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Background Dyslipidemia is a potentially modifiable risk factor in patients with chronic kidney disease (CKD). Information on the safety and efficacy of statins in pediatric CKD is limited. Methods Patients with CKD stage 2-5 and aged 5-18 years with low-density lipoprotein cholesterol (LDL-C) > 130 mg/dL and/or non-high-density lipoprotein cholesterol (non-HDL-C) > 145 mg/dL were enrolled from September 2019 to February 2021. All patients were administered atorvastatin 10 mg/day, which was escalated to 20 mg/day if LDL-C remained > 100 mg/dL and/or non-HDL-C > 120 mg/dL at 12 weeks. Proportion of patients achieving target lipid levels (LDL-C [less than or equal to] 100 mg/dL and non-HDL-C [less than or equal to] 120 mg/dL) and adverse events were assessed at 24 weeks. Results Of 31 patients enrolled, target lipid levels were achieved in 45.2% (95% CI 27.8-63.7%) at 24 weeks; 22 patients required dose escalation to 20 mg at 12 weeks. There was no difference in median lipid level reduction with 10 (n = 9) versus 20 mg/day (n = 22, P = 0.3). Higher baseline LDL-C (OR 1.06, 95% CI 1.00-1.11) and older age (OR 36.5, 95% CI 2.57-519.14) were independent predictors of failure to achieve target lipid levels with 10 mg/day atorvastatin. None had persistent rise in AST/ALT > 3 times upper normal limit (UNL) or CPK > 10 times UNL. No differences were noted in adverse events due to atorvastatin 10 or 20 mg/day. Conclusion Atorvastatin (10-20 mg/day) administered for 24 weeks was safe and effectively reduced LDL-C and non-HDL-C in children with CKD stages 2-5. Patients with higher baseline LDL-C required higher doses to achieve the target. Graphical abstract, Author(s): Punitha Lakxmi Ramesh [sup.1] , Priyanka Khandelwal [sup.1] , R. Lakshmy [sup.2] , Aditi Sinha [sup.1] , Arvind Bagga [sup.1] , Pankaj Hari [sup.1] Author Affiliations: (1) grid.413618.9, 0000 [...]

Background Coexisting genetic variants in patients with anti-factor H (FH)-associated atypical hemolytic uremic syndrome (aHUS) have implications for therapy. We estimated the prevalence of complement genetic variants in children with anti-FH aHUS from a prospective nationwide cohort and determined if significant genetic variants impact long-term kidney outcomes. Methods Of 436 patients in the database, 77 consecutive patients, 21 with a relapse and 9 with kidney failure and/or death were included. Targeted sequencing, using a 27-gene panel including CFH, CFI, CFB, C3, CD46, PLG, DGKE, and THBD and multiplex ligation-dependent probe amplification of CFH-CFHR region, was performed. The adverse outcome was eGFR < 30 ml/min/1.73 m.sup.2 or death. Results Patients had high anti-FH titers 5670 (2177-13,545) AU/ml, relapsing course (42.1%), and adverse outcomes (19.6%). Variants, chiefly of unknown significance, were found in 7 (6.5%; 95% CI 3.1-13.2%); a pathogenic variant was found in one patient. Homozygous deletion of CFHR1 was present in 91.6% compared to 9.8% in 184 healthy controls. Plasma exchanges and immunosuppression showed a trend of improving outcomes, independent of genetic defects (HR 0.32; P = 0.070). Meta-analysis of 18 studies (384 patients) showed that the pooled prevalence of pathogenic and likely pathogenic variants was 3% (95% CI 0-8%). Of 37 total variants in the meta-analysis, 7 (18.9%) each were pathogenic and likely pathogenic; others were variants of unknown significance. Conclusions Significant variants in complement regulatory genes are rare in patients with anti-FH-associated aHUS. Irrespective of genetic defects, plasma exchanges and immunosuppression showed a statistical trend to improved outcomes. Graphical abstract, Author(s): Priyanka Khandelwal [sup.1] , Aditi Joshi [sup.2] , Aradhana Mathur [sup.2] , Mamta Puraswani [sup.1] , Bahadur Singh Gurjar [sup.1] , Aditi Sinha [sup.1] , Pankaj Hari [sup.1] , [...]

