Mutational Landscape of Patients with Wiskott Aldrich Syndrome: Update from India.

Purpose: Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder characterized by distinctive features including microthrombocytopenia, eczema and recurrent infections. In the present study we report clinical, immunological and molecular spectrum of 41 WAS patients diagnosed over last five years.

Methods: Clinical and family history was collected from case records.

Factors Associated With Shock at Presentation in Kawasaki Disease Versus Multisystem Inflammatory Syndrome in Children Associated With Covid-19.

Background: While clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.

Methods: The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from January 1, 2020, to January 1, 2023.

Outcome of Injection Sclerotherapy With Doxycycline in Lymphatic, and Veno-lymphatic Malformation in Children: A Single Centre Experience.

Aim: Lymphatic malformations (LMs) and Veno-lymphatic malformations (VLMs) are congenital slow flow vascular malformations. Intralesional sclerotherapy is a good alternate option for treatment of LMs and VLMs. Our aim was to evaluate the outcome of doxycycline sclerotherapy for lymphatic and veno-lymphatic malformation in children in our institute.

The disappearing lesion in the cricopharynx: A hypopharyngeal cushion.

A 2-year-old girl presented to our aero-digestive clinic with complaints of dysphagia to solids. On awake endoscopy, a bluish, expansile lesion was noticed. posterior to the arytenoids, which became prominent when crying.

Ototoxic Drug Exposure and Hearing Loss in Neonates: A Scoping Review.

Purpose: This scoping review aims to map the effects of dosage levels, dosage intervals, duration of exposure, and serum concentration levels of gentamicin, amikacin, vancomycin, furosemide, and bumetanide on newborn hearing.

Method: Using PubMed, Scopus, and Ovid databases (January 2010-2022), a scoping review was conducted to identify studies on ototoxic drug exposure in neonates. The review included articles that described details on ototoxic drug exposure and hearing status, dosage levels, duration of exposure, and serum concentration levels.

Childhood maltreatment is linked to larger preferred interpersonal distances towards friends and strangers across the globe.

Childhood maltreatment (CM) is thought to be associated with altered responses to social stimuli and interpersonal signals. However, limited evidence exists that CM is linked to larger comfortable interpersonal distance (CID) - the physical distance humans prefer towards others during social interactions. However, no previous study has investigated this association in a comprehensive sample, yielding sufficient statistical power.

Abdominoscrotal Hydrocele - Considerations.

Aim: The aim of the study was to highlight the pathology, clinical spectrum, and approach considerations in abdominoscrotal hydrocele (ASH).

Materials And Methods: Our study included eight children with ASH from 2015 to 2022. The demographics, clinical presentation, investigations, operative details, and postoperative outcome were collected retrospectively from case files.

Demographic trend in dengue infection.

Background Objectives: Dengue fever is one of the most common causes of undifferentiated tropical fevers in hospitalized children across India with the highest recorded morbidity and mortality in children less than 15 years. Although common, there is still a knowledge gap regarding epidemiological factors. The aim was to study the clinical profile and demographic trend over the past 5 years in children with confirmed dengue infection.

Does Age Influence the Functional Recovery after Infant Pyeloplasty?

Purpose: The purpose was to study the correlation between age at surgery and functional recovery after infant pyeloplasty.

Materials And Methods: All infants who underwent pyeloplasty were analyzed retrospectively in this multicenter study. Anteroposterior diameter (APD) >2 cm, split renal function (SRF) <40%, and Society of Fetal Urology (SFU) grade 3-4 were surgical indications.

Whole genome analysis reveals unique traits of SARS-CoV-2 in pediatric patients.

Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) continues to challenge the global healthcare with emerging variants and higher infectivity as well as morbidities. This study investigated potential age-related variations through genomic characterization of the virus under common clinical settings. A cohort comprising 71 SARS-CoV-2 strains from both infected infants and accompanying adults, diagnosed via RT-PCR at a tertiary pediatric hospital and research center, underwent Illumina paired-end sequencing.

Evaluation of Pathway to Diagnosis of Pediatric Brain Tumors in Tamil Nadu, India.

