Mishra Om P, Sidar Minketan, Batra Vineeta V, Prasad Rajniti, Singh Ankur, Abhinay Abhishek, Mishra Akash, Yadav Ashish K
Banaras Hindu University, Institute of Medical Sciences, Department of Pediatrics, Division of Pediatric Nephrology, Varanasi, India.
G. B. Pant Institute of Post Graduate Medical Education & Research, Department of Pathology, New Delhi, India.
J Bras Nefrol. 2023 Apr-Jun;45(2):199-209. doi: 10.1590/2175-8239-JBN-2022-0073en.
Idiopathic steroid resistant nephrotic syndrome (SRNS) has variable outcomes in children. The primary objective of the present study was to assess the cumulative remission rate and the secondary objectives were to assess factors affecting the remission status, kidney function survival, and adverse effects of medications.
One hundred fourteen patients with SRNS were included. Calcineurin inhibitor-based treatment protocol along with prednisolone and angiotensin-converting enzyme inhibitor were used, and patients were followed over 5 years.
Median age was 4.5 years; 53.5% of cases were between 1 to 5 years of age. Sixty-two patients (54.4%) were at initial stage and 52 (45.6%) were at a late SRNS stage. Median eGFRcr was 83.5 mL/min/1.73m2 at presentation. Of the 110 patients, 63 (57.3%) achieved remission [complete remission 30 (27.3%), partial remission 33 (30%)], and 47 (42.7%) had no remission. Kidney function survival was 87.3% and 14 cases (12.7%) had progression to CKD (G3-8, G4-3, G5-1, and G5D-2). Median duration of follow up was 36 months (IQR 24, 60). Age of onset, cyclosporine/tacrolimus, eGFRcr, and histopathology (MCD/FSGS) did not affect remission. Similarly, remission status in addition to age of onset, drug protocol, and histopathology did not significantly affect kidney function during a period of 5 years. Hypertension, cushingoid facies, short stature, cataract, and obesity were observed in 37.7, 29.8, 25.5, 17.5, and 0.7% of cases, respectively.
About half of the cases achieved remission. Age of onset of disease, cyclosporine/tacrolimus use, and histopathological lesion neither affected remission status nor short-term kidney function survival in SRNS.
特发性类固醇抵抗性肾病综合征（SRNS）在儿童中的预后各不相同。本研究的主要目的是评估累积缓解率，次要目的是评估影响缓解状态、肾功能存活以及药物不良反应的因素。
纳入114例SRNS患者。采用基于钙调神经磷酸酶抑制剂的治疗方案，联合泼尼松龙和血管紧张素转换酶抑制剂，并对患者进行了5年的随访。
中位年龄为4.5岁；53.5%的病例年龄在1至5岁之间。62例患者（54.4%）处于初始阶段，52例（45.6%）处于SRNS晚期。就诊时中位估算肾小球滤过率（eGFRcr）为83.5 mL/min/1.73m²。110例患者中，63例（57.3%）实现缓解[完全缓解30例（27.3%），部分缓解33例（30%）]，47例（42.7%）未缓解。肾功能存活率为87.3%，14例（12.7%）进展为慢性肾脏病（G3 - 8例，G4 - 3例，G5 - 1例，G5D - 2例）。中位随访时间为36个月（四分位间距24, 60）。发病年龄、环孢素/他克莫司、eGFRcr和组织病理学（微小病变肾病/局灶节段性肾小球硬化）不影响缓解。同样，除发病年龄、药物方案和组织病理学外，缓解状态在5年期间对肾功能也无显著影响。分别有37.7%、29.8%、25.5%、17.5%和 的病例出现高血压、库欣样面容、身材矮小、白内障和肥胖。
约一半的病例实现缓解。疾病发病年龄、环孢素/他克莫司的使用以及组织病理学病变既不影响SRNS的缓解状态，也不影响短期肾功能存活。
