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与儿童特发性肾病综合征及其治疗相关的并发症以及患者的相关风险

Complications Related to Childhood Idiopathic Nephrotic Syndrome, Its Treatment and the Associated Risks in Patients.

作者信息

Karakaya Deniz, Güngör Tülin, Kargın Çakıcı Evrim, Yazilitaş Fatma, Çelikkaya Evra, Bülbül Mehmet

机构信息

Pediatric Nephrology, Dr Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, TUR.

Pediatric Nephrology, Ankara Etlik City Hospital, Ankara, TUR.

出版信息

Cureus. 2023 Aug 22;15(8):e43929. doi: 10.7759/cureus.43929. eCollection 2023 Aug.

DOI:10.7759/cureus.43929
PMID:37746397
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10513119/
Abstract

Aim Nephrotic syndrome is the most common childhood glomerular disorder, but data on the associated complications are limited and predisposing risk factors have not been fully defined. The aim of this study was to evaluate disease- and treatment-related acute and chronic complications in patients with childhood idiopathic nephrotic syndrome (INS), and to identify the risk factors involved in the development of complications. Methods This single-center study was performed at the pediatric nephrology department of a tertiary pediatric hospital in Turkey. The study included 411 patients with a diagnosis of childhood INS, 128 of whom had disease-related and treatment-related complications. Patients diagnosed and followed-up between January 2010 and January 2022 were evaluated retrospectively. Results Complications occurred in 31.1% of the 411 patients. Mean age at the time of diagnosis was 7.54 ± 4.37 years, and the male/female ratio was 0.9:1. Among the patients with complications, 96.9% were disease-related, and 50.8% were treatment-related complications. In older age, high proteinuria level, a low estimated glomerular filtration rate (eGFR) level at diagnosis, and female gender were significant risk factors for complication development (P = 0.000, P = 0.006, P = 0.04, and P = 0.07, respectively). Chronic kidney disease (CKD) developed in 7% of patients and 2.9% of patients had end-stage renal disease (ESRD). Additionally, three of 12 patients with progressive ESRD underwent transplantation. Also the incidence of ESRD was significantly higher in the patients with complications than in those without complications (P < 0.05). Conclusion The present findings suggest that careful monitoring of patients with childhood INS at risk for complications and implementation of personalized treatment programs can improve long-term outcomes, especially in patients that progress to ESRD and are followed-up with dialysis or transplantation as targeted therapy.

摘要

目的 肾病综合征是儿童最常见的肾小球疾病,但相关并发症的数据有限,诱发风险因素尚未完全明确。本研究的目的是评估儿童特发性肾病综合征(INS)患者与疾病及治疗相关的急慢性并发症,并确定并发症发生的风险因素。方法 本单中心研究在土耳其一家三级儿童医院的儿科肾病科进行。该研究纳入了411例诊断为儿童INS的患者,其中128例有与疾病和治疗相关的并发症。对2010年1月至2022年1月期间诊断并随访的患者进行回顾性评估。结果 411例患者中有31.1%发生并发症。诊断时的平均年龄为7.54±4.37岁,男女比例为0.9:1。在有并发症的患者中,96.9%为与疾病相关的并发症,50.8%为与治疗相关的并发症。年龄较大、蛋白尿水平高、诊断时估计肾小球滤过率(eGFR)水平低以及女性是并发症发生的显著风险因素(P分别为0.000、0.006、0.04和0.07)。7%的患者发展为慢性肾脏病(CKD),2.9%的患者患有终末期肾病(ESRD)。此外,12例进行性ESRD患者中有3例接受了移植。并发症患者的ESRD发生率也显著高于无并发症患者(P<0.05)。结论 目前的研究结果表明,对有并发症风险的儿童INS患者进行仔细监测并实施个性化治疗方案可以改善长期预后,尤其是对于进展为ESRD并接受透析或移植作为靶向治疗的患者。

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Int J Nephrol Renovasc Dis. 2022 Feb 25;15:53-62. doi: 10.2147/IJNRD.S351053. eCollection 2022.
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Risk Factors for Poor Prognosis of Severe Infection in Children With Idiopathic Nephrotic Syndrome: A Double-Center, Retrospective Study.特发性肾病综合征患儿重症感染预后不良的危险因素:一项双中心回顾性研究
Front Pediatr. 2021 Jul 14;9:656215. doi: 10.3389/fped.2021.656215. eCollection 2021.
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Iron deficiency in proteinuric children with nephrotic syndrome: A cross-sectional pilot study.蛋白尿肾病综合征患儿的缺铁:一项横断面初步研究。
Arch Pediatr. 2021 Aug;28(6):485-487. doi: 10.1016/j.arcped.2021.05.005. Epub 2021 Jul 2.
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Steroid-Associated Side Effects in Patients With Primary Proteinuric Kidney Disease.原发性蛋白尿性肾病患者的类固醇相关副作用
Kidney Int Rep. 2019 Sep 9;4(11):1608-1616. doi: 10.1016/j.ekir.2019.08.019. eCollection 2019 Nov.
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Prognosis and acute complications at the first onset of idiopathic nephrotic syndrome in children: a nationwide survey in Japan (JP-SHINE study).日本全国性调查(JP-SHINE 研究):儿童特发性肾病综合征首次发病时的预后和急性并发症。
Nephrol Dial Transplant. 2021 Feb 20;36(3):475-481. doi: 10.1093/ndt/gfz185.
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