Purpose: Delayed diagnosis and poor awareness are significant barriers to the early intervention of pediatric brain tumors. This multicenter observational study aimed to evaluate the baseline routes and time to diagnosis for pediatric brain tumors in Tamil Nadu (TN), with the goal of promoting early diagnosis and timely referrals in the future.

Methods: A standard proforma was used to retrospectively collect information on demographics, diagnosis, referral pathways, and symptoms of incident pediatric brain tumor cases between January 2018 and October 2020 across eight tertiary hospitals in TN.

Computational study of the piperidine and FtsZ interaction in Typhi: implications for disrupting cell division machinery.

FtsZ, a bacterial cell division protein, is essential for assembling the contractile Z-ring crucial in bacterial cytokinesis. Consequently, inhibiting FtsZ could impede proto-filaments, disrupting FtsZ and other associated proteins vital for cell division machinery. Conduct an drug interaction study to identify novel drug candidates that inhibit the FtsZ protein, aiming to prevent Multi-Drug Resistant (MDR) Typhi.

Sex-specific differences in systemic immune responses in MIS-C children.

Multisystem Inflammatory Syndrome in Children (MIS-C) is a rare manifestation of Severe Acute Respiratory Syndrome-CoronaVirus-2 (SARS-CoV-2) infection that can result in increased morbidity and mortality. Mounting evidence describes sex disparities in the clinical outcomes of coronavirus disease 2019 (COVID-19). However, there is a lack of information on sex-specific differences in immune responses in MIS-C.

Heparin vs Saline Infusion to Maintain Patency of Arterial Catheters in Children: A Randomized, Double-Blind, Noninferiority Trial.

Objective: To determine whether normal saline flush solution is noninferior to heparinised saline for maintaining the patency of arterial intravascular catheters in children.

Methods: A single centre, double blind, parallel group, noninferiority, randomized control study was conducted in the Pediatric Intensive Care Unit of Kanchi Kamakoti CHILDS Trust hospital, a tertiary children's hospital, Chennai, India. 92 children requiring arterial catheters for more than 12 hours were randomized to receive either normal saline or heparinized saline (1 U/ml) flush solution.

The appendix conduit with a conflict of interests - An alternate strategy.

Introduction: Conventionally the split appendix has been used to address the need for dual conduits such as the Mitrofanoff and the ACE, however limited by its length. We present a video demonstration of an alternate solution.

Material And Methods: Size-12 Nelaton catheter introduced via appendicular stump and skirted along the lateral wall of the caecum.

The Need for Culturally Appropriate Food Allergy Management Strategies: The Indian Milk Ladder.

Background: Cow's milk allergy (CMA) is one of the most common and complex food allergies affecting children worldwide and, with a few exceptions, presents in the first few months of life. Baked-milk-containing diets are well tolerated in the majority of milk-allergic children and allow dietary restrictions to be relaxed. In addition, the early introduction of tolerated forms of allergenic foods to an infant's diet in small amounts may enhance the outgrowth of their milk allergy through oral tolerance induction.

Global diversity and antimicrobial resistance of typhoid fever pathogens: Insights from a meta-analysis of 13,000 Typhi genomes.

Background: The Global Typhoid Genomics Consortium was established to bring together the typhoid research community to aggregate and analyse serovar Typhi (Typhi) genomic data to inform public health action. This analysis, which marks 22 years since the publication of the first Typhi genome, represents the largest Typhi genome sequence collection to date (n=13,000).

Methods: This is a meta-analysis of global genotype and antimicrobial resistance (AMR) determinants extracted from previously sequenced genome data and analysed using consistent methods implemented in open analysis platforms GenoTyphi and Pathogenwatch.

Feasibility of Enhanced Recovery after Surgery in Pediatric Colostomy Reversal.

Aim: Enhanced recovery after surgery (ERAS) are multimodal perioperative pathways that have shown improved outcomes. ERAS after colostomy reversal has shown promising results in adults and few pediatric studies. We report our experience using ERAS for a colostomy reversal.