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Amyloidosis is a group of disorders with extracellular accumulation of autologous fibrillary insoluble proteins in various tissues and organs such as the kidneys, liver, spleen, heart, and gastrointestinal tract leading to impaired function. Renal amyloidosis is usually considered to be a progressive disease manifesting as nephrotic syndrome and renal insufficiency with tuberculosis being one of the most common causes in adults. However, the occurrence of similar associations is rarely described in children. We report a case of an 11-year old girl who presented with nephrotic syndrome and pulmonary tuberculosis and attained disease remission with successful treatment of tuberculosis., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

IgA nephropathy and IgA vasculitis with nephritis, albeit rare, represent two relatively frequent glomerular conditions in childhood. Compared to adults, pediatric IgA nephropathy has a more acute presentation, most frequently with synpharyngitic macrohematuria and histologically with more intense inflammation and less intense chronic damage. Management of these conditions is controversial and supported by little high-quality evidence. The paucity of evidence is due to the disease heterogeneity, its inter-ethnic variability, and the difficulty of extrapolating data from adult studies due to the peculiarities of the condition in children. IgA vasculitis with nephritis is a kidney manifestation of a systemic disorder, typical of the pediatric age, in which both the diagnosis of kidney involvement and its management are poorly defined, and an interdisciplinary approach is crucial. Both conditions can have a profound and long-lasting impact on kidney function and the global health of affected children. The International Pediatric Nephrology Association has therefore convened a diverse international group of experts from different disciplines to provide guidance on the recommended management of these conditions in children and to establish common definitions and define priorities for future high-quality, evidence-based collaborative studies for the benefit of children., (© 2024. The Author(s).)

Background: There is a paucity of literature on the seroprevalence of SARS-CoV-2 antibodies among pediatric patients with underlying kidney disorders; few serosurveys among healthy children have shown seropositivity of 20-65% after different waves of infections., Methods: The study had a cross-sectional design and was conducted between January 2023 and July 2023; 163 children and adolescents (1-18 years) with nephrotic syndrome and chronic kidney disease (CKD) were screened for Anti-Spike SARS-COV-2 IgG antibodies as detected by a quantitative chemiluminescence immunoassay. Children with nephrotic syndrome, both steroid sensitive (SSNS) and steroid resistant (SRNS) were enrolled during disease remission. Correlation of SARS-CoV-2 seropositivity status was done with age, gender, disease type, treatment duration, immunosuppressants, previous SARS-CoV-2 infection, and immunization status., Results: Of 163 children (63.8% boys) with median age of 9 years; 101 (62%) had underlying nephrotic syndrome (61 SSNS and 40 SRNS), and 62 (38%) children had CKD. Seroprotective titers for SARS-COV2 antibodies were present in 100 (61.3%) children. The median titers for all patients were 37.1 BAU/mL; for nephrotic syndrome they were 27.1 BAU/mL and for CKD they were 76.7 BAU/mL (p = 0.0033). A total of 43 (26.4%) children had high positive antibody levels (> 200 BAU/ml). Among those with nephrotic syndrome 60.7% with SSNS and 43.5% SRNS had seropositive titers. Only 4 (2.5%) children had a history of previous COVID infection and 6 (3.7%) were vaccinated., Conclusions: In a largely unvaccinated population of children with nephrotic syndrome and CKD, 61.3% were seropositive for SARS-CoV-2 IgG antibody indicating a past asymptomatic infection; titers were significantly higher in CKD compared to nephrotic syndrome., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Congenital portosystemic shunts (CPSS) are rare congenital vascular anomalies characterized by abnormal connections between the portal vein and systemic circulation, bypassing the liver. They can lead to complications such as recurrent encephalopathy, liver nodules, portopulmonary hypertension, and neurocognitive issues due to hyperammonemia and rarely kidney involvement. Hepatic hemodynamic changes can lead to liver nodules and hepatocellular carcinoma, particularly in extrahepatic shunts. We describe here an 11-year-old girl with type 1 intrahepatic portosystemic shunt with focal nodular hyperplasia in the liver, presenting with nephrotic syndrome that was diagnosed as membranoproliferative glomerulonephritis on kidney biopsy and that responded partially to therapy with immunosuppressants., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Objectives: To study the urinary neutrophil gelatinase-associated lipocalin (NGAL) and beta-2-microglobulin (β2M) levels as markers of tubular damage in children with type 1 diabetes (T1DM)., Methods: Forty T1DM children and 40 age-matched controls were enrolled. Subjects with coexisting kidney disorder, intake of oral glucose lowering drugs and syndromic diabetes mellitus were excluded. Fasting plasma glucose, glycated hemoglobin (HbA 1c ), kidney function, urinary albumin-creatinine ratio (UACR), NGAL and β2M were measured and compared in cases and controls., Results: The median (IQR) age of cases and controls was 10.6 (8, 14.2) and 10.7 (8.4, 13.7) years, respectively. Cases had disease duration of 4 (3, 6.8) years and HbA 1c 10.9 (9, 13.1) %. Microalbuminuria was seen in 14 (35 %). Median (IQR) levels of UACR were higher in cases than controls [19.38 (10.27, 35.26) and 6.49 (3.10, 11.65) µg/mg; p<0.001], similarly NGAL/creatinine [352.21 (191.49, 572.45) and 190.54 (125.91, 322.83) ng/mg; p=0.006], unlike β2M/creatinine [1.7 (0.43, 6.02) and 2.12 (1.05, 4.47) µg/mg; p=0.637]. Children with higher HbA 1c (≥10 %) had higher urinary ACR and tubular biomarkers than HbA 1c <10 % (p>0.05). Urinary ACR showed positive correlation with NGAL/creatinine (r=0.38, p=0.019) and β2M/creatinine (r=0.42, p=0.009)., Conclusions: Urinary biomarkers NGAL and β2M were elevated in the presence of normal urinary microalbumin levels suggestive of early tubular damage in T1DM., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

