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儿童肾病综合征中类固醇抵抗的临床预测因素。

Clinical Predictors of Steroid Resistance in Childhood Nephrotic Syndrome.

机构信息

Department of Pediatric Nephrology, School of Medicine, Marmara University, Istanbul, Turkey.

出版信息

Clin Pediatr (Phila). 2024 Oct;63(9):1300-1307. doi: 10.1177/00099228231219109. Epub 2023 Dec 24.

Abstract

We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.9 (5.7; 0.1-16.8) and 5.5 (5.4; 0.1-20.3) years. A total of 82.7% of the patients were steroid-sensitive nephrotic syndrome (SSNS) and 17.3% were steroid-resistant nephrotic syndrome (SRNS). The median age at first presentation was lower in the SSNS group ( = .002). The most common histopathological findings were focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD). Hypertension and macroscopic and microscopic hematuria were higher in the SRNS group ( < .001). The age and microscopic hematuria were independent risk factors for steroid resistance ( = .019 and = .002, respectively). Complement 3 (C3) was evaluated in 148 patients and found low in 7 patients who were subsequently diagnosed as membranoproliferative glomerulonephritis. There is still no better clinical predictor for steroid response than late age of onset and microscopic hematuria. Hypertension may also give a hint for potential steroid resistance.

摘要

我们旨在评估单中心 162 例肾病综合征(NS)患者的临床参数、组织病理学发现及预测激素抵抗的独立因素。该回顾性队列研究纳入了 1998 年至 2018 年间随访的 162 例 NS 患儿(57 名女孩和 105 名男孩)。中位(四分位距;范围)年龄和随访时间分别为 4.9(5.7;0.1-16.8)和 5.5(5.4;0.1-20.3)岁。82.7%的患者为激素敏感型肾病综合征(SSNS),17.3%为激素抵抗型肾病综合征(SRNS)。SSNS 组首次就诊的中位年龄较低( =.002)。最常见的组织病理学发现为局灶节段性肾小球硬化(FSGS)和微小病变性肾病(MCD)。SRNS 组高血压和肉眼血尿及镜下血尿发生率更高(<.001)。年龄和镜下血尿是激素抵抗的独立危险因素( =.019 和 =.002)。148 例患者评估了补体 3(C3),其中 7 例发现 C3 降低,随后诊断为膜增生性肾小球肾炎。目前,尚没有比发病年龄晚和镜下血尿更好的激素反应预测指标。高血压也可能提示潜在的激素抵抗。

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