Background Children with chronic kidney disease (CKD) generally have worse educational and psychosocial outcomes compared with their healthy peers. This can impair their ability to manage their treatment, which in turn can have long-term health consequences through to adulthood. We attempted to capture the experiences of children with CKD and to describe the perspectives of their parents and caregivers on access to educational and psychosocial support. Methods Children with CKD (n = 34) and their caregivers (n = 62) were sampled via focus groups from pediatric hospitals in Australia, Canada, and the USA. Sixteen focus groups were convened and the transcripts were analyzed thematically. Results We identified four themes: disruption to self-esteem and identity (emotional turmoil of adolescence, wrestling with the sick self, powerlessness to alleviate child's suffering, balancing normality and protection); disadvantaged by lack of empathy and acceptance (alienated by ignorance, bearing the burden alone); a hidden and inaccessible support system (excluded from formal psychological support, falling behind due to being denied special considerations); and building resilience (finding partners in the journey, moving towards acceptance of the illness, re-establishing childhood). Conclusions Children with CKD and their caregivers encountered many barriers in accessing psychosocial and educational support and felt extremely disempowered and isolated as a consequence. Improved availability and access to psychosocial and educational interventions are needed to improve the wellbeing and educational advancement of children with CKD. Graphical Abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Yifan Zhang [sup.1] , Talia Gutman [sup.1] [sup.2] , Allison Tong [sup.1] [sup.2] , Jonathan C. Craig [sup.3] , Aditi Sinha [sup.4] , Allison Dart [sup.5] , Allison A. [...]

Author(s): Aditi Sinha [sup.1] , Arvind Bagga [sup.1] Author Affiliations: (1) grid.413618.9, 0000 0004 1767 6103, Division of Nephrology and ICMR Center for Advanced Research in Nephrology, Department of Pediatrics, [...]

Background Rituximab and tacrolimus are therapies reserved for patients with frequently relapsing or steroid-dependent nephrotic syndrome who have failed conventional steroid-sparing agents. Given their toxicities, demonstrating non-inferiority of rituximab to tacrolimus may enable choice between these medications. Methods This investigator-initiated, single-center, open-label, pilot randomized controlled trial examined the non-inferiority of two doses of intravenous (IV) rituximab given one-week apart to oral therapy with tacrolimus (1:1 allocation), in maintaining sustained remission over 12 months follow-up, in patients with difficult-to-treat steroid-sensitive nephrotic syndrome, defined as frequently relapsing or steroid-dependent disease that had failed [greater than or equal to] 2 steroid-sparing strategies. Secondary outcomes included frequency of relapses, proportion with frequent relapses, time to relapse and frequent relapses, and adverse events (CTRI/2018/11/016342). Results Baseline characteristics were comparable for 41 patients randomized to receive rituximab (n = 21) or tacrolimus (n = 20). While 55% of patients in each limb were in sustained remission at 1 year, non-inferiority of rituximab to tacrolimus was not demonstrated (mean difference 0%; 95% CI - 30.8%, 30.8%; non-inferiority limit - 20%; P = 0.50). Frequent relapses were more common in patients administered rituximab compared to tacrolimus (risk difference 30%, 95% CI 7.0, 53.0, P = 0.023). Both groups showed similar reductions in relapse rates and prednisolone use. Common adverse events were infusion-related with rituximab and gastrointestinal symptoms with tacrolimus. Conclusions Therapy with rituximab was not shown to be non-inferior to 12-months treatment with tacrolimus in maintaining remission in patients with difficult-to-treat steroid-sensitive nephrotic syndrome. Frequent relapses were more common with rituximab. While effective, both agents require close monitoring for adverse events. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information., Author(s): Georgie Mathew [sup.1] , Aditi Sinha [sup.1] , Aijaz Ahmed [sup.1] , Neetu Grewal [sup.1] , Priyanka Khandelwal [sup.1] , Pankaj Hari [sup.1] , Arvind Bagga [sup.1] Author Affiliations: [...]

