Long-Term Outcome of Secondary Steroid-Resistant Nephrotic Syndrome in Chinese Children

Ying, Daojing; Liu, Wangkai; Chen, Lizhi; Rong, Liping; Lin, Zhilang; Wen, Sijia; Zhuang, Hongkai; Li, Jinhua; Jiang, Xiaoyun

doi:10.1016/j.ekir.2021.05.001

Cited by 5 publications

(8 citation statements)

References 29 publications

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“…The study showed that the clinical features of secondary steroid resistance with respect to gender, age and blood pressure was similar compared to secondary SRNS reported in previous studies. 2 A complete or partial response to therapy in the first year after being diagnosed as secondary SRNS did not show any relation with respect to progression to CKD. The predominant histological finding of MCD in patients was similar to a previous study.…”

Section: Discussionmentioning

confidence: 81%

“…The predominant histological finding of MCD in patients was similar to a previous study. 2,3,12 Other studies related to primary SRNS reported FSGS as the primary finding. 4,8 CR and PR at 1 year the current study was similar to a study which reported CR 71.4% and PR 16%, while a multicentre study reported a remission rate of 69%.…”

Section: Discussionmentioning

confidence: 99%

“…Conversely, some patients with initial SSNS may experience secondary SRNS during subsequent relapses. 2 Secondary SRNS, also referred to as late SRNS or late steroid nonresponders, accounts for 13.8% to 35.9% of SRNS cases. 3 Typically, a positive prognosis and eventual resolution of the relapsing NS are linked to minimal change disease (MCD) histology and responsiveness to steroid treatment.…”

Section: Introductionmentioning

confidence: 99%

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A retrospective analysis of clinicopathological features and outcome of secondary steroid resistant nephrotic syndrome

Qaiser,

Bajeer,

Khatri

et al. 2024

J Pak Med Assoc

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Objective: To determine the clinico-pathological features and long-term outcome of secondary steroid-resistant nephrotic syndrome treated with steroids and calcineurin inhibitors. Method: The retrospective cohort study was conducted at the Sindh Institute of Urology and Transplant, Karachi, in June and July 2023, and comprised data from January 1, 2008, to December 31, 2020, of children aged 1-18 years who developed steroid resistance after initial sensitivity to steroids with at least 1-year of follow-up. Demographics as well as time taken to secondary steroid response were documented. Renal biopsy of all patients with secondary steroid resistance had been performed. Eventual outcomes after treatment with calcineurin inhibitors based on the degree of proteinuria and serum albumin levels were used to categorise complete remission, partial remission and no response. Kidney function, as determined by estimated glomerular filtration rate, was recorded. Data was analysed using SPSS 22. Results: Of the 1,000 patients who underwent renal biopsy for steroid resistance, 48(4.8%) had idiopathic steroid-resistant nephrotic syndrome; 32(66.7%) males, 16(33.3%) females and median age of 5 years (interquartile range: 4-7.3 years). Median age at diagnosis of nephrotic syndrome was 5 years (interquartile range: 3.6-7.3 years). The median time from nephrotic syndrome to secondary steroid-resistant nephrotic syndrome was 23 months (interquartile range: 8.75-44.5 months). Biopsy results at diagnosis showed that 27(56.3%) had minimal change disease. The mean follow-up time was 6.1±3.2 years. Of the 43(89.5%) patients who received cyclosporin for 1 year, 29(67%) obtained complete remission, 5(12%) attained partial remission and no response was seen in 9(21%) patients. Conclusion: Majority of the children had minimal change disease at the time of diagnosis of secondary steroid-resistant nephrotic syndrome. The long-term response with calcineurin inhibitors was favourable at 1 year. Key Words: Secondary steroid-resistant nephrotic syndrome, Late non-responder nephrotic syndrome, Calcineurin inhibitors, Childhood nephrotic syndrome.

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Section: Discussionmentioning

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A retrospective analysis of clinicopathological features and outcome of secondary steroid resistant nephrotic syndrome

Qaiser,

Bajeer,

Khatri

et al. 2024

J Pak Med Assoc

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“…The prognosis of primary FSGS varies according to its response to GCs ( Mason et al, 2020 ; Zhao and Liu, 2020 ). Steroid-resistant FSGS is an independent risk factor for decreased renal function ( Rosenberg and Kopp, 2017 ; Ying et al, 2021 ). At present, the main methods for the treatment of MCD, FSGS, and MN include angiotensin-converting enzyme inhibitors or angiotensin receptor blockers, steroid hormones, CNIs (cyclosporine or tacrolimus), cyclophosphamide, or CD20 monoclonal antibody.…”

Section: Protective Effects Of Traditional Chinese Medicine and Activ...mentioning

confidence: 99%

Traditional Chinese Medicine in Treating Primary Podocytosis: From Fundamental Science to Clinical Research

Tian

Ren

et al. 2022

Front. Pharmacol.

