Clinicopathological features and outcome of secondary steroid resistant nephrotic syndrome: A retrospective analysis.

Objective: To determine the clinico-pathological features and long-term outcome of secondary steroid-resistant nephrotic syndrome treated with steroids and calcineurin inhibitors. METHODS: The retrospective cohort study was conducted at the Sindh Institute of Urology and Transplant, Karachi, in June and July 2023, and comprised data from January 1, 2008, to December 31, 2020, of children aged 1-18 years who developed steroid resistance after initial sensitivity to steroids with at least 1-year of follow-up. Demographics as well as time taken to secondary steroid response were documented. Renal biopsy of all patients with secondary steroid resistance had been performed. Eventual outcomes after treatment with calcineurin inhibitors based on the degree of proteinuria and serum albumin levels were used to categorise complete remission, partial remission and no response. Kidney function, as determined by estimated glomerular filtration rate, was recorded. Data was analysed using SPSS 22. RESULTS: Of the 1,000 patients who underwent renal biopsy for steroid resistance, 48(4.8%) had idiopathic steroid-resistant nephrotic syndrome; 32(66.7%) males, 16(33.3%) females and median age of 5 years (interquartile range: 4-7.3 years). Median age at diagnosis of nephrotic syndrome was 5 years (interquartile range: 3.6-7.3 years). The median time from nephrotic syndrome to secondary steroid-resistant nephrotic syndrome was 23 months (interquartile range: 8.75-44.5 months). Biopsy results at diagnosis showed that 27(56.3%) had minimal change disease. The mean follow-up time was 6.1±3.2 years. Of the 43(89.5%) patients who received cyclosporin for 1 year, 29(67%) obtained complete remission, 5(12%) attained partial remission and no response was seen in 9(21%) patients. Conclusion: Majority of the children had minimal change disease at the time of diagnosis of secondary steroid-resistant nephrotic syndrome. The long-term response with calcineurin inhibitors was favourable at 1 year.

Steroid induced ocular complications in idiopathic nephrotic syndrome: a cross sectional single center study.

Objective: To screen children receiving steroids to identify ocular complications and their prevalence. METHODS: The cross-sectional study was conducted at the Paediatric Nephrology and Ophthalmology departments of the Sindh Institute of Urology and Transplantation, Karachi, from May to October 2022, and comprised patients who received at least 1500mg cumulative steroid dose for a minimum of 3 months. They were screened for steroidsensitive or steroid-resistant nephrotic syndrome. Ocular examinations, including visual acuity, intraocular pressure, slit-lamp biomicroscopy, lens examination and fundus evaluation, were performed. Data was analysed using SPSS 22. RESULTS: Of the 124 subjects with mean age 8.15±2.03 years (range: 6-12 years), 64(51.6%) were boys. Steroidsensitive nephrotic syndrome was present in 97(78%) cases. The mean cumulative steroid dose was 3999.31±1564.22mg. Overall, 36(29%) children developed ocular complications. Blood pressure, number of relapses and the duration of treatment were significantly associated (p<0.05). Conclusion: Refractive errors were the most frequent side effects/complication seen among children with nephrotic syndrome who received prolonged corticosteroids.

Calculus anuria: a urological emergency with an excellent outcome.

Calculus anuria is a catastrophic condition with dire consequences if not treated promptly. The purpose of this study was to identify factors which influence the short-term outcome of patients with calculus anuria. A retrospective analysis was conducted from January 2016 to December 2021, in children up to the age of 18 years, who presented with calculus anuria and required emergency decompression at Sindh Institute of Urology and Transplantation, Pakistan. One hundred and twenty-five children were included. Majority were born to consanguineous parents and a few of them had positive family history of stone disease. Severe illness was found in 25 (20%) patients and among them 8 (32%) required hemodialysis. Decompression by double J stenting is the preferred intervention in our institute and was done in 106 (85%) children, followed by percutaneous nephrostomy tube in 10 (8%) successfully. A small number of patients, 9 (7%) required both procedures to relieve their obstruction. A significant number of patients, about 115 (92%), attained normal renal functions after intervention. No pertinent factors were identified, relating to incomplete renal recovery in nine (7%) of the patients who unfortunately progressed to chronic kidney disease.

Acquired Cystic Kidney Disease: A Hidden Complication in Children on Chronic Hemodialysis.

Objective To determine the frequency of acquired cystic kidney disease (ACKD) in children on chronic hemodialysis. Material and methods In this single-center cross-sectional study, 150 children were included who were on chronic hemodialysis for six months. Ultrasound was done to see the renal cysts. Cystic changes that could not fulfill the criteria for ACKD were also noted and analyzed. Results The mean age was 14.5 ± 3.5 years, of these 63 (42%) were males. Acquired cysts were detected in 53 (35%) of the patient and 18 patients (12%) had solitary cysts. The distribution of these entities was similar across all age groups. The underlying etiologies in the descending order were unknown 64 (43%), stone disease 31 (21%), each of the congenital anomalies of the kidney and urinary tract, and glomerulonephritis 23 (15%), and others nine (6%). A higher frequency of ACKD was detected in the children on renal replacement therapy for more than two years (33 out of 53 children, 63% with a p-value of 0.004). Conclusion The ACKD was found in one-third of our hemodialysis children and its frequency increases with the duration of hemodialysis. This percentage may not reflect the true prevalence as there is a lack of consensus on the definition of ACKD. Periodic assessment of chronic kidney disease patients for the development of ACKD especially on chronic hemodialysis is required to reduce the morbidity.

Knowledge, attitudes and practices towards deceased organ donation among parents of children with End Stage Kidney Disease.

