinically definite was 8 d(4-15 d).Conclusion The endocrine-metabolic abnormality is variable in POEMS syndrome, which can cause misdiagnosis and incorrect therapy. For patients with confirmed diagnosis of POEMS syndromes, a thorough endocrine evaluation should be performed and hormone supplement should be administrated timely.

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备注/Memo

备注/Memo:
通信作者:王先令,Email:wangxianling1972@sohu.com
更新日期/Last Update: 2021-01-20