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儿科和青少年/年轻成人肾脏病患者的临床和组织学特征:日本肾活检登记处(J-RBR)的横断面分析。

Clinical and histological features in pediatric and adolescent/young adult patients with renal disease: a cross-sectional analysis of the Japan Renal Biopsy Registry (J-RBR).

机构信息

Department of Pediatrics, Institute of Biomedical Sciences, Tokushima University Graduate School, Kuramoto-cho 3-18-15, Tokushima, Tokushima, 770-8503, Japan.

Deparment of Internal Medicine, Sendai Hospital of East Japan Railway Company, Sendai, Japan.

出版信息

Clin Exp Nephrol. 2021 Sep;25(9):1018-1026. doi: 10.1007/s10157-021-02077-w. Epub 2021 May 28.

Abstract

BACKGROUND

Only a few studies have investigated epidemiological and clinicopathological information regarding pediatric and adolescent and young adult (AYA) patients with renal disease. The purpose of this study was to clarify the differences and relationship of clinicopathological findings between pediatric and AYA patients using the Japan Renal Biopsy Registry (J-RBR).

METHODS

This cross-sectional study analyzed data from patients registered in the J-RBR between 2007 and 2017. Clinicopathological findings at diagnosis were analyzed for 3,463 pediatric (age < 15 years) and 6,532 AYA (age 15-30 years) patients.

RESULTS

Although chronic nephritic syndrome was the most common clinical diagnosis at age > 5 years, nephrotic syndrome was the most frequent diagnosis at age < 4 years. The most common pathological diagnosis as classified by pathogenesis in pediatric patients was primary glomerular disease (except IgA nephropathy), whereas IgA nephropathy was increased in AYA patients. Mesangial proliferative glomerulonephritis was the most common pathological diagnosis as classified by histopathology in both pediatric and AYA patients. Minor glomerular abnormalities were the most frequent histopathologic diagnoses of nephrotic syndrome in childhood, but their frequency decreased with age.

CONCLUSION

To the best of our knowledge, this is the first report of clinicopathological features of pediatric and AYA patients in a large nationwide registry of renal biopsy. There were differences of clinical, pathological and histopathologic findings between pediatric and AYA patients.

摘要

背景

仅有少数研究调查了儿科和青少年及年轻成人(AYA)肾病患者的流行病学和临床病理信息。本研究的目的是使用日本肾活检登记处(J-RBR)阐明儿科和 AYA 患者的临床病理发现之间的差异和关系。

方法

本横断面研究分析了 2007 年至 2017 年间登记在 J-RBR 中的患者数据。分析了 3463 名儿科(年龄<15 岁)和 6532 名 AYA(年龄 15-30 岁)患者的诊断时临床病理发现。

结果

尽管慢性肾炎综合征是年龄>5 岁时最常见的临床诊断,但肾病综合征是年龄<4 岁时最常见的诊断。儿科患者中按发病机制分类最常见的病理诊断是原发性肾小球疾病(IgA 肾病除外),而 AYA 患者中 IgA 肾病增加。系膜增生性肾小球肾炎是儿科和 AYA 患者中最常见的组织病理学诊断。在儿童肾病综合征中,微小肾小球异常是最常见的组织病理学诊断,但随着年龄的增长,其频率逐渐降低。

结论

据我们所知,这是首次在大型全国性肾活检登记处报告儿科和 AYA 患者的临床病理特征。儿科和 AYA 患者的临床、病理和组织病理发现存在差异。

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