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Podocytes form a key component of the glomerular filtration barrier. Damage to podocytes is referred to as “podocyte disease.” There are many causes of podocyte injury, including primary injury, secondary injury, and gene mutations. Primary podocytosis mostly manifests as nephrotic syndrome. At present, first-line treatment is based on glucocorticoid administration combined with immunosuppressive therapy, but some patients still progress to end-stage renal disease. In Asia, especially in China, traditional Chinese medicine (TCM) still plays an important role in the treatment of kidney diseases. This study summarizes the potential mechanism of TCM and its active components in protecting podocytes, such as repairing podocyte injury, inhibiting podocyte proliferation, reducing podocyte apoptosis and excretion, maintaining podocyte skeleton structure, and upregulating podocyte-related protein expression. At the same time, the clinical efficacy of TCM in the treatment of primary podocytosis (including idiopathic membranous nephropathy, minimal change disease, and focal segmental glomerulosclerosis) is summarized to support the development of new treatment strategies for primary podocytosis.

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“…For the genetic subgroup of SRNS, complete remission is more uncommon. Around 20% of genetic SRNS patients have chronic kidney disease (CKD), progress to kidney failure (KF) (Trautmann et al, 2023 ) and no response to immunosuppression in the first year is a predictor of poor kidney function outcomes (Ying et al, 2021 ). The most common histological diagnoses in children with NS include minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranoproliferative glomerulonephritis and membranous glomerulonephritis (Churg et al, 1970 ).…”

Section: Introductionmentioning

confidence: 99%

Genetic analysis and outcomes of Omani children with steroid‐resistant nephrotic syndrome

Al Riyami,

Al Alawi,

Al Gaithi

et al. 2023

Molec Gen & Gen Med

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BackgroundNephrotic syndrome (NS) is one of the most common kidney disorders seen by pediatric nephrologists and is defined by the presence of heavy proteinuria (>3.5 g/24 h), hypoalbuminemia (<3.5 g/dL), edema, and hyperlipidemia. Most children with NS are steroid‐responsive and have a good prognosis following treatment with prednisolone. However, 10%–20% of them have steroid‐resistant nephrotic syndrome (SRNS) and fail to respond to treatment. A significant proportion of these children progress to kidney failure.MethodsThis retrospective study aimed to determine the underlying genetic causes of SRNS among Omani children below 13 years old, over a 15‐year period and included 77 children from 50 different families. We used targeted Sanger sequencing combined with next‐generation sequencing approaches to perform molecular diagnostics.ResultsWe found a high rate of underlying genetic causes of SRNS in 61 (79.2%) children with pathogenic variants in the associated genes. Most of these genetically solved SRNS patients were born to consanguineous parents and variants were in the homozygous state. Pathogenic variants in NPHS2 were the most common cause of SRNS in our study seen in 37 (48.05%) cases. Pathogenic variants in NPHS1 were also seen in 16 cases, especially in infants with congenital nephrotic syndrome (CNS). Other genetic causes identified included pathogenic variants in LAMB2, PLCE1, MYO1E, and NUP93.ConclusionNPHS2 and NPHS1 genetic variants were the most common inherited causes of SRNS in Omani children. However, patients with variants in several other SRNS causative genes were also identified. We recommend screening for all genes responsible for SRNS in all children who present with this phenotype, which will assist in clinical management decisions and genetic counseling for the affected families.

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Long-Term Outcome of Secondary Steroid-Resistant Nephrotic Syndrome in Chinese Children

Cited by 5 publications

References 29 publications

A retrospective analysis of clinicopathological features and outcome of secondary steroid resistant nephrotic syndrome

A retrospective analysis of clinicopathological features and outcome of secondary steroid resistant nephrotic syndrome

Traditional Chinese Medicine in Treating Primary Podocytosis: From Fundamental Science to Clinical Research

Genetic analysis and outcomes of Omani children with steroid‐resistant nephrotic syndrome

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