Background: Children with nephrotic syndrome are exposed to alternate day steroids for prolonged periods and this poses the need for evaluation of adrenocortical suppression using the adrenocorticotropic hormone (ACTH) stimulation test., Methods: This cross-sectional study enrolled children (2-18 years) both with steroid sensitive nephrotic syndrome (SSNS) (n = 27) and steroid resistant (SRNS) (n = 25); those on daily prednisolone or having serious bacterial infections or hospitalized were excluded. The primary objective was to determine prevalence of adrenocortical suppression in those on low dose alternate day steroids for more than 8 weeks or having received > 2 mg/kg/d for > 2 weeks in the past 1 year and currently in remission. A baseline morning fasting sample of serum cortisol was taken and 25 IU of ACTH (Acton Prolongatum*) injected intramuscularly and repeat serum cortisol sample taken after 1 h. All patients with 1 h post ACTH cortisol < 18.0 µgm/dl were diagnosed with adrenal insufficiency. Receiver operating characteristic curve was drawn to predict the prednisolone dose for adrenal insufficiency., Results: Fifty-two (33 males) children were enrolled (mean age 9.4 years); proportion of adrenal insufficiency was 50% and 64% using baseline and post stimulation cutoffs. The total cumulative annual dose of prednisolone 0.22 mg/kg/day predicted adrenocortical suppression with AUC 0.76 (95% CI 0.63-0.89), with sensitivity of 63.9% and specificity of 81.3%., Conclusions: A significant proportion of children with nephrotic syndrome were detected with adrenal insufficiency on ACTH stimulation test. A cumulative steroid intake of > 0.22 mg/kg/day on an alternate day basis emerged as a risk factor for predicting adrenocortical suppression., (© 2024. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: While the utility of beta-2 microglobulin (β2M) has been explored in various renal conditions to identify tubulointerstitial damage, it has not been adequately studied in nephrotic syndrome. The primary objective of the study was to compare urinary β2M levels in children with steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS) in disease remission., Materials and Methods: This cross-sectional study was done at a tertiary care hospital between April 2019 and March 2020. Sixty children (2-18 years) with SSNS and SRNS (30 in each group) in remission were enrolled. SRNS patients were included after ≥1 year of treatment with calcineurin inhibitors (CNIs). Biochemical investigations were done to confirm remission; spot samples for urinary β2M were collected and estimation was done by an enzyme-linked immunosorbent assay (ELISA)-based kit., Results: Of the 60 children, 63% were boys. The median (interquartile range [IQR]) age at enrollment for SSNS and SRNS patients was 7 (4.1-9) and 11 (8.3-12) years, respectively. Urinary β2M levels were significantly higher in SRNS patients compared to SSNS patients (2.6 vs. 0.75 mg/ml, P < 0.0001). Patients who received cyclosporine for >2 years had higher median urinary β2M levels compared to those who received it for a shorter period (2.63 vs. 1.83 mg/ml, P = 0.03). Median β2M levels were higher in focal segmental glomerulosclerosis than minimal change disease (3.5 vs. 2.5 mg/ml)., Conclusion: Urinary β2M levels were higher in SRNS compared to SSNS disease in remission, and β2M levels correlated well with CNI use of >2 years. It appears to be a promising noninvasive tool to identify early tubular damage and progression in patients with nephrotic syndrome, especially SRNS., Competing Interests: There are no conflicts of interest., (© 2024 Indian Journal of Nephrology | Published by Scientific Scholar.)

