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   Table of Contents - Current issue
January-June 2019
Volume 2 | Issue 1
Page Nos. 1-60

Online since Friday, May 17, 2019

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From the Editorial Board p. 1
Arvind Bagga
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Nutritional challenges across the spectrum of chronic kidney disease Highly accessed article p. 2
Kristen Sgambat, Kaushalendra Amatya, Asha Moudgil
Maintenance of optimal nutrition plays a key role in the management of children with chronic kidney disease (CKD) across different stages of CKD, during dialysis and following transplantation. Malnutrition, both under- and over-nutrition, is widely prevalent and negatively impacts short- and long-term outcomes. It leads to growth retardation, increased risk of hospitalization and infections, poor cognition, and decreased quality of life. Understanding of the pathogenesis of malnutrition is crucial for its proper management. This review discusses the definition, prevalence, pathogenesis, and management of malnutrition in different stages of CKD.
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Next-generation sequencing-based genetic diagnosis of steroid-resistant nephrotic syndrome: Benefits and challenges p. 16
Varsha Chottusing Pardeshi, Ambily Narikot, Anil Vasudevan
Steroid-resistant nephrotic syndrome (SRNS) is the second-most common cause of chronic kidney disease in children and in those requiring kidney transplantation. The disease shows significant heterogeneity in its age at onset and clinical course. The discovery of mutations in NPHS1, the gene encoding nephrin that is a key component of the podocyte slit diaphragm, in a subset of children with congenital NS, led to identification of a distinct subgroup of patients of SRNS that has an underlying genetic etiology. Subsequently, mutations in over 53 podocyte genes have been implicated in monogenic forms of SRNS with no clear genotype-phenotype correlations. The large number of genes implicated in SRNS, phenotypic variability, and lack of information about frequency of mutations in these genes, makes the use of genetic testing in the management of children with SRNS challenging in terms of decisions on who to test, which genes to screen, and how to use the information obtained from testing in the clinical setting. Given the genetic heterogeneity and phenotypic variability, Sanger sequencing is not a feasible approach for routine testing. Next-generation sequencing (NGS) technology is emerging as the preferred method to screen multiple genes in genetically heterogeneous diseases like SRNS. Such high-throughput sequencing method permits rapid and cost-effective simultaneous screening of large number of individuals and genes. However, the high throughput combined with significant phenotypic and genetic variability of monogenic SRNS poses unique challenges for clinicians in the interpretation of genetic result. This review provides an overview of utility of genetic testing with focus on NGS-based genetic testing and the challenges in the interpretation of genetic results in clinical settings.
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Renal angina index in the prediction of acute kidney injury in critically ill children p. 25
Suma Sundararaju, Aditi Sinha, Pankaj Hari, Rakesh Lodha, Arvind Bagga
Background: Renal Angina Index (RAI) was recently proposed as a tool to identify patients at high risk for severe acute kidney injury (AKI) by integrating baseline, contextual, and clinical evidence of renal injury and to optimize biomarker utility in intensive care units. Methods: In this unicentric prospective observational study, we estimated RAI at admission in 285 critically ill patients, aged 1 month to 18 years, and evaluated the utility of renal angina (RAI ≥8) in identifying patients with severe or any AKI on day 3 and day 7. The relationship between RAI and need for renal replacement therapy (RRT) and duration of mechanical ventilation and hospital stay was also examined. Results: Renal angina was present in 49.4% of 285 patients. Severe AKI, observed in 29 (10.2%) patients on day 3 and 13.2% of 144 patients followed to day 7, had an incidence of 1.1 (0.8–1.6) episodes per 100 person-days. Thirty-six (12.6%) patients required RRT. RAI satisfactorily identified patients at risk of severe AKI on days 3 (area under the curve [AUC]: 0.82; 95% confidence interval [CI]: 0.73–0.90) and 7 (AUC: 0.73; 95% CI: 0.62–0.84), was more useful than Pediatric Index of Mortality in such discrimination, and correlated with duration of mechanical ventilation and hospital stay. However, RAI thresholds ≥12 or ≥20 had higher specificity, Youden index, and positive predictive value than that of RAI ≥8 in discriminating severe AKI on day 3 or 7 and distinguished between patients with and without need for RRT. Conclusions: RAI usefully predicts the development of subsequent severe AKI on days 3 and 7, and is associated with duration of mechanical ventilation and hospital stay. A higher RAI threshold (≥12 or ≥20) is more discriminatory than RAI ≥8.
