Safety and Efficacy of Ecopipam in Patients with Tourette Syndrome: A Systematic Review and Meta-analysis.

Background And Objectives: Ecopipam is a selective antagonist of the dopamine D1 receptor, and its efficacy and safety have recently been explored in several clinical trials involving patients with Tourette syndrome (TS). The objectives of this systematic review were to determine the pooled estimate for efficacy [in terms of reduction in tic Yale Global Tic Severity Scale (YGTSS) scores] and safety of oral ecopipam in subjects with TS.

Methods: All clinical trials that explored the efficacy and/or safety of ecopipam in patients with TS were included to determine the pooled estimate for change in YGTSS, Clinical Global Impression (CGI)-TS, and the severity of comorbid attention-deficit hyperactive disorder (ADHD), obsessive compulsion disorder (OCD), and depressive symptoms, as well as the nature and frequency of adverse effects.

Modified Atkins Diet versus low glycemic index treatment in children with drug-resistant epilepsy: A systematic review and meta-analysis.

Introduction: Both the Modified Atkins Diet (MAD) and Low Glycemic Index Treatment(LGIT) are considered less restrictive than the ketogenic diet and effective in children with drug-resistant epilepsy(DRE). Several randomized controlled trials (RCTs) have compared these two diets.

Methods: All RCTs directly comparing MAD and LGIT for DRE were included in the review.

Efficacy of piracetam in children with breath-holding spells: a systematic review and meta-analysis.

A number of randomized-controlled trials (RCTs) have been conducted comparing the efficacy of piracetam with placebo and other medications in children with breath-holding spells (BHS). However, no systematic review has yet collated all this evidence. All RCTs comparing the efficacy and/or safety of piracetam with placebo or other medications in children with BHS were included.

Renal Amyloidosis in a Child with Recessive Dystrophic Epidermolysis Bullosa Due to a Novel Variant in Gene.

Secondary amyloidosis may complicate inherited dermatoses, but recessive dystrophic epidermolysis bullosa (RDEB) complicated by renal amyloidosis is rare. We report a case of a 12-year-old male child with RDEB presenting with progressive generalized anasarca for 20 days. Kidney biopsy showed diffuse expansion of mesangial matrix by pale acellular Periodic Acid-Schiff (PAS)-negative amorphous material, which was congophilic on Congo red stain and gave apple green birefringence on polarization and extending along the glomerular basement membrane, suggestive of amyloidosis.

Serum tumour necrosis factor-alpha as a marker of disease activity in children with nephrotic syndrome.

Idiopathic nephrotic syndrome (NS) is a common glomerular disease in children throughout the world; however, the exact pathogenesis of the disease remains unknown. Several studies have shown that tumour necrosis factor-alpha (TNF-α), a proinflammatory cytokine, plays a significant role in the pathogenesis of NS. The literature lacks sufficient data to establish the relationship between TNF-α and NS.

Copeptin as a potential biomarker of chronic kidney disease to predict the disease progression in children with chronic kidney disease.

Background: Biomarkers to predict the onset and progression of chronic kidney disease (CKD) in children are lacking, and no such definite biomarkers have been implicated in the diagnosis of CKD. We conducted this study to evaluate copeptin as a CKD marker and predict the disease progression by estimating the copeptin levels at baseline and 12 months follow-up in children with CKD stage 2 and above.

Materials And Methods: This prospective single-centre cohort study was conducted in children under 14 years with CKD stages 2-4.

Shear wave elastography of kidneys in children: utility in distinguishing steroid-resistant and steroid-sensitive nephrotic syndrome.

Purpose: To assess the renal elasticity values using (SWE) and correlate the values with steroid sensitivity to distinguish between steroid-resistant nephrotic syndrome (SRNS) and steroid-sensitive nephrotic syndrome (SSNS) in children.

Methods: In this IRB-approved cross-sectional study, 83 children (4-14 years) diagnosed with nephrotic syndrome were included from July 2021 to December 2022. SWE measurements were done for each kidney's upper pole, interpolar region, and lower pole.

Factors predicting the occurrence of disease-causing variants on next-generation sequencing in children with steroid-resistant nephrotic syndrome - implications for resource-constrained settings.

Background: Enhanced availability of high-throughput sequencing (at progressively reducing costs) has revolutionized the identification of monogenic SRNS. However, in resource-poor settings, it may not be possible to perform next-generation sequencing (NGS) in all children wherein monogenic SRNS is suspected. Besides, the optimal strategy of genetic evaluation (in patients with SRNS) in routine clinical practice in resource-limited settings is unknown.

Incidence, predictors, and short-term outcomes of acute kidney injury in children with diabetic ketoacidosis: a systematic review.

Background: One-third of children with type 1 diabetes mellitus manifest with diabetic ketoacidosis (DKA). Most children presenting with DKA are in a volume-depleted state, leading to acute kidney injury (AKI). Besides volume depletion, hyperglycemia can induce tubular injury and kidney inflammation.

Prevalence and risk factors for functional iron deficiency in children with chronic kidney disease.

Introduction: Anemia in chronic kidney disease (CKD) is multifactorial. The presence of functional iron deficiency (FID), whereby, there is a block in the transport of iron from macrophage to erythroid marrow is one possible etiology. In this study, we aim to assess the prevalence and risk factors of FID in pediatric CKD.

