Clinicopathological study of nephrotic syndrome in Indian children :A tertiary care experience

Chaudhari, Chetan; Gadgil, Nitin M; Kumavat, Prashant Vijay; Kshirsagar, Ganesh Ramdas; Dhamne, Sagar

doi:10.21276/apalm.2017.995

Cited by 3 publications

(1 citation statement)

References 7 publications

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“…Nephrotic syndrome (NS) defines as a heterozygous group of disorders with clinical features like proteinuria, hypoalbuminemia, hyperlipidemia, and edema [1,2]. The prevalence of nephrotic syndrome ranges from 12-16 per 100,001, and the annual impact in children ranges from 2-7 per 100,000 in India [3]. Based on steroid therapy, nephrotic syndrome is classified as steroid-sensitive nephrotic syndrome (SSNS) and steroid resistance nephrotic syndrome (SRNS) [4].…”

Section: Introductionmentioning

confidence: 99%

Prediction of Functional, Structural and Stability Changes in Pmm2 Gene Associated With Nephrotic Syndrome Using Computational Analysis

Thakor¹,

Mistry²,

Gang

et al. 2021

Int J Pharm Pharm Sci

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Objective: Nephrotic syndrome defines as a disorder with a group of symptoms like proteinuria, hypoalbuminemia, hyperlipidemia, and edema. PMM2 encodes phosphomannosemutase protein enzyme involved in the synthesis of N-glycan. Methods: Different Insilico analysis tools: SIFT, PolyPhen, PROVEAN, SNPandGO, MetaSNP, PhDSNP, MutPred, I-Mutant, STRUM, PROCHECK-Ramachandran, COACH and ConSurf, were used to check the effect of nsSNP on protein structure and function. Results: The genetic polymorphism in the PMM2 gene was retrieved from NCBI ClinVar and UniProtKB. Total 20 SNPs were predicted most significant and responsible for disease-causing and decrease protein stability. Conclusion: This study helps to discover disease-causing deleterious SNPs with different computational tools and gives information about potent SNPs.

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Section: Introductionmentioning

confidence: 99%

Prediction of Functional, Structural and Stability Changes in Pmm2 Gene Associated With Nephrotic Syndrome Using Computational Analysis

Thakor¹,

Mistry²,

Gang

et al. 2021

Int J Pharm Pharm Sci

View full text Add to dashboard Cite

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Idiopathic nephrotic syndrome in Syrian children: clinicopathological spectrum, treatment, and outcomes

Wannous

2024

Pediatr Nephrol

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Background: Idiopathic nephrotic syndrome (INS) is the most common glomerular disorder of childhood.We performed this study to report the histopathological ndings, correlation between the clinical and histopathological features, with the response to steroids and other immunosuppressive drugs and outcome in Syrian children with INS.Methods: A single-center observational cohort study was conducted at Children's University Hospital in Damascus, included all patients aged 1 -14 years, admitted from January 2013 to December 2022 with INS and underwent kidney biopsy.Results: The study included 109 patients, with a male/female ratio of 1.13:1, and a mean age of 6.08 years ± 3.73 SD. The main indication of kidney biopsy was steroid-resistant nephrotic syndrome (SRNS) (57.8%). The main histopathological patterns were minimal change disease (MCD) (45%) and focal segmental glomerulosclerosis (FSGS) (37.6%). FSGS was the most common histopathological pattern in SRNS (44.3%). In SRNS, we used Tacrolimus in 49 patients with response rate of 69.4% and Cyclosporine in 20 patients with response rate of 50%. In steroid-dependent nephrotic syndrome (SDNS), we used MMF in 9 patients with response rate of 89% and Cyclophosphamide in 3 patients with response rate of 66.7%. Rituximab was used in four patients with FSGS, two SRNS patients and two SDNS patients, with response rate of 100% and continuous remission till now (more than two years). Fifteen patients (13.7%) progressed to chronic kidney disease stage V, of them 7 patients had FSGS and 8 patients had focal and global glomerulosclerosis, 14 patients of them were steroid resistant and one patient was steroid dependent, and all of them were resistant to immunosuppressants. The most common outcome was recovery (47%) in MCD and frequent relapses (31.7%) in FSGS.Conclusions: FSGS was the most common histopathological pattern in idiopathic SRNS and has the worst prognosis. Tacrolimus is the most effective second-line therapy in SRNS. Rituximab is an effective and safe treatment that induces prolonged remission mainly in SDNS and frequently relapsing NS, and even in SRNS.

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Idiopathic Nephrotic Syndrome in Syrian Children: Clinicopathological Spectrum, Treatment, and Outcome

wannous

2023

Preprint

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Background: Idiopathic nephrotic syndrome (INS) is the most common glomerular disorder of childhood. We performed this study to report the histopathological findings, correlation between the clinical and histopathological features, with the response to steroids and other immunosuppressive drugs and outcome in Syrian children with INS. Methods: A single-center observational cohort study was conducted at Children’s University Hospital in Damascus, included all patients aged 1 – 14 years, admitted from January 2013 to December 2022 with INS and underwent kidney biopsy. Results: The study included 109 patients, with a male/female ratio of 1.13:1, and a mean age of 6.08 years ± 3.73 SD. The main indication of kidney biopsy was steroid-resistant nephrotic syndrome (SRNS) (57.8%). The main histopathological patterns were minimal change disease (MCD) (45%) and focal segmental glomerulosclerosis (FSGS) (37.6%). FSGS was the most common histopathological pattern in SRNS (44.3%). In SRNS, we used Tacrolimus in 49 patients with response rate of 69.4% and Cyclosporine in 20 patients with response rate of 50%. In steroid-dependent nephrotic syndrome (SDNS), we used MMF in 9 patients with response rate of 89% and Cyclophosphamide in 3 patients with response rate of 66.7%. Rituximab was used in four patients with FSGS, two SRNS patients and two SDNS patients, with response rate of 100% and continuous remission till now (more than two years). Fifteen patients (13.7%) progressed to chronic kidney disease stage V, of them 7 patients had FSGS and 8 patients had focal and global glomerulosclerosis, 14 patients of them were steroid resistant and one patient was steroid dependent, and all of them were resistant to immunosuppressants. The most common outcome was recovery (47%) in MCD and frequent relapses (31.7%) in FSGS. Conclusions: FSGS was the most common histopathological pattern in idiopathic SRNS and has the worst prognosis. Tacrolimus is the most effective second-line therapy in SRNS. Rituximab is an effective and safe treatment that induces prolonged remission mainly in SDNS and frequently relapsing NS, and even in SRNS.

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Clinicopathological study of nephrotic syndrome in Indian children :A tertiary care experience

Cited by 3 publications

References 7 publications

Prediction of Functional, Structural and Stability Changes in Pmm2 Gene Associated With Nephrotic Syndrome Using Computational Analysis

Prediction of Functional, Structural and Stability Changes in Pmm2 Gene Associated With Nephrotic Syndrome Using Computational Analysis

Idiopathic nephrotic syndrome in Syrian children: clinicopathological spectrum, treatment, and outcomes

Idiopathic Nephrotic Syndrome in Syrian Children: Clinicopathological Spectrum, Treatment, and Outcome

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