Background The pathogenesis of autoantibody generation in anti-factor H (FH) antibody associated atypical hemolytic uremic syndrome (aHUS) is unknown and is perhaps triggered by an infectious or environmental agent. We observed an unusual increase of patients with anti-FH antibody associated aHUS coinciding with the second pandemic wave in New Delhi and suspected that SARS-CoV-2 infection might be a potential trigger. Methods We screened for SARS-CoV-2 infection using reverse transcriptase polymerase chain reaction (RT-PCR) and serology in 13 consecutive patients with anti-FH antibody associated aHUS during the past year in New Delhi. Results We report 5 patients, 4-13 years old, who presented with a febrile illness without respiratory symptoms during the second pandemic wave. Of these, 3 patients presented with a relapse 25-85 months following the initial episode of aHUS. SARS-CoV-2 was detected by RT-PCR in 1 patient and by serology in 4 patients (median titer 47.1 cut-off index). Patients had high titers of anti-FH antibodies (median 2,300 AU/ml). Genetic studies, done in 3 of the 5 patients, showed homozygous CFHR1 deletion without other significant genetic abnormalities. Specific management comprised plasma exchanges and oral prednisolone, combined with either cyclophosphamide or mycophenolate mofetil. At median follow-up of 3.3 months, the estimated glomerular filtration rate in 4 patients ranged from 62 to 110 ml/min/1.73 m.sup.2; one patient was dialysis-dependent. Conclusion Increased vigilance is required during the pandemic, especially in patients with anti-FH associated aHUS, who might relapse despite quiescent disease for a prolonged period. Graphical abstract, Author(s): Priyanka Khandelwal [sup.1] , Sudarsan Krishnasamy [sup.1] , Srinivasavaradan Govindarajan [sup.1] , Manish Kumar [sup.2] , Binata Marik [sup.1] , Aditi Sinha [sup.1] , Pankaj Hari [sup.1] , Arvind [...]

Academic stress is the most common mental state that medical students experience during their training period. To assess academic stress, to find out its determinants, to assess other sources of stress and to explore the various coping styles against academic stress adopted by students. Methods: It was a cross sectional study done among medical students from first to fourth year. Standard self-administered questionnaires were used to assess academic stress and coping behaviour. Mean age of the 400 participants was 20.3 ± 1.5 years. 166(41.5%) of them were males. The academic stress was found to be of mild, moderate and severe level among 68(17%), 309(77.3%) and 23(5.7%) participants respectively. Overall coping with stress was found to be poor, average and good among 15(3.8%), 380(95%) and 5(1.2%) participants respectively. Passive emotional (p = 0.054) and passive problem (p = 0.001) coping behaviours were significantly better among males. Active problem coping behaviour (p = 0.007) was significantly better among females. Active emotional coping behaviour did not vary significantly between genders (p = 0.54). Majority of the students preferred sharing their personal problems with parents 211(52.7%) followed by friends 202(50.5%). Binary logistic regression analysis found worrying about future (p = 0.023) and poor self-esteem (p = 0.026) to be independently associated with academic stress. Academic stress although a common finding among students, the coping style to deal with it, was good only in a few. The coping behaviours were not satisfactory particularly among male participants. This along with other determinants of academic stress identified in this study need to be addressed during counselling sessions., Author(s): Nitin Joseph [sup.1] [sup.2] , Aneesha Nallapati [sup.2] [sup.3] , Mitchelle Xavier Machado [sup.2] [sup.3] , Varsha Nair [sup.2] [sup.3] , Shreya Matele [sup.2] [sup.3] , Navya Muthusamy [sup.2] [...]