Background: Dyslipidemia in children with chronic kidney disease (CKD) is identified based on lipid profile parameters; however, changes in lipoprotein quality precede quantitative changes., Methods: A cross-sectional study was done from January to October 2021; overweight, obese children, known cases of diabetes mellitus, hypothyroidism or on steroid therapy, or lipid lowering drugs were excluded. Clinical details were elicited and examinations done. Besides hemogram, kidney function tests, liver function tests, total cholesterol, low density lipoproteins (LDL), triglycerides, high density lipoproteins (HDL), and apolipoproteins A-1 and B were estimated to identify dyslipidemia. Relevant tests of significance were applied, and ROC curves were drawn for apoA-1, apoB, and apoB/apoA-1 ratios., Results: A total of 76 (61 M:15 F) children with median (IQR) age 7 (3.25-11) years were enrolled; cause of CKD was CAKUT in 82.3% patients. Dyslipidemia (alteration of 1 or more lipid parameters) was seen in 78.9% with a prevalence of 71.7% in early and 95.7% in later stages of CKD (P = 0.02); most had elevated serum triglyceride levels. The median (IQR) values of apoB, apoA-1, and apoB/apoA-1 ratio were 78 (58-110) mg/dl, 80 (63-96.75) mg/dl, and 0.88 (0.68-1.41), respectively; apoB, apoA-1, and apoB/apoA-1 ratio had a sensitivity of 26.67%, 86.67%, and 70%, respectively, and specificity of 87.5%, 62.5%, and 62.5%, respectively, for diagnosis of dyslipidemia. The ROC for apoB, apoA-1, and apoB/apoA-1 ratio showed AUC of 0.66, 0.68, and 0.74 (P = 0.4, 0.02, < 0.01), respectively., Conclusions: The prevalence (78.9%) of dyslipidemia was high in patients with CKD especially in those with later stages. The ratio of apoB/apoA-1 was altered early and appears to be promising for early detection., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Objectives: To compare the change in serum vitamin D levels and to compare the changes in serum levels of calcium, phosphate, alkaline phosphatase and parathyroid hormone in vitamin D supplemented and unsupplemented groups after 3 mo., Methods: In this randomized, parallel group, nonblinded, controlled trial, 40 children, 2-12 y of age with newly diagnosed epilepsy, and vitamin D sufficient status, and started on valproate monotherapy, were randomized into the intervention group (n = 20), which was given daily oral 600 IU vitamin D supplementation, and the control group (n = 20), which was not given any supplementation. Changes in the biochemical parameters was measured in the two groups after 3 mo., Results: There was a significant reduction in the median (IQR) vitamin D levels in the control group as compared to an increase seen in the intervention group [-6.64 (-8.4, -2.65) vs. 5.66 (1.81, 7.12); p < 0.001]. In the control group, 37.5% children developed vitamin D insufficiency and 12.5% developed deficiency whereas only 5% of the intervention group developed vitamin D insufficiency (p = 0.005). There was a significant decrease in ionized calcium (p = 0.02), increase in serum phosphate (p = 0.02), and alkaline phosphatase level (p = 0.003) in the unsupplemented group as compared to the supplemented group., Conclusion: Vitamin D supplementation can reduce the valproate-associated decline in vitamin D levels and the negative impact on other markers of bone mineral metabolism., Trial Registration: TCTR20200621002, 19.06.2020, retrospectively registered., (© 2022. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

Introduction: There may be a role of reducing the total steroid doses for the treatment of relapses of nephrotic syndrome in children with milder and more stable disease. The primary objective of this study was to compare the effectiveness of a low-dose prednisolone regimen with standard therapy for the treatment of relapses in steroid-sensitive nephrotic syndrome (SSNS) at the end of treatment, the secondary objectives being time to remission and sustained remission after 3 months., Methods: This randomized controlled trial included a total of 40 children (20 in each group) with SSNS (presently infrequently relapsing course) and with a relapse. Both groups received prednisolone at a dose of 2 mg/kg/day until remission; subsequently, the patients in the study group received 1 mg/kg, and the control group participants received 1.5 mg/kg prednisolone on alternate days for 4 weeks. The patients were followed up till 3 months after stopping the therapy., Results: The median (IQR) age of children enrolled was 7.5 (range: 5-9.65) years, and the age at onset of nephrotic syndrome was 4 (range: 2.3-5.5) years. The median time to achieve remission was 9 days (comparable in low dose vs. standard therapy group; P = 0.14). All patients were in remission at the end of therapy; 85% of patients were in the low-dose group and 90% in the standard therapy group after 1 month ( P = 0.32). At the end of 3 months, 60% continued to be in remission in the low-dose group and 65% with standard therapy ( P = 0.37). Hazard ratios for relapse at the end of 1, 2, and 3 months were 1.05, 1.08, and 1.13, respectively. Patients who were infrequently relapsing (79%) from the onset of nephrotic syndrome had higher remission rates at the end of 3 months (80% in the low-dose group vs. 76.9% in the standard therapy group). Hazard ratios for relapse in these patients at the end of 1, 2, and 3 months were 1.01, 1.03, and 1.08, respectively., Conclusions: Lower doses of prednisolone can be used for the treatment of relapse of steroid sensitive nephrotic syndrome, with an infrequently relapsing course., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Indian Journal of Nephrology.)