OBJECTIVE: To assess the knowledge, attitudes, and practices (KAP) towards deceased organ donation (DOD) of the parents/ guardians of children with end stage kidney disease (ESKD). METHODS: This cross sectional survey was conducted between April and December 2020. A structured questionnaire was filled to assess the sociodemographic information, knowledge, attitude, and practice about DOD. RESULTS: A total of 130 participants with a mean age of 37±7 years were included in the study. Among all, 66 (50.8%) claimed that they had prior knowledge about DOD. However, on further questioning, no one knew who can be the deceased organ donor. Their responses about registration place and permission from religion were positive in 24(36.4%) and 31(47.0%) cases respectively. Regarding attitude, positive responses to willingness to get registered and discussing DOD in social circle were given by 37(56.1%) and 21 (31.8%) participants respectively. Only one participant was registered as donor. The remaining 64(49.2%) participants who had no prior knowledge were given relevant information and were interviewed after one week. Only 24(37.5%) showed willingness to get registered as donors and 06 (9.4%) participants discussed the topic of DOD in their social circle. CONCLUSIONS: The results showed that the knowledge, attitudes, and practices of people who are most desperate for transplantation of their children were poor and did not change significantly even after providing them relevant information.

Factors Predicting Short Term Outcome in Children With Idiopathic Nephrotic Syndrome: A Prospective Cohort Study.

Objective The objective of the article is to determine the risk factors associated with relapses in children with idiopathic nephrotic syndrome (INS). Material and methods Fifty-seven children with the first episode of INS were included and followed up prospectively for a minimum period of one year to identify the risk factors related to relapses. The study subjects were divided into early (less than eight days) and late (equal to or more than eight days) responder groups and were compared in terms of the number of days to achieve complete remission, time to first relapse, and the pattern of relapse at the last follow-up. Results Of the 57 children, 32 (56%) were male and 25 (44%) female. The mean age of the study cohort was 5.3 ± 3 years. Sixteen (55%) children with ages ranging from one to four years had a higher propensity to develop relapse, although the p-value (p=0.11) was not significant. Gender analysis did not reveal any significant correlation (p=0.32); however, a higher proportion of males (n=17; 63%) responded within eight days of starting steroids than female counterparts (n=10; 37%). Microscopic hematuria at the disease onset was seen in 12 (21%) children, and out of them, five (41.6%) remained in complete remission. The mean time to achieve complete remission was 8.1 ± 3.5 days, while the early responder group had delayed time to first relapse as compared to the late responders (3.1 ± 5.2 vs. 1.6± 3.8; p=0.21). Among all the study participants, a significant number of children (n=20; 51%) were in complete remission at their last follow-up visit. Baseline serum albumin, cholesterol, body mass index (BMI), and serum creatinine had no significant difference. Conclusion The delayed response to steroids and younger age at presentation can predict the time to first relapse and number of relapses in children with INS, respectively.

Short-term outcome of clinical and histopathologic variants of mesangiocapillary glomerulonephritis in children: A retrospective analysis from a tertiary care center.

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 failure (RF)was complete remission (CR) in 1(7.7%), persistent proteinuria with normal renal functions in 1(7.7%),chronic kidney disease (CKD) in 3 (23%), end-stage renal disease (ESRD) in 4 (30.8%), while 4 (30.8%) children died, while in children with NS and normal renal functions, CR was obtained in 3(14.2%), partial remission (PR) in 10(47.6%),CKD in 4(19%), and ESRD in 3 (14.3%).Outcome of cases presenting as RPGN was CR in 13 (44.8%), CKD in 2(6.9%) and ESRD in 7(24.1%) cases. Four children (13.8%) were lost to follow-up, while 3(10.3%) died. CONCLUSIONS: Children with MCGN presenting clinically with NS with impaired renal functions have worst outcome.

Histopathological Spectrum and Short-Term Outcome of Treatment with Cyclophosphamide in Relapsing Steroid-Sensitive Nephrotic Syndrome.

OBJECTIVE: To determine the short-term outcome of cyclophosphamide (CPO) course in children with relapsing steroid sensitive nephrotic syndrome (SSNS) with different histopathological lesions. STUDY DESIGN: Descriptive, observational study. PLACE AND DURATION OF STUDY: Pediatric Nephrology Department, Sindh Institute of Urology and Transplantation, Karachi, from January 2012 to December 2014. METHODOLOGY: All children with relapsing steroid-sensitive nephrotic syndrome, who underwent renal biopsy and received cyclophosphamide therapy, were included and followed up for 2 years. Histopathological features in renal biopsy, duration of treatment, duration of complete remission and complication frequency was noted. RESULTS: Of the total 74 patients, 47 (63.5%) were males and 27 (36.5%) females. Median age with Interquartile range (IQR) at presentation was 5 years (4-7 years). Minimal change disease (MCD) was the most common histopathological diagnosis (n=54, 73%) followed by focal segmental glomerulosclerosis (FSGS) (n=13, 17.5%), mesangioproliferative glomerulonephritis(MesPGN) (n=6, 8.1%), IgA nephropathy (n=1, 1.4%). The median number of glomeruli included in each biopsy sample was 15. The median duration of treatment with CPO was 11 weeks (9 to 13 weeks), whereas the median duration of complete remission post-therapy was 13 months (7-23 months). A median timeframe of 17 months (13-24.2 months) lapsed between establishing the diagnosis of NS and initiating CPO treatment. Leucopenia was noted in six (8.1%) patients. CONCLUSION: The short-term outcome of relapsing SSNS can be improved with CPO and steroids, with minimum short-term side effects.