Aydin Malik, Franke Ingo, Kurylowicz Lisa, Ganschow Rainer, Lentze Michael, Born Mark, Hagemann Rebekka
Department of General Pediatrics, University Children's Hospital Bonn, Bonn, Germany.
HELIOS University Medical Center Wuppertal, Children's Hospital, Center for Clinical and Translational Research (CCTR), Center for Biomedical Education and Research (ZBAF), Witten/Herdecke University, Heusnerstr. 40, 42283, Wuppertal, Germany.
Clin Exp Nephrol. 2019 May;23(5):676-688. doi: 10.1007/s10157-019-01696-8. Epub 2019 Feb 5.
Long-term outcomes of children with nephrotic syndrome have not been well described in the literature.
Cross-sectional study data analysis of n = 43 patients with steroid-sensitive (SSNS) and n = 7 patients with steroid-resistant (SRNS) nephrotic syndrome were retrospectively collected; patients were clinically examined at a follow-up visit (FUV), on average 30 years after onset, there was the longest follow-up period to date.
The mean age at FUV was 33.6 years (14.4-50.8 years, n = 41). The mean age of patients with SSNS at onset was 4.7 years (median 3.8 years (1.2-14.5 years), the mean number of relapses was 5.8 (0 to 29 relapses). Seven patients (16.3%) had no relapses. Eleven patients were "frequent relapsers" (25.6%) and four patients still had relapses beyond the age of 18 years. Except of cataracts and arterial hypertension, there were no negative long-term outcomes and only one patient was using immunosuppressant therapy at FUV. 55% of patients suffered from allergies and 47.5% had hypercholesterolemia. Two patients suffered a heart attack in adulthood. A younger age at onset (< 4 years) was a risk factor for frequent relapses. An early relapse (within 6 months after onset) was a risk factor and a low birth weight was not a significant risk factor for a complicated NS course. The mean age of patients with SRNS at onset was 4.6 ± 4.4 years and 27.5 ± 9.9 years at FUV. Three patients received kidney transplantations.
The positive long-term prognosis of SSNS can reduce the concern of parents about the probability of the child developing a chronic renal disease during the clinical course after onset.
肾病综合征患儿的长期预后在文献中尚未得到充分描述。
回顾性收集了n = 43例激素敏感型（SSNS）和n = 7例激素抵抗型（SRNS）肾病综合征患者的横断面研究数据分析；对患者进行了临床检查，随访平均在发病后30年，是迄今为止最长的随访期。
随访时的平均年龄为33.6岁（14.4 - 50.8岁，n = 41）。SSNS患者发病时的平均年龄为4.7岁（中位数3.8岁（1.2 - 14.5岁），平均复发次数为5.8次（0至29次复发）。7例患者（16.3%）未复发。11例患者为“频繁复发者（25.6%），4例患者在18岁后仍有复发。除白内障和动脉高血压外，无不良长期预后，随访时只有1例患者使用免疫抑制治疗。55%的患者患有过敏症，47.5%的患者患有高胆固醇血症。2例患者在成年后发生心脏病发作。发病时年龄较小（<4岁）是频繁复发的危险因素。早期复发（发病后6个月内）是危险因素，低出生体重不是NS病程复杂的显著危险因素。SRNS患者发病时的平均年龄为4.6±4.4岁，随访时为27.5±9.9岁。3例患者接受了肾移植。
SSNS良好的长期预后可减轻家长对患儿发病后临床过程中发展为慢性肾病可能性的担忧。