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Outcomes of pediatric renal transplantation at a single center p. 31
Mital Parikh, Umapati Hedge, Abhijit Konnur, Sishir Gang, Mohan Rajapurkar, Hardik Patel, Neel Patel
Introduction: Renal transplantation is the preferred treatment option for children with end-stage renal disease (ESRD). There is scant information on comparative outcomes of pediatric renal transplantation in centers in developing regions. Materials and Methods: We retrospectively reviewed outcomes of renal transplantation in pediatric recipients of renal transplantation at a single center between 2001 and 2018. Information of underlying etiology of ESRD, pretransplant renal replacement therapy (RRT) modality, donor type, immunosuppression regimen, surgical complications, rates of rejection, compliance to immunosuppression, graft survival, and overall patient survival is presented. Results: Of 100 patients (77 boys) who received transplantation at the mean age of 15.7 ± 2.3 (5–18) years, chief underlying etiology of ESRD was chronic glomerulonephritis (n = 19), chronic tubulointerstitial nephritis (n = 31), and Alport syndrome (n = 1). Modality of RRT was predominantly hemodialysis (n = 90) or peritoneal dialysis (n = 6). Allograft survival was 98%, 85%, 80%, and 73% at 1, 3, 5, and 10 years, respectively. No deaths were observed. Conclusions: Pediatric renal transplantation at a single center in India was associated with satisfactory short-term patient and allograft outcomes. Rates of allograft survival at 10-year follow-up were lower as compared to other unicentric reports from the region.
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Pediatric acute poststreptococcal glomerulonephritis: A single-center experience p. 36
Sherif Mohamed El-Desoky, Lujain K I. Al-Sulimani, Manar T O. Alkhatieb, Khalid A Alhasan, Amr S Albanna, Jameela A Kari
We evaluated the frequency, severity, and outcome of children with acute poststreptococcal glomerulonephritis (PSGN) by retrospectively reviewing case records of all children diagnosed with PSGN between 2007 and 2015. The diagnosis of PSGN was based on the evidence of recent streptococcal infection, including positive streptococcal culture from the skin or throat, high or rising antistreptolysin O titer (>333 IU/ml), or high anti-deoxyribonuclease B level (>200 IU/ml). In addition, low serum complement 3 level and/or kidney biopsy results were considered. Children with evidence of chronic kidney disease (CKD) were excluded from the study. Sixty-six children (50 boys) with age 8.4 ± 3.4 years were included in the study. Ninety percent of the children had proteinuria and 79% had impaired glomerular filtration rate at presentation. One child had rapidly progressive glomerulonephritis. At 1-year follow-up, 10% of the children had renal impairment, 25% had hypertension, and 40% had proteinuria. As a considerable percentage of patients with PSGN in this series had CKD at 1-year follow-up, careful prolonged follow-up of such patients is advisable.
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Plasma Infusion versus plasma exchange as first line therapy in atypical hemolytic uremic syndrome: A single centre experience p. 41
Shankar Prasad Yadav, Arpana Iyengar, Anil Vasudevan
Plasma exchange (PEX) remains the standard therapy for the management of atypical hemolytic-uremic syndrome (aHUS) in regions where eculizumab is not available. Where even PEX is not feasible or afforded, patients are managed with plasma infusion (PI). This single-center, retrospective study compares the efficacy of PI with PEX and examined for predictors of renal recovery in pediatric patients with aHUS admitted at a single center in a developing country from January 2005 to June 2017. Endpoints for comparison were time to hematological remission, duration of hospitalization, rates of complete and partial renal recovery, time to progression to chronic kidney disease Stage 5, and occurrence of relapses. Of 53 children presenting at 6.3 ± 3.9 years of age, 24 and 22 patients were managed with PI and PEX, respectively. The latter group of patients was sicker at presentation and included 13 children with antibodies to complement factor H. All patients achieved hematological remission, including four patients refractory to PI who were switched to PEX. Patients managed on PI were discharged from hospital earlier than those on PEX and were more often with complete renal recovery (55% vs. 27%). At 1.5 (0.5–72) months of follow-up, similar proportions of 31 patients managed with PI and PEX showed complete and partial renal recovery and had comparable rates of relapse and disease progression. Presentation with acute kidney injury Stage 3 and edema were independent predictors of incomplete renal recovery at discharge. Both PEX and PI are associated with satisfactory short-term outcomes in patients with aHUS lacking access to eculizumab, and PEX might be preferred over PI in patients with severe disease and those with anticomplement factor H antibodies.
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Ulbright-Hodes syndrome p. 46
Majid Malaki
Phocomelia is a well known dysmorphism associated with antenatal use of thalidomide. Phocomelia may also be a part of many syndromes that overlap with each other. This case report presents a case of Ulbright-Hodes syndrome in a 7-yr-old boy with phocomelia, hypoplastic kidneys and short stature.
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Atypical hemolytic uremic syndrome with critical illness neuropathy p. 48
Neethu Rajeev, Georgie Mathew, Aditi Sinha, Pankaj Hari, Arvind Bagga
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Fractures, abnormal skull shape, and metabolic acidosis in a young child p. 50
Sherif Mohamed El-Desoky, Jameela A Kari
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Targeting dry weight of children on maintenance dialysis by bio-impedance analysis p. 54
Arpana Aprameya Iyengar, Anil Vasudevan
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Journal Scan p. 56
Christy Cathreen Thomas
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