We performed a cross-sectional study on 25 children (17 boys) with urolithiasis with normal glomerular functions at a tertiary care teaching hospital between March, 2018 to March, 2019. Dietary assessment showed that caloric intake was below recommended dietary allowance (RDA) in 68% patients while the median protein intake was 34.3% more. The fluid intake was below the recommended standards in 56%, and 48% of the children had urine output below 1.5 mL/kg/hour. The urinary sodium was elevated in 96% of the children, urinary potassium was low in 40%, and hypercalciuria was seen in 28%. While metabolic causes predominate in childhood urolithiasis, other factors like dietary changes, liberal fluid and low sodium intake are advised for prevention of recurrences as they have a contributory role too.

Introduction: Steroid-resistant nephrotic syndrome (SRNS) is a rare condition that accounts for about 10% to 20% of all nephrotic syndromes in children. While calcineurin inhibitors induce remission in the majority, the data on long-term outcomes are limited. This retrospective study aimed to look at the clinical profile, biopsy findings, and long-term treatment outcomes in children with SRNS., Methods: The records of all children (1-18 years) with SRNS with biopsy findings of minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangioproliferative glomerulonephritis, who received treatment for a minimum period of 12 months and were in follow-up during the years 2007-2018 at a tertiary care teaching hospital were retrieved. The clinical, histopathological, and biochemical factors and treatment outcomes were recorded and analyzed., Results: Ninety-one (72 boys) children with a median (interquartile range [IQR]) age of onset of nephrotic syndrome as 48 (24-87) months were included. MCD and FSGS were the most common histopathological types (57.1% and 36.3%, respectively) and 62 (68.1%) patients had late steroid resistance. Calcineurin inhibitors (CNIs) were used in 86.8% of the children, and response rates with cyclosporine and tacrolimus for complete remission (CR) were 80% and 73.7%, respectively, with median (IQR) time to response being 3 (2-4) months. The presence of MCD on histology and the use of CNIs were significantly associated with CR ( P < 0.01). At a median (IQR) follow-up of 5 (3-7) years, 76 (83.5%) children had either CR or partial remission, four (4.4%) developed chronic kidney disease and five (5.5%) died (three due to end-stage renal disease and two of infective complications)., Conclusion: SRNS children with MCD on biopsy, late resistance, and response to CNIs have better long-term outcomes. Most patients respond to CNIs within the first 6 months of use and need therapy for at least 24 to 36 months., Competing Interests: There are no conflicts of interest., (Copyright: © Indian Journal of Nephrology.)

Background: Information on the course of SARS-CoV-2 infection in children with chronic kidney disease (CKD) is limited., Methods: We retrospectively reviewed the presentation and outcomes of SARS-CoV-2 infection in patients with CKD followed at any of the four pediatric nephrology centers in New Delhi from April 2020 to June 2021. Outcomes, including cardiopulmonary and renal complications, were reported in relation to underlying disease category and illness severity at presentation., Results: Underlying illness in 88 patients included nephrotic syndrome (50%), other CKD stages 1-4 (18.2%), CKD 5D (17%), and CKD 5T (14.8%). Thirty-two of 61 patients with symptomatic COVID-19 and 9/27 asymptomatic patients were admitted for median 10 (interquartile range 7-15) days. Seventeen (19.3%) patients developed moderate or severe COVID-19. Systemic complications, observed in 30 (34.1%), included acute kidney injury (AKI, 34.2%), COVID-19 pneumonia (15.9%), unrelated pulmonary disease (2.3%), and shock (4.5%). Nineteen (21.6%) had severe complications (AKI stage 2-3, encephalopathy, respiratory failure, shock). Eight (11%) of twelve (16.4%) patients with severe AKI required dialysis. Three (3.4%) patients, two with steroid-resistant nephrotic syndrome in relapse and one with CKD 1-4, died due to respiratory failure. Univariate logistic regression indicated that patients presenting with nephrotic syndrome in relapse or moderate to severe COVID-19 were at risk of AKI (respective odds ratio, 95%CI: 3.62, 1.01-12.99; 4.58, 1.06-19.86) and/or severe complications (respective odds ratio, 95%CI: 5.92, 1.99-17.66; 61.2, 6.99-536.01)., Conclusions: Children with CKD presenting with moderate-to-severe COVID-19 or in nephrotic syndrome relapse are at risk of severe complications, including severe AKI and mortality. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2021. IPNA.)

