Journal of Pakistan Medical Association, May 14, 2022
Objective: To screen asymptomatic siblings of steroid-resistant nephrotic syndrome patients for p... more Objective: To screen asymptomatic siblings of steroid-resistant nephrotic syndrome patients for proteinuria using the urinary dipstick method to determine the involvement of siblings in the familial and likely genetic cause of the steroid-resistant nephrotic syndrome. Methods: This cross-sectional study was performed at the outpatient department of Sindh Institute of Urology and Transplantation (SIUT) from May to July 2021. Results: Out of 104 patients with steroid-resistant nephrotic syndrome, siblings of 66 patients were enrolled. Mean age of primary patients with steroid resistant nephrotic syndrome was 8.7±4.3 years. Most common histopathological diagnosis was focal segmental glomerulosclerosis in 25 (37.9%) children followed by minimal change disease in 17(25.8%) of them. The majority, 48 (72.7%) patients were on immunosuppressive treatment, while 4 (6.1%) had progressed to chronic kidney disease (CKD). A total of 178 siblings were recruited in the study. There were 99(55.6%) boys and 79(44.4%) girls. Their mean age was 10.67±6.2 years. Consanguinity was high in our study population i.e. 56(84%) families. Positive proteinuria on dipstick was detected in only 5(7.5%) enrolled SRNS families. One family refused further testing. Two of the five affected siblings had nephrotic range proteinuria. Renal biopsy of one of them showed membranous nephropathy while the second showed mesangiocapillary glomerulonephritis. Both had normal renal functions. Conclusion: The frequency of proteinuria in asymptomatic siblings of children with steroid-resistant syndrome is low in our population despite a high prevalence of consanguineous marriages. Hence, familial involvement of nephrotic syndrome is low and further genetic testing for monogenic causes is required in steroid-resistant nephrotic syndrome cases. Keywords: steroid-resistant, nephrotic, syndrome, proteinuria.
Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV... more Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV collagen in the GBM, may present with rapidly progressive glomerulonephritis and/or diffuse alveolar hemorrhage. While rare in childhood, pediatric patients usually have both renal and pulmonary involvement. We report a child who presented with crescentic glomerulonephritis and severe renal-limited anti-GBM disease. The diagnosis was aided by findings of characteristic linear IgG deposits on GBM on immunofluorescence on kidney biopsy and high titers of anti-GBM antibody. As patients with renal-limited disease and severe renal failure usually do not recover renal function despite aggressive immunosuppression and plasmapheresis, intensive therapy was withdrawn while continuing maintenance hemodialysis. However, the patient later developed severe alveolar hemorrhage that responded completely to plasmapheresis and intensification of immunosuppression. Sustained serological and pulmonary response was documented, even though renal dysfunction persisted. Our case emphasizes the utility of kidney biopsy and serology in enabling rare diagnosis in patients with rapidly progressive glomerulonephritis and the role of aggressive plasmapheresis in enabling recovery from delayed catastrophic alveolar hemorrhage.
JPMA. The Journal of the Pakistan Medical Association, 2018
OBJECTIVE To study the frequency, clinicopathological features and short-term outcome of mesangio... more OBJECTIVE To study the frequency, clinicopathological features and short-term outcome of mesangiocapillary glomerulonephritis (MCGN) in children at a tertiary care kidney center in Pakistan. METHODS A descriptive, observational study was conducted at the Paediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2011 till December 2015. A review of all paediatric (<18 years) renal biopsies during the study period was performed and cases of MCGN were enrolled. The clinical presentation, laboratory findings, histology and outcome were analyzed. RESULTS During the study period, 890 paediatric renal biopsies were performed. Of these, 63(7%) were MCGN. Among these, 34(54%) were males and 29 (46%) females. Mean age was 9.9 ± 3.2years. Thirty four (54%) presented with nephrotic syndrome (NS), and29 (46%) with rapidly progressive glomerulonephritis (RPGN).Mean duration of follow-up was 1.66 ± 1.34 years. Outcome of patients with NS with renal ...
