A retrospective analysis of endocrinopathy manifestations in 136 Chinese patients with POEMS syndrome.

IF 0.6 4区医学 Q4 ENDOCRINOLOGY & METABOLISM Neuro endocrinology letters Pub Date : 2021-09-10

Yi Shao, Chao Zhang, Xianling Wang, Qinghua Gu, Jianming Ba, Zhaohui Lv, Jingtao Dou, Yiming Mu

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Abstract

Objective: POEMS syndrome is a rare multisystemic disease, with a wide spectrum of clinical endocrinopathies. Patients with POEMS syndrome may present with one or more hormone disorders during the disease course, but such phenomenon has usually been underestimated. In this report, we analyzed the prevalence and clinical characteristics of endocrine abnormalities in a large Chinese cohort with POEMS syndrome.

Methods: This retrospective review was performed in patients with a definite diagnosis of POEMS syndrome who were hospitalized in our hospital between January 2000 and January 2020. The clinical data about endocrine abnormalities were extracted from their medical records and analyzed.

Results: This study comprised 136 patients (95 male, 41 female) with a median age of 48(40-56) years old. Endocrine abnormalities were frequent (127 cases, 93.38%) in patients with POEMS syndrome. The prevalence of single endocrinopathy and multiple endocrinopathies were 12.60% (16/127 cases) and 87.40% (111/127), respectively. The most frequent endocrinopathy was hypogonadism (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%). In patients with multiple endocrinopathies, the prevalence 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. Such hormone disorders cause complex clinical presentations, including overt or subclinical situations.

Conclusion: Endocrinopathy manifestations in POEMS syndrome are more frequent, and its clinical complicacy should be emphasized in differential diagnosis. For patients with a definite diagnosis of POEMS syndrome, an early and thorough endocrine evaluation should be performed.

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对136名中国POEMS综合征患者内分泌病症表现的回顾性分析。

目的：POEMS 综合征是一种罕见的多系统疾病，具有广泛的临床内分泌病变。POEMS 综合征患者在病程中可能出现一种或多种激素紊乱，但这种现象通常被低估。在这份报告中，我们分析了中国大样本 POEMS 综合征患者内分泌异常的发生率和临床特征：方法：我们对 2000 年 1 月至 2020 年 1 月期间在我院住院的明确诊断为 POEMS 综合征的患者进行了回顾性研究。从病历中提取内分泌异常的临床数据并进行分析：本研究包括 136 名患者（95 名男性，41 名女性），中位年龄为 48（40-56）岁。在 POEMS 综合征患者中，内分泌异常是常见病（127 例，占 93.38%）。单一内分泌病变和多重内分泌病变的发病率分别为12.60%（16/127例）和87.40%（111/127例）。最常见的内分泌疾病是性腺功能减退（98/136，72.06%），其次是甲状腺功能减退（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%）和肾上腺功能不全（41/136，30.15%）。在多发性内分泌疾病患者中，涉及 2、3、4、5 和 6 种内分泌轴的比例分别为 29.92%（38/127）、30.71%（39/127）、17.32%（22/127）、7.09%（9/127）和 2.36%（3/127）。这些激素紊乱导致复杂的临床表现，包括显性或亚临床情况：结论：POEMS 综合征的内分泌病变表现更为常见，在鉴别诊断时应重视其临床复杂性。对于确诊为 POEMS 综合征的患者，应及早进行全面的内分泌评估。

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来源期刊

Neuro endocrinology letters 医学-内分泌学与代谢

CiteScore

1.00

自引率

14.30%

发文量

审稿时长

6 months

期刊介绍： Neuroendocrinology Letters is an international, peer-reviewed interdisciplinary journal covering the fields of Neuroendocrinology, Neuroscience, Neurophysiology, Neuropsychopharmacology, Psychoneuroimmunology, Reproductive Medicine, Chronobiology, Human Ethology and related fields for RAPID publication of Original Papers, Review Articles, State-of-the-art, Clinical Reports and other contributions from all the fields covered by Neuroendocrinology Letters. Papers from both basic research (methodology, molecular and cellular biology, anatomy, histology, biology, embryology, teratology, normal and pathological physiology, biophysics, pharmacology, pathology and experimental pathology, biochemistry, neurochemistry, enzymology, chronobiology, receptor studies, endocrinology, immunology and neuroimmunology, animal physiology, animal breeding and ethology, human ethology, psychology and others) and from clinical research (neurology, psychiatry and child psychiatry, obstetrics and gynecology, pediatrics, endocrinology, immunology, cardiovascular studies, internal medicine, oncology and others) will be considered. The Journal publishes Original papers and Review Articles. Brief reports, Special Communications, proved they are based on adequate experimental evidence, Clinical Studies, Case Reports, Commentaries, Discussions, Letters to the Editor (correspondence column), Book Reviews, Congress Reports and other categories of articles (philosophy, art, social issues, medical and health policies, biomedical history, etc.) will be taken under consideration.