Purpose: The study was conducted to evaluate the role of Mycoplasma pneumoniae (M. pneumoniae) in children with community-acquired lower respiratory tract infections (LRTIs)., Methods: Seventy five children aged 2 months -12 years with community-acquired LRTIs were investigated for M. pneumoniae etiology employing paired serum samples to assay M. pneumoniae antibodies. Nasopharyngeal aspirates were obtained for the detection of M. pneumoniae by using polymerase chain reaction(PCR) and nested PCR., Results: M. pneumoniae infection was positive in 24(85.71%) children aged <5 years and 4 (14.29%) ​≥ ​5-12 years and the difference was statistically insignificant (P ​= ​0.18). Difference in prevalence of M. pneumoniae infection across male and female groups was statistically insignificant (P ​= ​0.69). Clinical and radiological profiles across M. pneumoniae positive and negative cases were comparable except bronchopneumonia which was statistically significant (P ​= ​0.04). Serological evidence of M. pneumoniae infection was observed in 26(33%); PCR was positive in 9 (12%) and nested PCR in 10 (13.33%) children. Together, serology, PCR and nested PCR diagnosed M. pneumoniae infection in 28(37.33%) patients. Sensitivity of serology was 77.78%: specificity 68.18%; positive predictive value 25.00% and negative predictive value at 95.74%., Conclusions: Serological and molecular methods in combination is useful for detection of M. pneumoniae. Our data underline the role of M. pneumoniae in community-acquired LRTIs in children of all ages., Competing Interests: Declaration of competing interest None., (Copyright © 2022 Indian Association of Medical Microbiologists. Published by Elsevier B.V. All rights reserved.)

Background: Coronavirus disease 2019 (COVID-19) is a novel disease caused by the SARS-CoV-2 virus and has emerged as a deadly pandemic affecting countries all over the world. Here we share our experience of managing adults with chronic kidney disease (CKD) and concomitant COVID-19 infection jointly managed by pediatric and adult nephrology teams., Methods: This retrospective study was done on patient admissions (>18 years) between 20 th June- 30 th October 2020 with previously diagnosed CKD and hospitalised with COVID-19 infection. The demographic details, underlying comorbidities, clinical presentation, medications, laboratory, radiological profile and outcomes were studied., Results: A total of 213 adults (62% males) with CKD were admitted during this period with a median (IQR) age of 52 (42, 60) years; 75 (35.2%) had associated diabetes mellitus, 83.1% hypertension, 5.2% hypothyroidism and 7% coronary artery disease. 165 (77.5%) were on maintenance haemodialysis (MHD), and 72.8% had arteriovenous fistula as vascular access at presentation. Most (84.5%) patients were symptomatic for COVID-19, and about 2/3 diarrhoea had moderate to severe disease. Oxygen therapy was needed in 62.9%, and medications used were hydroxychloroquine in 84.5%, azithromycin in 21.6%, ivermectin in 82.6%, steroids in 63.8% and Low molecular weight heparin in 59.2%. A further comparison of patients with CKD5D and CKDND revealed similar parameters except for a higher incidence of diarrhoea, acute kidney injury (AKI) and a shorter period to RTPCR negativity (12.5 vs 15 days; P = 0.038) in CKDND. The overall mortality was 24.4%, with similar mortality rates in both groups (P = 0.709) and 20.7% needed ICU transfer., Conclusions: Adults with CKD especially on haemodialysis, are prone to more severe COVID-19 infection and take a longer time for viral clearance (>2 weeks); the mortality too is higher in these patients., Competing Interests: The authors have none to declare., (© 2022 Director General, Armed Forces Medical Services. Published by Elsevier, a division of RELX India Pvt. Ltd.)

Hepatitis B-related glomerulonephritis (GN) is an uncommon but important cause of renal morbidity in children. While immunosuppressive therapy has been tried along with antivirals for treatment, some children may undergo spontaneous remission or achieve remission with antivirals alone. We retrospectively studied the outcomes of children with nephrotic syndrome (NS) and chronic hepatitis B infection treated at our nephrology clinic over a five years period; seven children were included of which six (86%) presented with NS and one with nephritic syndrome. Renal biopsy (done in 5 children) showed membranous GN in two (40%), membranoproliferative GN in one (20%), and focal segmental glomerulosclerosis in two (40%). Entecavir therapy was started in 6/7(86%) and four (57%) achieved remission after a median period of 2.7 months and achieved hepatitis B e-antigen seroconversion after mean duration of 1.2 years of treatment with entecavir; the remaining achieved remission with immunosuppression with calcineurin inhibitors., Competing Interests: None