Journal of Pakistan Medical Association, May 14, 2022
Objective: To screen asymptomatic siblings of steroid-resistant nephrotic syndrome patients for p... more Objective: To screen asymptomatic siblings of steroid-resistant nephrotic syndrome patients for proteinuria using the urinary dipstick method to determine the involvement of siblings in the familial and likely genetic cause of the steroid-resistant nephrotic syndrome. Methods: This cross-sectional study was performed at the outpatient department of Sindh Institute of Urology and Transplantation (SIUT) from May to July 2021. Results: Out of 104 patients with steroid-resistant nephrotic syndrome, siblings of 66 patients were enrolled. Mean age of primary patients with steroid resistant nephrotic syndrome was 8.7±4.3 years. Most common histopathological diagnosis was focal segmental glomerulosclerosis in 25 (37.9%) children followed by minimal change disease in 17(25.8%) of them. The majority, 48 (72.7%) patients were on immunosuppressive treatment, while 4 (6.1%) had progressed to chronic kidney disease (CKD). A total of 178 siblings were recruited in the study. There were 99(55.6%) boys and 79(44.4%) girls. Their mean age was 10.67±6.2 years. Consanguinity was high in our study population i.e. 56(84%) families. Positive proteinuria on dipstick was detected in only 5(7.5%) enrolled SRNS families. One family refused further testing. Two of the five affected siblings had nephrotic range proteinuria. Renal biopsy of one of them showed membranous nephropathy while the second showed mesangiocapillary glomerulonephritis. Both had normal renal functions. Conclusion: The frequency of proteinuria in asymptomatic siblings of children with steroid-resistant syndrome is low in our population despite a high prevalence of consanguineous marriages. Hence, familial involvement of nephrotic syndrome is low and further genetic testing for monogenic causes is required in steroid-resistant nephrotic syndrome cases. Keywords: steroid-resistant, nephrotic, syndrome, proteinuria.
Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV... more Antiglomerular basement membrane (GBM) disease, caused by IgG antibodies directed against type IV collagen in the GBM, may present with rapidly progressive glomerulonephritis and/or diffuse alveolar hemorrhage. While rare in childhood, pediatric patients usually have both renal and pulmonary involvement. We report a child who presented with crescentic glomerulonephritis and severe renal-limited anti-GBM disease. The diagnosis was aided by findings of characteristic linear IgG deposits on GBM on immunofluorescence on kidney biopsy and high titers of anti-GBM antibody. As patients with renal-limited disease and severe renal failure usually do not recover renal function despite aggressive immunosuppression and plasmapheresis, intensive therapy was withdrawn while continuing maintenance hemodialysis. However, the patient later developed severe alveolar hemorrhage that responded completely to plasmapheresis and intensification of immunosuppression. Sustained serological and pulmonary response was documented, even though renal dysfunction persisted. Our case emphasizes the utility of kidney biopsy and serology in enabling rare diagnosis in patients with rapidly progressive glomerulonephritis and the role of aggressive plasmapheresis in enabling recovery from delayed catastrophic alveolar hemorrhage.
JPMA. The Journal of the Pakistan Medical Association, 2018
OBJECTIVE To study the frequency, clinicopathological features and short-term outcome of mesangio... more OBJECTIVE To study the frequency, clinicopathological features and short-term outcome of mesangiocapillary glomerulonephritis (MCGN) in children at a tertiary care kidney center in Pakistan. METHODS A descriptive, observational study was conducted at the Paediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2011 till December 2015. A review of all paediatric (<18 years) renal biopsies during the study period was performed and cases of MCGN were enrolled. The clinical presentation, laboratory findings, histology and outcome were analyzed. RESULTS During the study period, 890 paediatric renal biopsies were performed. Of these, 63(7%) were MCGN. Among these, 34(54%) were males and 29 (46%) females. Mean age was 9.9 ± 3.2years. Thirty four (54%) presented with nephrotic syndrome (NS), and29 (46%) with rapidly progressive glomerulonephritis (RPGN).Mean duration of follow-up was 1.66 ± 1.34 years. Outcome of patients with NS with renal ...
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Papers by Sabeeta Khatri