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Published on: August 19, 2020
A clinicopathologic study of primary focal segmental glomerulosclerosis in children
Ali Lanewala1, Muhammed Mubarak, Javed Iqbal Kazi
1Department of Pediatric Nephrology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan. alilanewala@yahoo.com
Insights
Pediatric primary focal segmental glomerulosclerosis (FSGS) treatment in Pakistan is understudied. This review shows immunosuppressive therapies offer good remission rates and prognosis for children with FSGS.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Immunosuppressive Therapy
Background:
- Limited data exists on primary focal segmental glomerulosclerosis (FSGS) treatment and prognosis in Pakistani children.
- Primary FSGS is a significant cause of kidney disease in pediatric populations.
Purpose of the Study:
- To review the treatment and outcomes of primary FSGS in children at a major Pakistani center.
- To analyze demographic, clinical, pathological, and therapeutic responses in pediatric FSGS cases.
Main Methods:
- Retrospective review of 94 children (≤16 years) diagnosed with primary FSGS from 1995-2008.
- Analysis of clinical records, renal biopsy reports (light, immunofluorescence, electron microscopy), and treatment responses.
- Evaluation of therapeutic regimens including cyclophosphamide, cyclosporine, and tacrolimus.
Main Results:
- 63.8% had steroid-dependent nephrotic syndrome (SDNS); 35% had steroid-resistant nephrotic syndrome (SRNS).
- Cyclophosphamide achieved complete remission (CR) in 69.4% of SDNS cases. Cyclosporine showed CR in 52.6% of SRNS/SDNS cases. Tacrolimus yielded CR in 28.5% of cases.
- 12.7% of children developed renal insufficiency; overall prognosis was fairly good with sustained remission.
Conclusions:
- Second- and third-line immunosuppressive therapies can achieve high remission rates in pediatric primary FSGS.
- Effective management strategies exist, offering a fairly good prognosis for affected children in Pakistan.
- Further research is needed to optimize treatment protocols and long-term outcomes for pediatric FSGS.
Abstract:
There is very little information in the literature on the treatment and prognosis of primary focal segmental glomerulosclerosis (FSGS) among children in Pakistan. This is a review of 94 children (≤16 years) with a diagnosis of primary FSGS who presented to the Sindh Institute of Urology and Transplantation between 1995 and 2008. The clinical records and original renal biopsy reports were reviewed to determine demographic, clinical, laboratory and pathologic features. Renal biopsies were studied by light microscopy, immunofluoroscence and electron microscopy. Thera-peutic regimens and response to therapy were analyzed. Majority of the children (60, 63.8%) had steroid-dependant nephrotic syndrome (SDNS) and 33 (35%) had steroid-resistant nephrotic syndrome (SRNS). Cyclosphosphamide was used in SDNS, and this produced complete remission (CR) in 25/36 (69.4%), partial response (PR) in 4/36 (11%) and no response in 7/36 (19.4%) cases. Cyclosporine was used in SRNS and some SDNS children, and showed a CR in 30 (52.6%), PR in 20 (35%) and no response in seven (12.2%) cases. Tacrolimus was used in seven (7.44%) children. CR was obtained in two (28.5%) and PR in five (71.4%) cases. Renal insufficiency developed in 12 (12.7%) children. Results from this study show that majority of the children with primary FSGS at our center could achieve high rates of sustained remission with second- and third-line immunosuppressive therapies with fairly good prognosis.
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