Long-term complications in patients with childhood-onset nephrotic syndrome.

Background: Reports on long-term complications of childhood-onset nephrotic syndrome (NS), such as obesity, osteoporosis, growth failure, and hypertension, are mostly from developed countries not representing South Asian ethnicities. Furthermore, data on cardiovascular health among patients with childhood-onset NS are limited.

Methods: This was an observational study involving patients attending a tertiary care center.

Efficacy and safety profile of selumetinib in symptomatic inoperable plexiform neurofibromas.

Introduction: Selumetinib is a MEK inhibitor, which is effective with an acceptable safety profile in reducing the volume of symptomatic inoperable plexiform neurofibromas in some clinical trials and also has been recently approved by FDA for use in children aged 2 years or older. However, no systematic review has yet been performed to provide a collective estimate of the results of all these trials.

Evidence Acquisition: Articles describing the use of selumetinib in patients with neurofibromatosis type-1 (NF1) with inoperable plexiform neurofibromas were searched from different electronic databases.

Distress, anxiety, and its correlates among caregivers of children with kidney diseases during COVID-19 pandemic lockdown.

Background: The COVID-19 pandemic has generated a significant amount of psychological burden in the form of stress, anxiety, uncertainty, depression, anger, and helplessness. The caregivers of children with chronic diseases in particular are at a higher risk of mental stress and burden.

Material And Methods: We conducted an online survey among caregivers of children with kidney diseases to assess the psychosocial impact of COVID-19.

Efficacy and Tolerability of Lacosamide in Lennox-Gastaut Syndrome: A Systematic Review and Meta-analysis.

 Lennox-Gastaut syndrome (LGS) is one of the most difficult to treat childhood-onset epileptic encephalopathies. There is growing evidence that lacosamide is safe and efficacious in patients and adults with refractory epilepsy. However, the evidence regarding the efficacy of lacosamide in LGS is controversial so far.

Systematic Review and Meta-analysis of Efficacy and Safety of Melatonin and Triclofos for Inducing Adequate Sedation for Sleep Electroencephalogram in Children.

 Triclofos and melatonin are commonly used oral sedatives in children for obtaining a sleep electroencephalogram (EEG) record. There has been no systematic review till now to compare the efficacy and safety of these two medications.  The review intended to compare the efficacy of oral triclofos and melatonin in children <18 years of age for inducing adequate sedation for obtaining a sleep EEG record.

Impact of anemia on the cardiovascular status in children with chronic kidney disease: A pilot study.

Background & Aims: Myocardial dysfunction is one of the common complications in children with chronic kidney disease which results in significant morbidity and mortality. We aimed to find the impact of anemia with cardiac changes in children with chronic kidney disease (CKD).

Methods: In this cross-sectional pilot study, 54 children (38 males) up to the age of 16 years with different stages of CKD, not on dialysis, were enrolled.

Diagnostic accuracy of renal angina index alone or in combination with biomarkers for predicting acute kidney injury in children.

Early recognition of patients at risk for severe acute kidney injury (AKI) by renal angina index (RAI) may help in the early institution of preventive measures. Objective was to evaluate performance of RAI alone or in combination with biomarkers in predicting severe AKI (KDIGO stage 2 and 3 or equivalent) and receipt of kidney replacement therapy (KRT) in critically ill children. We searched PubMed, EMBASE, Web of Sciences, and CENTRAL for studies published till May 2021.

Chronic peritoneal dialysis in children with chronic kidney disease: An experience from a North Indian teaching institute.

Introduction: Chronic peritoneal dialysis (CPD) is an important modality of renal replacement therapy (RRT) in children of all ages with end-stage renal disease (ESRD). We retrospectively assessed the clinical profile of children with chronic kidney disease (CKD) initiated on CPD at a tertiary care centre in Northern India.

Materials And Methods: Retrospective data of 13 children with CKD and initiated on CPD between 2016 and 2019 were retrieved and analysed.

Anti-Compliment Factor H Antibody Associated Hemolytic Uremic Syndrome in Children with Abbreviated Plasma Exchanges: A 12-Month Follow-up Study.

Introduction: Anti-Compliment Factor H antibody hemolytic uremic syndrome (AFH-HUS) is a common cause of paediatric atypical HUS in India. We wanted to study the outcome of patients receiving less than recommended plasma exchange (PLEX) but adequate immunosuppression, with respect to hypertension, preservation of renal function and proteinuria.

Methods: A retrospective study was performed in 15 children admitted from 2016 to 2018 with AFH-HUS.

COVID-19 treatment in children: A systematic review and meta-analysis.

Background: Exact information about the efficacy of various medications proposed by regulatory bodies in children with COVID-19 is limited due to the lack of controlled trials in the existing literature.

Methods: Different electronic databases (MEDLINE, EMBASE, Web of Science, COCHRANE CENTRAL, LitCovid, medRxiv, and bioRxiv) were searched for articles describing the management of COVID-19 cases in children with 18 shortlisted medications. Prospective/retrospective studies/case series (with at least 20 cases) reporting COVID-19 in patients aged ≤14 years were searched to collect information regarding clinical details and severity of participants, medications used, and outcome.