Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome

To investigate the clinical efficacy of sirolimus in treating children with refractory nephrotic syndrome, the clinical data for 22 children from the Children's Hospital of Hebei Province were analyzed retrospectively. There were 16 boys and six girls, and the treatment period was from Septembe...

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Main Authors: Nan Zhang, Le Jiang, Sanni Li, Yingying Zheng, Lei Dong, Yile Zhao
Format: Article
Language:English
Published: Taylor & Francis Group 2024-12-01
Series:Renal Failure
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Online Access:https://www.tandfonline.com/doi/10.1080/0886022X.2024.2404486
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author Nan Zhang
Le Jiang
Sanni Li
Yingying Zheng
Lei Dong
Yile Zhao
author_facet Nan Zhang
Le Jiang
Sanni Li
Yingying Zheng
Lei Dong
Yile Zhao
author_sort Nan Zhang
collection DOAJ
description To investigate the clinical efficacy of sirolimus in treating children with refractory nephrotic syndrome, the clinical data for 22 children from the Children's Hospital of Hebei Province were analyzed retrospectively. There were 16 boys and six girls, and the treatment period was from September 2015 to April 2021. There were two patients with steroid-dependent nephrotic syndrome (SDNS), six patients with frequently relapsing nephrotic syndrome (FRNS), and 14 patients with steroid-resistant nephrotic syndrome (SRNS). All patients were defined as having refractory nephrotic syndrome. There were 12 patients (including nine SRNS patients and three FRNS patients) with minimal change disease (MCD), three patients (three SRNS patients) with focal segmental glomerular sclerosis (FSGS), one FRNS patient with mesangial proliferative glomerulonephritis (MsPGN), and six patients without a kidney biopsy. Compared with levels before sirolimus treatment, 24-hour urine protein (24-h UP), low-density lipoprotein cholesterol (LDL-C), urea (Ur) and serum creatinine (SCr) levels were significantly lower (all p < 0.05). Moreover, albumin (Alb) was significantly increased (p < 0.05), and there were no significant differences in total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), triglyceride (TG), immunoglobulin A (IgA), immunoglobulin G (IgG) or immunoglobulin M (IgM) (all p > 0.05) at the first follow-up. Sirolimus is effective as the first treatment of some children with refractory nephrotic syndrome, but its long-term efficacy and adverse reactions still require follow-up.
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spelling doaj-art-2e0a5d16bd384cf3a558ae82e76e237a2025-08-20T02:29:56ZengTaylor & Francis GroupRenal Failure0886-022X1525-60492024-12-0146210.1080/0886022X.2024.2404486Clinical analysis of sirolimus therapy in children with refractory nephrotic syndromeNan Zhang0Le Jiang1Sanni Li2Yingying Zheng3Lei Dong4Yile Zhao5Department of Pharmacy, Children's Hospital of Hebei Province Affiliated with Hebei Medical University, Shijiazhuang, ChinaOffice of Academic Research, The Second Hospital of Hebei Medical University, Shijiazhuang, ChinaDepartment of Pharmacy, Children's Hospital of Hebei Province Affiliated with Hebei Medical University, Shijiazhuang, ChinaDepartment of Pharmacy, The Third Hospital of Hebei Medical University, Shijiazhuang, ChinaDepartment of Pharmacy, Children's Hospital of Hebei Province Affiliated with Hebei Medical University, Shijiazhuang, ChinaDepartment of Pharmacy, Children's Hospital of Hebei Province Affiliated with Hebei Medical University, Shijiazhuang, ChinaTo investigate the clinical efficacy of sirolimus in treating children with refractory nephrotic syndrome, the clinical data for 22 children from the Children's Hospital of Hebei Province were analyzed retrospectively. There were 16 boys and six girls, and the treatment period was from September 2015 to April 2021. There were two patients with steroid-dependent nephrotic syndrome (SDNS), six patients with frequently relapsing nephrotic syndrome (FRNS), and 14 patients with steroid-resistant nephrotic syndrome (SRNS). All patients were defined as having refractory nephrotic syndrome. There were 12 patients (including nine SRNS patients and three FRNS patients) with minimal change disease (MCD), three patients (three SRNS patients) with focal segmental glomerular sclerosis (FSGS), one FRNS patient with mesangial proliferative glomerulonephritis (MsPGN), and six patients without a kidney biopsy. Compared with levels before sirolimus treatment, 24-hour urine protein (24-h UP), low-density lipoprotein cholesterol (LDL-C), urea (Ur) and serum creatinine (SCr) levels were significantly lower (all p < 0.05). Moreover, albumin (Alb) was significantly increased (p < 0.05), and there were no significant differences in total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), triglyceride (TG), immunoglobulin A (IgA), immunoglobulin G (IgG) or immunoglobulin M (IgM) (all p > 0.05) at the first follow-up. Sirolimus is effective as the first treatment of some children with refractory nephrotic syndrome, but its long-term efficacy and adverse reactions still require follow-up.https://www.tandfonline.com/doi/10.1080/0886022X.2024.2404486Sirolimuschildrefractory nephrotic syndrometreatment
spellingShingle Nan Zhang
Le Jiang
Sanni Li
Yingying Zheng
Lei Dong
Yile Zhao
Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
Renal Failure
Sirolimus
child
refractory nephrotic syndrome
treatment
title Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
title_full Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
title_fullStr Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
title_full_unstemmed Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
title_short Clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
title_sort clinical analysis of sirolimus therapy in children with refractory nephrotic syndrome
topic Sirolimus
child
refractory nephrotic syndrome
treatment
url https://www.tandfonline.com/doi/10.1080/0886022X.2024.2404486
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