Background Distal renal tubular acidosis (RTA) is typically caused by defects in ATP6V0A4, ATP6V1B1, and SLC4A1, accounting for 60-80% of patients. Genes recently implicated include FOXI1, ATP6V1C2, and WDR72, of which WDR72 is associated with dental enamel defects. Methods We describe 4 patients, from three unrelated consanguineous families, with RTA and amelogenesis imperfecta. Distal tubular acidification was evaluated by furosemide-fludrocortisone test, urine-to-blood PCO2 gradient and fractional excretion of bicarbonate. Exome sequencing was performed using a panel of genes implicated in human disease. Results Patients had polyuria, hypokalemia, hypercalciuria, and nephrocalcinosis, but metabolic acidosis varied in severity. Although all patients acidified urine to pH < 5.3 during furosemide-fludrocortisone test, urine-to-blood PCO.sub.2 gradient was < 20 mmHg during bicarbonate loading. All patients had transient proximal tubular dysfunction with urinary losses of phosphate and beta-2-microglobulin, and generalized aminoaciduria. Homozygous pathogenic truncating variants in WDR72 was detected in all probands. Conclusion Patients with WDR72 mutations show mild rate-dependent distal RTA with variable metabolic acidosis, and intact ability to acidify the urine on provocative testing. Concomitant proximal tubular dysfunction may be present. Mutations in WDR72 should be considered in patients with suspected distal RTA, especially if associated with dental defects., Author(s): Priyanka Khandelwal [sup.1] Mahesh V [sup.1] , Vijay Prakash Mathur [sup.2] , Sumantra Raut [sup.1] , Thenral S. Geetha [sup.3] , Sandhya Nair [sup.3] , Pankaj Hari [sup.1] , [...]

Idiopathic nephrotic syndrome newly affects 1-3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4-6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10-30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed comprehensive clinical practice recommendations on the diagnosis and management of SRNS in children. The team performed a systematic literature review on 9 clinically relevant PICO (Patient or Population covered, Intervention, Comparator, Outcome) questions, formulated recommendations and formally graded them at a consensus meeting, with input from patient representatives and a dietician acting as external advisors and a voting panel of pediatric nephrologists. Research recommendations are also given., Author(s): Agnes Trautmann [sup.1] , Marina Vivarelli [sup.2] , Susan Samuel [sup.3] , Debbie Gipson [sup.4] , Aditi Sinha [sup.5] , Franz Schaefer [sup.1] , Ng Kar Hui [sup.6] , [...]

Background While patients with frequently relapsing nephrotic syndrome (FRNS) are initially treated with long-term alternate-day prednisolone, relapses and adverse effects are common. In an open-label randomized controlled trial, we compared the efficacy of therapy with low-dose daily to standard alternate-day prednisolone in reducing relapse rates over 12-month follow-up. Methods Consecutive patients, aged 2-18 years, with FRNS were included. Following therapy of relapse, prednisolone was tapered to 0.75 mg/kg on alternate days. Stratifying for steroid dependence, patients were randomly assigned to prednisolone at 0.2-0.3 mg/kg daily or 0.5-0.7 mg/kg alternate day for 12 months. Relapses were treated with daily prednisolone, followed by return to intervention. Primary outcome was the incidence of relapses. Proportion with therapy failure ([greater than or equal to] 2 relapses in any 6 months or significant steroid toxicity) and sustained remission, cumulative prednisolone intake and adverse events were evaluated. Results Patients receiving daily prednisolone (n = 30) showed significantly fewer relapses than those on alternate-day therapy (n = 31) (0.55 relapses/person-year versus 1.94 relapses/person-year; incidence rate ratio 0.28; 95% CI 0.15, 0.52). Daily therapy was associated with higher rates of sustained remission at 6 months (73.3 versus 48.4%) and 1 year (60 versus 31.6%; log rank p = 0.013), lower rates of treatment failure at 6 months (3.3 versus 32.8%) and 1 year (6.7 versus 57.4%; p < 0.0001), and lower prednisolone use (0.27 ± 0.07 versus 0.39 ± 0.19 mg/kg/day; p = 0.003). Three and two patients need to receive the study intervention to enable sustained remission and prevent treatment failure, respectively. Conclusions In patients with FRNS, daily administration of low-dose prednisolone is more effective than standard-dose alternate day therapy in lowering relapse rates, sustaining remission, and enabling steroid sparing., Author(s): Menka Yadav [sup.1] , Aditi Sinha [sup.1] , Priyanka Khandelwal [sup.1] , Pankaj Hari [sup.1] , Arvind Bagga [sup.1] Author Affiliations: (Aff1) 0000 0004 1767 6103, grid.413618.9, Division of [...]

