Focal Segmental Glomerulosclerosis in Paediatric Population of South Punjab Pakistan: A Tertiary Care Hospital Experience

Abstract

Objectives: To find out frequency, clinicopathological features, response of treatment and outcome among children with primary focal segmental glomerulosclerosis (FSGS).
 Methods: This retrospective, non-interventional medical charts review study was conducted from a period of January 2011 to January 2020 at Pediatric Department of Nishtar Medical University Hospital, Multan, Pakistan. During the nine years study period, children of both genders, aged less than 16 years, with renal biopsies proven FSGS were included. Patient’s demographic along with clinical and laboratory data, urine dipstick for proteinuria, renal functions, 24 hours urinary protein and ultrasonography findings of kidneys, ureters and bladder (KUB) were noted from case records. Response rates of various treatment options and their outcome like remission, partial remission, no remission with stable kidney disease & no remission with progression of kidney disease were noted.
 Results: During the study duration, out of 307 renal biopsies performed in glomerulonephritis cases, 124 (40.4%) had primary FSGS. In 124 primary FSGS cases, mean age was 8.83±3.05 years while most of the children, 70 (56.5%) were above 10 years of age. Majority of the cases, 64 (51.6%) were male. Mean follow up duration was noted to be 28.35+18.47 months. Most of the cases, 68 (54.8%) were found to have complete remission, 22 (17.7%) partial remission while 11 (8.9%) progressed to ESKD.
 Conclusions: Among children, frequency of primary FSGS was high at our setting. Most of the cases achieved sustained remission rates with the help of immunosuppressive drugs. Cyclosporine and tacrolimus were found to be the most effective drugs.
 doi: https://doi.org/10.12669/pjms.37.2.3535
 How to cite this:Safdar RS, Mehar MF, Khan AA, Buzdar N. Focal Segmental Glomerulosclerosis in Paediatric Population of South Punjab Pakistan: A Tertiary Care Hospital Experience. Pak J Med Sci. 2021;37(2):---------. doi: https://doi.org/10.12669/pjms.37.2.3535
 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Overview of Nephrotic Syndrome in Arab Children: Systematic review

“…Nephrotic syndrome was the most common indication in some, while SLE, nephritic syndrome, and other indications were reported in others. This variability concurs with previously published data internationally [28,29]. Primary glomerular diseases, particularly the primary ones, were found to be the most common pediatric renal diseases, accounting for 75.6% of all renal diseases.…”

“…Studies also reported differences in the prevalence of FSGS, ranging from 15% to 59% [26,27]. FSGS was consistently reported as the primary underlying histopathology in children with SRNS in various studies [28]. Furthermore, the clinical indications for renal biopsies in different studies varied.…”

IgM and C3 Deposition in Primary Focal Segmental Glomerulosclerosis (FSGS): A Clinical and Histopathological Spectrum

“…Focal segmental glomerulosclerosis is now one of the most common causes of nephrotic syndrome in pediatric and adult age groups [ 4 , 15 - 16 ] within the Pakistani population as shown by several studies. The prognosis is variable, with some patients going into remission while others progress to end-stage renal disease.…”

“…FSGS is not a distinct clinical disease, rather it encompasses a large group of disorders that may result in this pattern of glomerular injury. The incidence of FSGS is on the rise and it is now one of the most common causes of steroid resistant-nephrotic syndrome in pediatric [ 3 - 4 ] and adult age groups [ 5 ], both worldwide and in the Pakistani population. It also has frequent progression to end-stage renal disease, with the literature citing [ 6 ] the African population as being particularly susceptible.…”