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利用临床个人记录全国登记处分析日本原发性膜性增殖性肾小球肾炎患者的人口统计学和治疗情况。

Demographics and treatment of patients with primary membranoproliferative glomerulonephritis in Japan using a national registry of clinical personal records.

机构信息

Division of Cardiology, Nephrology, Pulmonology and Neurology, Department of Internal Medicine, Asahikawa Medical University, 2-1-1-1 Midorigaoka-Higashi, Asahikawa, Japan.

Reverse Translational Research Project, Center for Rare Disease Research, National Institutes of Biomedical Innovation, Health and Nutrition (NIBIOHN), Ibaraki, Japan.

出版信息

Clin Exp Nephrol. 2023 Nov;27(11):928-935. doi: 10.1007/s10157-023-02387-1. Epub 2023 Jul 29.

Abstract

BACKGROUND

Membranoproliferative glomerulonephritis (MPGN) is a rare glomerular injury that causes nephrotic syndrome and end-stage kidney disease. The nationwide demographics and treatment of Japanese patients with primary MPGN have not yet been reported.

METHODS

We collected clinical personal records of patients with primary MPGN between 2015 and 2018 from the national registry organized by the Japanese Ministry of Health, Labour, and Welfare and investigated the characteristics of primary MPGN throughout Japan.

RESULTS

Of 258 patients with primary MPGN, 199 and 59 showed nephrotic and non-nephrotic syndrome, respectively. The median age at onset was higher in patients with nephrotic syndrome than in those with non-nephrotic syndrome (45 [24-63] vs. 35 [14-53] years, respectively; P = 0.010). The use of oral prednisolone was significantly higher in patients with nephrotic syndrome than in those with non-nephrotic syndrome (73.9% vs. 59.3%, respectively; P = 0.032). When patients were divided into three age groups: adolescent and young adult group (≤ 39 years; n = 80), middle adult group (40-64 years; n = 111), and older adult group (≥ 65 years; n = 67), the use of oral prednisolone, cyclosporine, and mizoribine was significantly higher in the adolescent and young adult group than in the middle adult group. The mean dosage of oral prednisolone and mizoribine showed no differences among the three age groups.

CONCLUSION

The national registry of clinical personal records of primary MPGN could provide an informative insight into the characteristics, clinical features, and treatment approaches for patients with primary MPGN in Japan.

摘要

背景

膜增生性肾小球肾炎(MPGN)是一种罕见的肾小球损伤,可导致肾病综合征和终末期肾病。目前尚未报道日本原发性 MPGN 患者的全国人口统计学和治疗情况。

方法

我们从日本厚生劳动省组织的全国登记处收集了 2015 年至 2018 年间原发性 MPGN 患者的临床个人记录,并调查了日本各地原发性 MPGN 的特征。

结果

在 258 例原发性 MPGN 患者中,199 例表现为肾病综合征,59 例表现为非肾病综合征。肾病综合征患者的发病年龄中位数高于非肾病综合征患者(分别为 45[24-63]岁和 35[14-53]岁;P=0.010)。肾病综合征患者口服泼尼松龙的使用率明显高于非肾病综合征患者(分别为 73.9%和 59.3%;P=0.032)。当患者分为青少年和年轻成年组(≤39 岁;n=80)、中年组(40-64 岁;n=111)和老年组(≥65 岁;n=67)时,青少年和年轻成年组口服泼尼松龙、环孢素和米佐布宁的使用率明显高于中年组。三组患者口服泼尼松龙和米佐布宁的平均剂量无差异。

结论

原发性 MPGN 的临床个人记录全国登记处可以为了解日本原发性 MPGN 患者的特征、临床特征和治疗方法提供有价值的信息。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/329e/10581954/04ab719e9dba/10157_2023_2387_Fig1_HTML.jpg

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