Background Dyslipidemia is an important cardiovascular risk factor in steroid-resistant nephrotic syndrome (SRNS). Efficacy of statins for treatment of hyperlipidemia in children with SRNS is unclear. Methods This prospective, randomized, double-blind, placebo-controlled, parallel-group clinical trial enrolled 30 patients with SRNS, aged 5-18 years, with serum low-density lipoprotein cholesterol (LDL-C) levels between 130 and 300 mg/dl, to receive a fixed dose of atorvastatin (n = 15, 10 mg/d) or placebo (n = 15) by block randomization in a 1:1 ratio. Primary outcome was change in serum LDL-C at 12 months. Change in levels of other lipid fractions, carotid intima-media thickness (cIMT), flow-mediated dilation (FMD) of the brachial artery, and adverse events were also evaluated. Results At the end of 12 months, atorvastatin was not superior to placebo in reducing plasma LDL-C levels, median percentage reduction 15.8% and 9.5% respectively, in atorvastatin and placebo arms (n = 14 in each; P = 0.40). Apolipoprotein B levels significantly declined with atorvastatin in modified intention-to-treat analysis (P = 0.01) but not in the per-protocol analysis. There was no significant effect on other lipid fractions, cIMT and FMD. Adverse events were similar between groups. Change in serum albumin was negatively associated with change in serum LDL-C, very low-density lipoprotein cholesterol, total cholesterol, triglyceride, and apolipoprotein B (P < 0.001), irrespective of receiving atorvastatin, age, gender, body mass index, and serum creatinine. Conclusions Atorvastatin, administered at a fixed daily dose of 10 mg, was not beneficial in lowering lipid levels in children with SRNS; rise in serum albumin was associated with improvement in dyslipidemia., Author(s): Pankaj Hari [sup.1] , Priyanka Khandelwal [sup.1] , Amit Satpathy [sup.1] , Smriti Hari [sup.1] , Ranjeet Thergaonkar [sup.1] , R Lakshmy [sup.2] , Aditi Sinha [sup.1] , Arvind [...]

Author(s): Sudarsan Krishnasamy [sup.1] , Aditi Sinha [sup.1] , Arvind Bagga [sup.1] Author Affiliations: (1) grid.413618.9, 0000 0004 1767 6103, Division of Nephology, Department of Pediatrics, All India Institute of [...]

Byline: Priyanka. Madaan, Prashant. Jauhari, Shruthi. Michael, Aditi. Sinha, Biswaroop. Chakrabarty, Sheffali. Gulati Sir, Pediatric arterial ischemic stroke (AIS) is relatively uncommon and often under-recognized. It comprises 5%-10% of all [...]

Background Chronic kidney disease (CKD) predisposes to accelerated atherosclerosis that is measured by carotid artery intima-media thickness (cIMT) and brachial artery flow-mediated dilation (FMD). Information on the association of these parameters with dyslipidemia in pre-dialysis pediatric CKD is limited. Methods Eighty patients aged 9.9 ± 3.2 years, with estimated glomerular filtration rate of 38.8 ± 10.8 ml/1.73 m.sup.2/min, and 42 pediatric controls underwent cross-sectional analysis of lipid profile, cIMT, and brachial artery FMD. Significant differences in these parameters between patients and controls were analyzed using Student's t test. Predictors of cIMT and dyslipidemia were assessed using linear and logistic regression respectively. Results Patients had elevated blood levels of triglyceride and of total and LDL cholesterol than controls (P ≤ 0.001); 73.8 % were dyslipidemic. Mean cIMT was higher (0.421 ± 0.054 mm vs 0.388 ± 0.036 mm, P = 0.001) and brachial artery FMD was reduced (10.6 ± 4.9 % vs 18.9 ± 4.1 %, P < 0.0001) in patients compared with controls. On multivariate analysis, hypertension (OR 3.68, P = 0.044) and male gender (OR 10.21, P = 0.004) were associated with dyslipidemia; cIMT was significantly associated with LDL cholesterol (β = 28.36, P = 0.033). Conclusion Dyslipidemia was prevalent and cIMT significantly elevated in pre-dialysis pediatric CKD, indicating increased cardiovascular risk. Elevated LDL cholesterol predicted increased cIMT, strengthening the association between dyslipidemia and atherosclerosis in early CKD., Author(s): Priyanka Khandelwal[sup.1] , Vijaya Murugan[sup.1] , Smriti Hari[sup.2] , Ramakrishnan Lakshmy[sup.3] , Aditi Sinha[sup.1] , Pankaj Hari[sup.1] , Arvind Bagga[sup.1] Author Affiliations: (1) Division of Nephrology, Department of Pediatrics, [...]

