Background: There is currently little information in literature about the pattern of glomerulonephritides (GN) in adults with nephrotic syndrome in this part of the world, particularly that involving the use of immunofluorescence (IMF) and electron microscopy (EM). A few studies reported are based on light microscopic study alone and hence do not reflect the true pattern of GN underlying nephrotic syndrome. We carried out this study in the Department of Histopathology, Sindh Institute of Urology and Transplantation (SIUT), Karachi, Pakistan to determine, for the first time, the true pattern of GN in adult nephrotic patients from Pakistan. SIUT is a tertiary care center for renal and urologic disease in Pakistan. The Histopathology Laboratory of SIUT is equipped with all the modalities, including EM, required for precise diagnosis of glomerular disease.
Methods: This is a retrospective clinicopathologic study involving retrieval of clinical and pathological data from a review of original renal biopsy reports of adult patients with nephrotic syndrome who presented at the adult nephrology clinic of SIUT from July 1996 till July 2006. Two cores of renal tissue were routinely obtained. One core was fixed in 10% buffered formalin and processed for light microscopy; the other core was divided into two halves, for EM and the IMF study.
Results: A total of 316 adult patients were included. Of these, 201 (63.6%) were male and 115 (36.4%) were female. Mean age was 28.4 +/- 10.51 years with a range of 16-78 years. The spectrum of pathological lesions in the adult nephrotic population was wide and comprised focal segmental glomerulosclerosis (FSGS) (39.87%), followed by membranous GN (MGN) (26.58%), minimal change disease (MCD) (14.82%), mesangiocapillary GN (4.3%), mesangioproliferative GN (4.11%), post-infectious GN (2.84%), IgA nephropathy (2.53%), and other rare lesions.
Conclusions: Results from this study indicate that FSGS is the single most common cause of nephrotic syndrome in adult nephrotic patients, followed by MGN, and MCD. Our data are similar to those reported in recent series from the US. The study defines the pattern of glomerular disease in adult nephrotic patients for the first time in this region, because it is based on light microscopy, serology, IMF, and EM findings.
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http://dx.doi.org/10.1007/s10157-008-0075-0 | DOI Listing |
Pediatr Allergy Immunol Pulmonol
January 2025
Clinical Immunology Unit, Faculty of Medicine and Health Sciences, Department of Paediatrics, Universiti Putra Malaysia, Selangor, Malaysia.
: RAS guanyl-releasing protein 1 (RASGRP1) deficiency is characterized by immune dysregulation and Epstein-Barr virus (EBV)-related lymphoproliferation. Diffuse mesangial sclerosis is one of the infrequent causes of infantile nephrotic syndrome. : Here, we described a 7-year-old girl who was diagnosed with diffuse mesangial sclerosis at 5 months old and subsequently developed chronic bilateral neck swelling at the age of 3 years.
View Article and Find Full Text PDFJ Nephrol
December 2024
Nephrology Unit, Department of Internal Medicine, Faculty of Medicine, Minia University, Taha Hussein Street, Minia, Egypt.
Background: Studies on renal vein thrombosis have been conducted as case reports or case series. The renal outcomes and mortality risk of renal vein thrombosis have not been fully established. We aimed to investigate the clinical characteristics, treatment modalities, and predictors of renal outcomes and mortality in patients with renal vein thrombosis in a large multicenter cohort.
View Article and Find Full Text PDFCEN Case Rep
December 2024
Department of Pediatrics, Songjiang Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, No.748, Zhongshan Middle Road, Songjiang District, Shanghai, 201600, China.
Nephrotic syndrome (NS) and atopic diseases are linked through shared immunological pathways, with allergic triggers often contributing to NS relapses, particularly in immunoglobulin E (IgE)-mediated pathways. Omalizumab, a humanized monoclonal antibody targeting free and cell-bound IgE, is commonly used in treating atopic diseases. We presented a pediatric case with a history of eczema, asthma, and recurrent atopic rhinitis, who first developed NS at age three, responding well to steroid therapy.
View Article and Find Full Text PDFInt Urol Nephrol
December 2024
Department of Emergency, Zhuzhou Hospital Affiliated to Xiangya School of Medicine, Central South University, Zhuzhou, 412007, China.
Objective: Nephrotic syndrome, a debilitating manifestation of kidney disease, often arises from diverse glomerular disorders and is accompanied by notable comorbidities. Despite indications of an immunological etiology, the precise role of immune cells in its pathogenesis remains unclear. This study aimed to elucidate the causal relationships between circulating immune cell phenotypes and nephrotic syndrome using a rigorous bidirectional Mendelian randomization approach.
View Article and Find Full Text PDFCan J Kidney Health Dis
December 2024
Division of Nephrology, BC Children's Hospital, Vancouver, Canada.
Background: In 2013, the British Columbia (BC) Childhood Nephrotic Syndrome Clinical Pathway (CNSCP) was developed to standardize the care of children with nephrotic syndrome (NS). In BC, children access nephrology care at BC Children's Hospital (BCCH) and multiple regional clinics.
Objective: The primary objective was to compare induction therapy and clinical outcomes between BCCH and regional clinics since implementation of the CNSCP.
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