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Background Sequential rituximab (RTX) administration has emerged as an important strategy to sustain remission of disease in patients with difficult-to-treat nephrotic syndrome. Methods We report the efficacy and safety of sequential therapy with two or more courses of intravenous RTX in 250 patients with difficult-to-treat steroid dependence (n  = 127) and calcineurin inhibitor (CNI)-dependent or CNI-refractory steroid resistance (n  = 123) managed at one center during 2015–2021. Subsets of patients were cross-sectionally tested for hypogammaglobulinemia, seroprotection against and hyporesponsiveness to vaccines for hepatitis B and tetanus, BK/JC viruria and human antichimeric antibodies (HACAs). Results Sequential RTX therapy, initiated at a median of 10 years [interquartile range (IQR) 7.3–14.4], was administered for 1.8 courses/person-year [95% confidence interval (CI) 1.7–2.0] over 2.0 years (95% CI 1.2–3.0). Therapy was associated with postponement of relapses by a median of 3 years in patients with steroid-sensitive disease and 2 years in those with steroid resistance. Relapses were reduced by a mean of 2.0 relapses/person-year (95% CI 1.8–2.2), enabling a reduction in prednisolone dose to 0.04 mg/kg/day (95% CI 0.01–0.11) and withdrawal of additional immunosuppression in 154 (62%) patients. RTX-associated adverse events, occurring at 0.20 events/person-year (95% CI 0.17–0.23), were chiefly comprised of infusion reactions (n  = 108) and infections (n  = 46); serious adverse events were observed in 10.8% patients, at 0.03 events/person-year (95% CI 0.02–0.05). Hypogammaglobulinemia was observed in 35% of 177 patients and was moderate to severe in 8.5% of cases. Rates of seroprotection at baseline and response following vaccination were lower for hepatitis B [1.9% and 29.4% (n  = 52)] than tetanus [65.5% and 34.5% (n  = 58)]. BK/JC viruria, without viremia, was observed in 7.3% of 109 cases. A total of 19 of 107 patients (17.8%) had HACAs, which were associated with B cell nondepletion and serum sickness. Age at therapy of <9–10 years was associated with a risk of early relapse, treatment failure and hypogammaglobulinemia following RTX therapy. Conclusions Sequential therapy with RTX effectively reduces relapses in patients with difficult-to-treat steroid- and/or CNI-dependent or CNI-refractory nephrotic syndrome. Therapy is associated with high rates of hypogammaglobulinemia and infusion reactions. [ABSTRACT FROM AUTHOR]

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, [...]

Objectives: To provide up-to-date information on the use of long-term ventilation (LTV) in the UK paediatric population and to compare the results with data collected 10 and 20 years previously.Design: A single timepoint census completed by LTV centres in the UK, carried out via an online survey.Setting and Patients: All patients attending paediatric LTV services in the UK.Results: Data were collected from 25 LTV centres in the UK. The total study population was 2383 children and young people, representing a 2.5-fold increase in the last 10 years. The median age was 9 years (range 0-20 years). Notable changes since 2008 were an increase in the proportion of children with central hypoventilation syndrome using mask ventilation, an increase in overall numbers of children with spinal muscular atrophy (SMA) type 1, chronic lung disease of prematurity and cerebral palsy being ventilated, and a 4.2-fold increase in children using LTV for airway obstruction. The use of 24-hour ventilation, negative pressure ventilation and tracheostomy as an interface had declined. 115 children had received a disease-modifying drug. The use of ataluren and Myozyme did not influence the decision to treat with LTV, but in 35% of the children with SMA type 1 treated with nusinersin, the clinician stated that the use of this drug had or may have influenced their decision to initiate LTV.Conclusion: The results support the need for national database for children and young people using LTV at home to inform future recommendations and assist in resource allocation planning. [ABSTRACT FROM AUTHOR]

Objectives To assess the awareness and practice of two-wheeler riders regarding the helmet use, and to determine the association of awareness and practices with selected variables. Setting and Design This article is a descriptive survey, which was completed at All India Institute of Medical Sciences (AIIMS) premises, New Delhi, India. Materials and Methods The pretested and validated tools developed by researcher consisted of demographic sheet (8 items) along with structured awareness and practice questionnaire. Results and Conclusions Maximumparticipants were male (71.06%) with majority riding for 8 years. Asmuch as 48%of the sample population had accidentswhile driving. Only 2.9% of them reported to have sustained severe injury during these accidents. The mean awareness and practice score related to helmet use were 49.58±6.019.75±5.56. There was weak correlation between awareness and practice. Association of awareness and practices with selected variables could not be observed (p-value--0.4870). Although public awareness is present, but law needs to be more stringent. Accidents are fatal and it can happen anywhere and anytime, irrespective of long or short distance, and wearing of helmet can save a person from major injuries. [ABSTRACT FROM AUTHOR]

The importance of the citrus crops is recognized throughout the world. Despite tremendous advancements in agricultural technology, citrus fruit production is still considered a significant challenge. Various biotic factors have trampled down the production rate of Citrus in many places. Among them, canker caused by Xanthomonas axonopodis pv. citri (Xac) and Xanthomonas citri pv. citri (Xcc) is of great importance. Infection caused by Xac leads to lesion development in leaves, fruits, and stem. Defoliation and early fruit drop can occur as a result of severe infection, resulting in a loss of fruit output. Preliminary management techniques involve quarantining and sanitizing. Chemical copper-based bactericides are frequently employed in places with large-scale production. However, the continued use of chemicals, on the other hand, has led to the evolution of resistant microorganisms and increased the rate of soil pollution. Various alternative strategies have been suggested to address this issue. This review will go over the advances in biocontrol agents that have been used to combat the disease. [ABSTRACT FROM AUTHOR]

Background More than 50% of children with chronic kidney disease (CKD) have uncontrolled hypertension, increasing their long-term risk of cardiovascular disease and progression to kidney failure. Children receiving medications or dialysis may also experience acute blood pressure fluctuations accompanied by debilitating symptoms. We aimed to describe the perspectives of children with CKD and their parental caregivers on blood pressure to inform patient-centered care. Methods Secondary thematic analysis was conducted on qualitative data from the Standardized Outcomes in Nephrology—Children and Adolescents initiative, encompassing 16 focus groups, an international Delphi survey and two consensus workshops. We analyzed responses from children with CKD (ages 8–21 years) and caregivers (of children ages 0–21 years) pertaining to blood pressure. Results Overall, 120 patients and 250 caregivers from 22 countries participated. We identified five themes: invisibility and normalization (reassured by apparent normotension, absence of symptoms and expected links with CKD), confused by ambiguity (hypertension indistinguishable from cardiovascular disease, questioning the need for prophylactic intervention, frustrated by inconsistent messages and struggling with technical skills in measurement), enabling monitoring and maintaining health (gaging well-being and preventing vascular complications), debilitating and constraining daily living (provoking anxiety and agitation, helpless and powerless and limiting life activities) and burden of medications (overwhelmed by the quantity of tablets and distress from unexpected side effects). Conclusions For children with CKD and their caregivers, blood pressure was an important heath indicator, but uncertainty around its implications and treatment hampered management. Providing educational resources to track blood pressure and minimizing symptoms and treatment burden may improve outcomes in children with CKD. [ABSTRACT FROM AUTHOR]