Background Anti-complement factor H (anti-CFH) antibody-associated hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury in Indian children. While management comprises plasma exchange and immunosuppression, information on the impact on serial antibody titers and outcomes is limited. Methods This retrospective study included 45 patients with anti-CFH-associated HUS who were followed for [greater than or equal to] 12 months. Following the initial plasma exchange sessions, patients received prednisolone and either intravenous (IV) cyclophosphamide (n=31) or IV rituximab (n=14), followed by maintenance immunosuppression. Results The median anti-CFH antibody titers fell from 3,215.5 [interquartile range (IQR) 1,977.9-8,453.9 to 414.6 (IQR 251.6-1,368.2) AU/ml with plasma exchange therapy (P Conclusions Our patients with anti-CFH antibody-associated HUS showed a significant fall in antibody titers following daily plasma exchange sessions. Therapy with cyclophosphamide- or rituximab-based regimens was associated with similar outcomes and a comparable decline in antibody titers. Keywords Plasma exchange * Rituximab * Thrombotic microangiopathy * Cyclophosphamide * Complement factor H, Introduction Anti-complement factor H (CFH) autoantibodies are detected in a significant proportion of patients with hemolytic uremic syndrome (HUS) in India [1] compared to frequencies reported elsewhere [2, 3]. The [...]

Background The benefits of long-term low-dose antibiotics in preventing urinary tract infection (UTI) and renal damage in children with primary vesicoureteric reflux (VUR) are unclear. Methods Children aged between 1 and 12 years with VUR grade I-IV and a microbiologically proven UTI were randomized into two groups to receive either antibiotic prophylaxis [2 mg/kg trimethoprim + sulfamethoxazole (TMP-SMX)] daily or placebo, respectively, for 12 months. Primary outcome was microbiologically confirmed symptomatic UTI. Intention-to-treat analysis using time-to-event data was performed. Results A total of 93 children (66.7% boys) with a median age of 4.6 years were enrolled in this study; VUR grade III--IV was present in 73.1% of these children. At least one symptomatic UTI occurred in ten (21.3%) patients receiving antibiotic prophylaxis and in three (6.5%) patients receiving placebo [hazard ratio in antibiotic group 3.9; 95% confidence interval (CI) 1-14; log rank test P = 0.02). Compared to the group receiving placebo, the antibiotic group had a 14.8% increased risk for developing UTI (95% CI 1-28; P = 0.03). Of the total number of episodes of UTI, 58 3% of those in the antibiotic group were caused by TMP-SMX-resistant bacteria compared to 20% in the placebo group (P = 0.15). A renal scan at 12 months revealed that six of 37 (16.2%) patients in the antibiotic group and seven of 43 (16.3%) patients in the placebo group had new or worsening of pre-existing scar. Conclusions Long-term antibiotic prophylaxis with TMP-SMX is associated with increased risk of symptomatic UTI compared to placebo in children with grade I--IV VUR. Keywords Urinary tract infection * Trimethoprim-sulphamethoxazole * Asymptomatic bacteriuria * Bacterial resistance * DMSA scan * Renal scar, Introduction Urinary tract infection (UTI) occurs in about 2% of boys and 8% of girls [1], and one-third of children with proven UTI have vesicoureteric reflux (VUR) [2]. Data from [...]