Primary hypothyroidism is observed in adult patients with chronic kidney disease (CKD) though described scantily in the pediatric population. The primary objective of this study was to detect the prevalence of hypothyroidism in children (1-18 years) with CKD as assessed by thyroid profile. This cross-sectional study was conducted in the department of pediatrics of a tertiary care teaching hospital between January 2016 and January 2017. Clinical examination and biochemical investigations were performed for children with CKD aged 1 -18 years. Sixty-five children (51 boys, 43 CKD Stages 1-3) with a mean [standard deviation (SD)] age of 7.9 (3.2) years were enrolled. Overall, 17 (26.2%) had thyroid dysfunction; nine (13.8%) had subclinical hypothyroidism, three (4.6%) overt hypothyroidism, and five (7.7%) had isolated low T3 levels. The prevalence of hypothyroidism increased from 20.9% in CKD Stages 1-3 to 40.9% in Stages 4-5 of CKD; P = 0.09. The mean (SD) height SD scores were lower in those with hypothyroidism than with normal thyroid function [-1.02 (1.69) and -1.89 (1.12), P = 0.003, respectively], lowest at -2.79 (0.65) in overt hypothyroidism. A significant proportion of children with CKD manifest with hypothyroidism who have more profound growth failure. It may be prudent to screen CKD patients for thyroid dysfunction.

Justification: The management of steroid resistant nephrotic syndrome (SRNS) is challenging. These guidelines update existing 2009 Indian Society of Pediatric Nephrology recommendations on its management., Objective: To frame revised guidelines on diagnosis and evaluation, treatment and follow up, and supportive care of patients with the illness., Process: The guidelines combine evidence-based recommendations and expert opinion. Formulation of key questions was followed by systematic review of literature, evaluation of evidence by experts and two face-to-face meetings., Recommendations: Fourteen statements provide updated advice for managing steroid resistance, and underscore the importance of estimating proteinuria and baseline kidney function, and the need for kidney biopsy and genetic screening. Calcineurin inhibitors are recommended as most effective in inducing remission of proteinuria, the chief factor associated with long-term renal survival. Advice on managing allograft recurrence, congenital nephrotic syndrome, and monitoring and supportive care, including transition of care, are described. This revised practice guideline is intended to improve management and patient outcomes, and provide direction for future research.

Objective: To determine seroprotective titres for diphtheria, pertussis, tetanus and measles in children with nephrotic syndrome who had received essential immunization., Methods: Children (2-18 years) with steroid sensitive nephrotic syndrome (SSNS) or steroid-resistant nephrotic syndrome (SRNS) who were in disease remission and had received essential childhood immunization were included. Anti-diphtheria, anti-pertussis, anti-tetanus and anti-measles antibody titres were measured., Results: Seventy-six (40 with SSNS; 36 with SRNS) children with mean (SD) age 7.54 (3.96) years were enrolled. The time elapsed since last vaccination was >5 years in 68.4% patients. The seroprotection rates for diphtheria, tetanus, pertussis, and measles were 86.8%, 93.4%, 31.6% and 77.6% respectively; lower in SRNS subjects compared to SSNS. Robust seroprotection titers (1.0 IU/mL) for diphtheria were seen in 23.8% SSNS and 17.9% SRNS; P=0.04, and for tetanus in 69.3% SSNS and 43.8% of SRNS subjects; P=0.03, respectively., Conclusions: Children with nephrotic syndrome especially those with SRNS have lower seroprotective titers for diphtheria, tetanus, pertussis and measles, necessitating a booster dose of DPT/DT/Td and MR/MMR.

Children with nephrotic syndrome (NS) have a number of potential risk factors for the development of acute kidney injury (AKI) including intravascular volume depletion, infection, exposure to nephrotoxic medication, and renal interstitial edema. This study was aimed to determine the incidence of AKI in children hospitalized with a relapse of NS and its short-term outcome. This prospective observational study was conducted from February 2017 to January 2018 at a tertiary care teaching hospital. A total of 54 children and adolescents (1-18 years) hospitalized with a diagnosis of NS and relapse with/or without other complications were enrolled. Clinical data and examination were recorded. AKI was defined using the Kidney Disease Improving Global Outcomes (KDIGO) serum creatinine criteria and Pediatric Risk, Injury, Failure, Loss, End-Stage Renal Disease (p-RIFLE) classification. Children who developed AKI during the first two weeks of hospitalization were followed up till recovery or six weeks whichever was earlier to determine the outcome and factors predisposing to AKI. The mean age of the study population was 59.5 months and 35 (64.8%) patients were male. Of the 54 patients hospitalized, 42 (77.8%) were admitted with infection-associated relapses while 22.2% of children had relapse alone. Diarrhea and spontaneous bacterial peritonitis were the most common infections (26.1% each) followed by urinary tract infections in 19% and pneumonia in 14.3%. Twenty-three (42.6%) children developed AKI according to the KDIGO definition and 27 (50%) using the pRIFLE classification. Fourteen (60.9%) had stage 2 AKI while 21.7% had stage 3 AKI. Infections [odds ratio (OR) 1.24] and use of angiotensin-converting enzyme inhibitors (ACEI) (OR 2.3) were the most common predisposing factors for AKI. The mean recovery time for AKI was 7.34 days. Development of AKI was associated with prolonged hospital stay (12.57 vs.8.55 days P <0.01) and delayed recovery. At the end of follow-up all children recovered from AKI. The incidence of AKI in children hospitalized with complications of NS is high. While the occurrence of these AKI episodes may appear transient, a recurrence of such episodes may be detrimental to the long-term outcome of children with NS. Infections and the use of ACEI during relapses are risk factor for the occurrence of AKI.

