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俄罗斯原发性甲状旁腺功能亢进症登记处,最新更新。

The Russian registry of primary hyperparathyroidism, latest update.

机构信息

Directorate, Endocrinology Research Centre, Moscow, Russia.

Department of Parathyroid Glands Pathology, Endocrinology Research Centre, Moscow, Russia.

出版信息

Front Endocrinol (Lausanne). 2023 Jul 3;14:1203437. doi: 10.3389/fendo.2023.1203437. eCollection 2023.

DOI:10.3389/fendo.2023.1203437
PMID:37465121
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10352025/
Abstract

INTRODUCTION

Until recently no major epidemiological research of primary hyperparathyroidism (PHPT) has been conducted in the Russian Federation, this led to the creation of the Russian online registry. The objective of this study is to estimate the clinical and biochemical profile, classical and non-classical complications, surgical intervention and medical therapy of the patients with different forms of PHPT in the Russian Federation.

MATERIALS AND METHODS

The cross-sectional, observational, continuous study was conducted at the Endocrinology Research Centre (Moscow). The present study explored retrospective data from 6003 patients submitted to the Registry between 12.12.2016 and 25.10.2022 from 81 regions of the Russian Federation (http://pgpt.clin-reg.ru/).

RESULTS

The median age was 59 [60; 66] years with a female:male ratio of 11.7:1. Symptomatic PHPT was observed in 74.3% while asymptomatic form - only in 25.7% of cases. Bone pathology was the predominant clinical manifestation in 62.5% of cases (n=2293), mostly in combination with visceral complications 45.7% (n=1676). The majority of patients (63.3%) had combined visceral disorders including kidney damage in 51.8% and gastroduodenal erosions/ulcers in 32.3% of patients. Symptomatic patients were older (60 [53; 67] vs. 54 [45; 62] years, p<0.001) and had more severe biochemical alterations of calcium-phosphorus metabolism. Cardiovascular disease (СVD) was recorded in 48% of patients, among them the most frequent was arterial hypertension (up to 93.9%). A genetic test was conducted in 183 cases (suspicious for hereditary PHPT) revealing the mutations in , , genes in 107, 6 and 2 cases, respectively. Surgery was performed in 53.4% of patients with remission achievement in 87%, the relapse/persistence were recorded in 13% of cases. Histological examination revealed carcinoma in 4%, atypical adenoma in 2%, adenoma in 84% and hyperplasia in 11% of cases. Drug therapy was prescribed in 54.0% of cases, most often cholecalciferol.

CONCLUSION

The detection rate of PHPT has increased in the Russian Federation in recent years. This increase is associated with the start of online registration. However, the majority of patients remain symptomatic with significant alterations of phosphorus-calcium metabolism that indicates delayed diagnosis and requires further modifications of medical care.

摘要

简介

直到最近,俄罗斯联邦还没有进行过原发性甲状旁腺功能亢进症(PHPT)的主要流行病学研究,这导致了俄罗斯在线注册系统的建立。本研究旨在评估俄罗斯联邦不同形式 PHPT 患者的临床和生化特征、经典和非经典并发症、手术干预和药物治疗。

材料和方法

这是一项在内分泌学研究中心(莫斯科)进行的横断面、观察性、连续性研究。本研究探索了 2016 年 12 月 12 日至 2022 年 10 月 25 日期间,来自俄罗斯联邦 81 个地区的 6003 名患者向注册系统提交的回顾性数据(http://pgpt.clin-reg.ru/)。

结果

中位年龄为 59[60;66]岁,女性与男性的比例为 11.7:1。有症状的 PHPT 占 74.3%,而无症状形式仅占 25.7%。骨病变是 62.5%(n=2293)患者的主要临床表现,主要与内脏并发症 45.7%(n=1676)相结合。大多数患者(63.3%)存在合并的内脏疾病,包括 51.8%的肾脏损害和 32.3%的胃十二指肠糜烂/溃疡。有症状的患者年龄更大(60[53;67] vs. 54[45;62]岁,p<0.001),且钙磷代谢的生化改变更严重。心血管疾病(CVD)在 48%的患者中被记录,其中最常见的是高血压(高达 93.9%)。对 183 例(怀疑遗传性 PHPT)患者进行了基因检测,分别在 、 、 基因中发现了 107、6 和 2 例突变。对 53.4%的患者进行了手术,其中 87%患者获得缓解,13%的患者出现复发/持续存在。组织学检查发现 4%的患者患有癌,2%的患者患有非典型腺瘤,84%的患者患有腺瘤,11%的患者患有增生。54.0%的患者接受了药物治疗,最常使用胆钙化醇。

结论

近年来,俄罗斯联邦的 PHPT 检出率有所增加。这种增加与在线注册的开始有关。然而,大多数患者仍有症状,磷钙代谢显著改变,这表明诊断延迟,需要进一步修改医疗护理。

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