Patients with end-stage renal disease require renal replacement therapy with maintenance hemodialysis or chronic peritoneal dialysis while awaiting transplantation. In addition to economic issues and limited state funding for advanced health care, the lack of trained medical personnel contributes to scarce dialysis facilities for children in developing countries. The establishment and operation of a hemodialysis unit with multidisciplinary facilities is both cost- and labori-ntensive. Hemodialysis is usually carried out three times a week in a hospital setting and affects the curricular and extracurricular activities of the patient. Chronic ambulatory or cyclic peritoneal dialysis is technically simpler and allows better nutrition and growth, but is expensive for the majority of patients who must pay out of their own pocket. Multiple initiatives to enhance the training of pediatricians and nurses in skills related to initiating and managing patients on maintenance dialysis have resulted in the improved survival of children with end-stage renal disease. Support from state governments and philanthropic institutions have helped in establishing pediatric nephrology units that are equipped to provide renal replacement therapy for children. Keywords Hemodialysis * International Pediatric Nephrology Association * Peritoneal dialysis * Developing countries, Introduction Renal replacement therapy (RRT) is increasingly used in the care of children with end-stage renal disease (ESRD). While the modality of the treatment is influenced by its availability, costs, [...]

Although therapy with intravenous (IV) rituximab and tacrolimus reduces the relapse rate in steroid-dependent nephrotic syndrome (SDNS), studies on comparative efficacy are lacking. We retrospectively reviewed the records of patients with difficult-to-treat SDNS who had previously received levamisole, cyclophosphamide and/or mycophenolate mofetil, then treated with either rituximab or tacrolimus and followed for 12 months. Between January 2009 and April 2010, ten patients received two to three doses of IV rituximab (375 mg/[m.sup.2]/week) and 13 received tacrolimus (0.1-0.2 mg/ kg/day) for 12 months; none had previously received either agent. Patients received tapering doses of alternate-day prednisolone; other immunosuppressive agents were discontinued. The mean age of the patients at treatment initiation with rituximab and tacrolimus was 12.2 [+ or -] 2.3 and 12.3 [+ or -] 3.0 years, respectively. The respective pre-treatment relapse rates (3.1 [+ or -] 1.1 and 3.5 [+ or -] 1.6 relapses per year) and cumulative prednisolone dose (137.2 [+ or -] 69.4 and 140.5 [+ or -] 59.0 mg/kg/year) were similar. Therapy resulted in a decline in relapse rate in both groups (P Keywords Focal segmental glomerulosclerosis. Minimal change disease.Proteinuria, Introduction A significant proportion of patients with idiopathic nephrotic syndrome show frequent relapses or steroid dependence [1]. Although alternative medications, such as cyclophosphamide, levamisole and mycophenolate mofetil (MMF), reduce the [...]

Frasier syndrome is characterized by progressive glomerulopathy that is unresponsive to corticosteroids, male pseudohermaphroditism, and an increased risk of genitourinary tumors. Of 21 girls with steroid-resistant nephrotic syndrome secondary to focal segmental glomerulosclerosis (FSGS) who were screened for mutations in the WT1 gene, two showed Frasier syndrome. Both patients had donor splice-site mutations in intron 9 of the WT1 gene and a male karyotype (46, XY). Long-term therapy with cyclosporine resulted in partial remission in both cases. One patient showed foci of gonadoblastoma in the excised dysgenetic gonads. This report highlights the need for screening for mutations in the WT1 gene in girls with steroid-resistant FSGS. Patients with Frasier syndrome might benefit from early gonadectomy. Keywords Cyclosporine * Gonadoblastoma. WT1 gene, Introduction Frasier syndrome, characterized by male pseudohermaphroditism and nephropathy presenting as steroid-resistant nephrotic syndrome (SRNS) is caused by mutations in the Wilms tumor (WT1) gene (OMIM 607102) [1]. Elective gonadectomy [...]

Byline: Aditi. Sinha, Harinderjeet. Goyal, Shailja. Singh, Shiv. Rana Background: A correlational survey to assess the quality of life (QoL) of ostomates with selected factors in a selected hospital of [...]

Sirs, We read with interest the article describing the utility of eculizumab in two patients with anti-complement factor H (CFH)-antibody-associated hemolytic uremic syndrome (HUS) [1]. Eculizumab, administered every 1-2 weeks [...]