Competing Interests: There are no conflicts of interest.

The coronavirus outbreak is a rapidly evolving pandemic, placing unprecedented strain on health-care systems. COVID-19 presents challenges for management of children with renal diseases, especially those receiving long-term immunosuppressive medications, including renal transplant recipients and those with chronic kidney disease and acute kidney injury requiring dialysis. Our preparedness for managing this vulnerable group of children is the need of the hour. The purpose of this article is to provide guidance to caregivers and health care personnel involved in management of children with renal diseases and to ensure patient well-being, while protecting staff from infection.

Renal biopsy is an important investigation to make the diagnosis of an underlying glomerular or tubular disease, and is commonly done by trained pediatricians. In this review, we discuss the procedure and also detail important points in interpretation of renal biopsy in children.

Children with nephrotic syndrome (NS) encounter multiple episodes of relapses associated/triggered by an episode of infection. The primary objective of this study was to find the proportion of infection associated relapses that resolve on the treatment of acute infection over an observation period of two weeks in children with NS. This prospective observational study enrolled 45 children with steroid-sensitive NS presenting with an infection associated relapse during the study period (February 2015 to February 2016). Baseline information and examination findings of all children were recorded. Biochemical and other investigations were performed according to the site of infection for all patients and were treated appropriately. None of the patients received daily 2 mg/kg of prednisolone during the observation period. All children were followed for two weeks for resolution of relapse and subsequently every month for another three months. The 45 patients (median age 66 months) enrolled in the study had 64 episodes of infections, of which upper respiratory tract infections (45%) were the commonest, followed by peritonitis (18.5%) and diarrhea in 12%. Twenty-seven (60%) patients achieved remission on symptomatic treatment of infection with/without the use of stress doses of prednisolone. Most (77.8%) patients who achieved remission without the use of daily 2 mg/kg of prednisolone did so within the 1 st week and a majority of patients were still in remission at three months follow-up. We conclude that most infection associated relapses can be managed with treatment of underlying infection alone and use of stress doses of steroids for inducing remission without increasing the prednisolone doses to 2 mg/kg/d and thus reducing the cumulative steroid doses.

Systemic lupus erythematosus (SLE) in children is associated with renal involvement in a majority. While glomerular involvement is a common manifestation, tubular involvement is rare. Tubular dysfunctions previously described with SLE are renal tubular acidosis (Type 1 and less commonly type 4). Isolated renal tubular acidosis without glomerular involvement has not been reported in childhood SLE. We report an adolescent girl with SLE, autoimmune thyroiditis and distal RTA who subsequently developed autoimmune hemolytic anemia. During a follow up of almost 7 years the girl never developed any proteinuria while the RTA persisted. Also during this period her mother was also diagnosed with SLE manifesting as thrombocytopenia.

