Rice is one of the oldest cultivated crops and most staple food crop for more than a third of the world's population.

Rituximab is a chimeric monoclonal antibody capable of depleting B cell populations by targeting the CD20 antigen expressed on the cell surface. Its use in oncology, initially in B cell lymphoma and post-transplant lymphoproliferative disorders, predates its current utility in various fields of medicine wherein it has become one of the safest and most effective antibody-based therapies. It was subsequently found to be effective for rheumatological conditions such as rheumatoid arthritis and antineutrophil cytoplasmic antibody-associated vasculitis. Over the past decade, rituximab has generated a lot of interest in nephrology and has become an emerging or accepted therapy for multiple renal conditions, including systemic lupus erythematosus, lupus nephritis, vasculitis, nephrotic syndrome and in different scenarios before and after kidney transplantation. This review outlines its current use in paediatric nephrology practice, focusing on the knowledge required for general paediatricians who may be caring for children prescribed this medication and reviewing them on a shared care basis.

Acute kidney injury (AKI) is a well-known life-threatening systemic effect of snake envenomation which commonly happens secondary to snake bites from families of Viperidae and Elapidae. Enzymatic toxins in snake venom result in injuries to all kidney cell types including glomerular, tubulo-interstitial and kidney vasculature. Pathogenesis of kidney injury due to snake envenomation includes ischaemia secondary to decreased kidney blood flow caused by systemic bleeding and vascular leakage, proteolytic degradation of the glomerular basement membrane by snake venom metalloproteinases (SVMPs), deposition of microthrombi in the kidney microvasculature (thrombotic microangiopathy), direct cytotoxic action of venom, systemic myotoxicity (rhabdomyolysis) and accumulation of large amounts of myoglobin in kidney tubules. Clinical features of AKI include fatigue, loss of appetite, headache, nausea, vomiting, oliguria and anuria. Monitoring of blood pressure, fluid balance, serum creatinine, blood urea nitrogen and serum electrolytes is useful in managing AKI induced by snake envenomation. Early initiation of anti-snake venom and early diagnosis of AKI are always desirable. Biomarkers which will help in early prediction of AKI are being explored, and current studies suggest that urinary clusterin, urinary neutrophil gelatinase-associated lipocalin, and serum cystatin C may play an important clinical role in the future. Apart from fluid and electrolyte management, kidney support including early and prompt initiation of kidney replacement therapy when indicated forms the bedrock in managing snake biteassociated AKI. Long-term follow-up is important because of chances of progression towards CKD.

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Background: Inherited tubulopathies are a heterogeneous group of genetic disorders making whole exome sequencing (WES) the preferred diagnostic methodology. Methods: This was a multi-centric descriptive study wherein children (<18 years) with clinically suspected tubular disorders were recruited for molecular testing through WES. Multiplex ligation-dependent probe amplification (MLPA) and Sanger sequencing were done when required. Variants were classified as per American College of Medical Genetics 2015 guidelines and pathogenic (P) / likely pathogenic (LP) variants were considered causative. Results: There were 77 index cases (Male =73%; female). Median age of diagnosis Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation was 48 months (IQR 18.5 to 108 months). At recruitment, number of children in each clinical group were as follows: Distal Renal Tubular Acidosis (dRTA) =25, Bartter syndrome=18, Isolated Hypophosphatemic rickets (HP) =6, Proximal tubular dysfunction (pTD) = 12, Nephrogenic Diabetes Insipidus (NDI) =6, Kidney stone / Nephrocalcinosis (NC) =6 and Others =4. We detected 55 (24 novel) P/LP variants, providing genetic diagnoses in 54 children (70%). The diagnostic yield of WES was highest for NDI (100%), followed by HP (83%; all X-linked HP), Bartter syndrome (78%), pTD (75%), dRTA (64%), and NC (33%). Molecular testing had a definite impact on clinical management in 24 (31%) children. This included revising clinical diagnosis among 14 children (26% of those with a confirmed genetic diagnosis and 18% of the overall cohort), detection of previously unrecognized co-morbidities among 8 children (sensorineural deafness: n=5, hemolytic anemia: n=2, and dental changes: n=1) and facilitating specific medical treatment among 7 children (Primary Hyperoxaluria: n=1, Cystinosis: n=4, Tyrosinemia: n=2). Conclusion: WES is a powerful tool in the diagnosis and management of children with inherited tubulopathies in the Indian population. Response to Reviewers:Reviewers' comments:Reviewer #1:Major concernsComment 1:The a priori inclusion criteria of this study remain unclear. Since the authors investigate multiple disease entities, they list a "phenotype description" for each disease entity in TI. However, it remains unclear if and to which extent this phenotype description needed to be fulfilled for the respective patient to be included in this supposedly prospective study in general and in the respective cohort in particular. Furthermore, the category "Others" seems to entirely consist of patients that did not fulfill any clear inclusion criteria beyond a presumptive diagnosis of tubulopathy by their treating physician. To avoid the impression of selective in-and exclusion of patients and post-hoc grouping of patient cohorts, the authors should clearly explain their inclusion process and, most importantly, add the actual phenotype of the respective patient in TII.Response: We would like to thank the reviewer for highlighting this concern. At the time of collecting blood sample for g...

<b><i>Background:</i></b> There is a paucity of information on epidemiology, diagnosis, and treatment outcomes of congenital nephrotic syndrome (CNS) in developing countries. <b><i>Methods:</i></b> Retrospective (2012–2017) review of case records undertaken across 12 Indian pediatric nephrology centers. <b><i>Results:</i></b> Sixty-five children (58% male, median birth weight 2.4 kg [interquartile range (IQR) 2.1–2.86]) were identified with CNS. Nearly half (45%) were preterm with previous history of fetal loss/sibling death in 22% and history of consanguinity in a third. No infective etiology was confirmed. Genetic reports available for 15 (23%) children identified causal mutations in 10 (8 in NPHS1 [1 novel variant], 1 in WT 1 [novel variant], and 1 in PLCE-1 gene). In addition, 1 child was clinically diagnosed as Galloway Mowat syndrome. Next-generation sequencing showed 80% yield and Sanger sequencing 20%. Albumin infusion and angiotensin-converting enzyme inhibitors were used initially in around two-third of cohort, while only 12% of children received indomethacin. Totally, 22 (34%) children were lost to follow-up after initial visit, and among the rest median follow-up was 69 days (IQR 20–180) with 18 (42%) deaths. Eight children showed partial response (including 2 with NPHS1 compound mutation), 1 complete response, and all of them were alive at last follow-up in contrast to 53% mortality among nonresponders, <i>p</i> = 0.004. <b><i>Conclusion:</i></b> This largest reported series on CNS from India revealed suboptimal management with poor outcome as well as low number of CNS being subjected to genetic evaluation.

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