Background: A liquid Pentavalent (DTwP-Hb-Hib) combination vaccine, developed by Human Biologicals Institute, underwent a Phase III clinical study in India. In this randomized, single blind, non-inferiority study, the immunogenicity and safety of this Investigational vaccine was compared with Pentavac SD® vaccine in 6-8 weeks old healthy infants., Methods: A total of 405 healthy infants aged 6-8 weeks old were randomized in 2:1 ratio to receive three doses of either the Investigational liquid Pentavalent (DTwP-Hb-Hib) combination vaccine or Pentavac SD® vaccine at four to six weeks interval. Immunogenicity was compared by estimation of antibody titers before the first dose and 4-6 weeks after the third dose of vaccination. Safety of each vaccine was assessed and compared by collection of data on solicited and unsolicited adverse events throughout the study period., Results: Out of a total of 405 enrolled subjects, 387 subjects completed the study. The seroconversion rates, seroprotection rates and geometric mean titres of the Investigational liquid Pentavalent (DTwP-Hb-Hib) combination vaccine group were found to be comparable and non-inferior to the Pentavac SD® vaccine group at 4-6 weeks after the third dose of vaccination. Pain, erythema and swelling at the site of injection were found to be the most common local adverse events whereas fever, irritability and unusual crying were found to be the most common systemic adverse events in both the vaccine groups. No vaccine related serious adverse event was reported. In this study, both the Investigational vaccine as well as the Comparator vaccine were found to be immunogenic and well tolerated., Conclusion: After assessment of the results of the study it was concluded that the Investigational liquid Pentavalent (DTwP-Hb-Hib) combination vaccine developed by Human Biologicals Institute was immunogenic and safe when administered to infants aged 6-8 weeks and was non-inferior in immunogenicity and safety to Pentavac SD® vaccine. Clinical Trial Registry of India Identifier: CTRI/2016/01/006541., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of unchecked activation of the immune system leading to phagocytosis of blood cells and proliferation of histiocytes in solid organs. HLH can be primary or secondary to infective, autoimmune and malignant conditions. Scrub typhus is an infective illness caused by Orientia tsutsugamushi, transmitted by mite. The illness ranges from mild fever with rash to severe multisystem illness. Scrub typhus has rarely been associated with secondary HLH. We report an infant with scrub typhus who progressed to develop HLH with central nervous system involvement with fatal outcome.

Introduction: Mantoux test aids in the diagnosis of tuberculosis (TB), however its application and interpretation are dependent on multiple factors., Methodology: A prospective study enrolling 400 children (aged 2-12) suspected to have tuberculosis. All participants received Mantoux test with two different strengths (1 TU and 5 TU) of Purified Protein Derivative (PPD) on different forearms. The test was read by two readers after 48 ± 2 and 72 ± 2 hours. Primary outcome was difference in the size of induration when read by two readers (interobserver variability). Secondary outcomes were difference in the size of induration at different intervals, with different strengths of PPD and percentage positivity of Mantoux test in TB patients., Results: Statistically significant difference was seen in the size of induration when read by two different readers, with fair to moderate agreement when read at 48 and 72 hours (1 TU: p = 0.002, k = 0.52 and p = 0.1, k = 0.73 respectively, 5 TU: p = 0.001, k = 0.39 and p = 0.0009, k = 0.33 respectively). Tendency of under-reading occurred when size of induration was close to significant level (10-14 mm). Size of induration was similar when read at 48 or 72 hours (1 TU: p = 0.9, 5 TU: p = 1.0). Mantoux positivity rate in patients with TB was more with 5 TU as compared to 1 TU (61.2% vs. 16.3%)., Conclusions: There is significant interobserver variability with a tendency to under-read around the cutoff point. The use of 5 TU PPD at 48 hours by a trained physician can aid in early and more reliable diagnosis of TB., Competing Interests: No Conflict of Interest is declared, (Copyright (c) 2018 Dimple Goel, Mukta Mantan, GR Sethi.)

Introduction: Acute Encephalitis Syndrome has heralded the emergence of multiple virulent pathogens, which may result in severe morbidity and mortality. In India, encephalitis is not notified and there has been a dearth of analysis for trends in encephalitis death rates and causation. A downward trend has been observed in encephalitis deaths, due to 'known' causes, which can be largely explained by improvement in diagnostic, treatment, and prevention methods. There is still a very high proportion of encephalitis deaths in developing countries, where the aetiological diagnosis of the pathogen is not established and thus, lies the importance of monitoring encephalitis morbidity and mortality with a view to improve pathogen diagnosis and identify emerging infectious diseases., Aim: To formulate a diagnostic approach to viral acute encephalitis syndrome in paediatric age group., Materials and Methods: A cross-sectional study including 50 paediatric patients, clinically diagnosed with acute encephalitis syndrome using WHO criteria was conducted. The CSF of all the patients was evaluated to diagnose the aetiology for viral pathogens. ELISA was used for diagnosing Japanese encephalitis and dengue encephalitis; and multiplex real time PCR was used for detecting HSV-1, HSV-2, Varicella zoster virus, Mumps virus, Enterovirus and Parechovirus., Results: Confirmed diagnosis was established in 11 (22%) of 50 cases. A confirmed or probable viral agent of encephalitis was found in 7 (14%), bacterial agent was found in 2 (4%), non-infectious aetiology was found in 2 (4%). Fatal outcome was independently associated with patient age., Conclusion: Despite extensive testing, the aetiologies of more than three fourth of the cases remains elusive. Nevertheless the result from the present study may be useful for future design of early diagnosis and treatment of the disease. New strategies for pathogen identification and continued analysis of clinical features and case histories should help us improve our ability to diagnose, treat and prevent encephalitis.