Six-Month Outcomes in the Long-Term Outcomes After the Multisystem Inflammatory Syndrome in Children Study.

Importance: Multisystem inflammatory syndrome in children (MIS-C) is a life-threatening complication of COVID-19 infection. Data on midterm outcomes are limited.

Objective: To characterize the frequency and time course of cardiac dysfunction (left ventricular ejection fraction [LVEF] <55%), coronary artery aneurysms (z score ≥2.

Effects of Food Processing on Allergenicity.

Purpose Of Review: There is an increasing awareness among clinicians that industrial and household food processing methods can increase or decrease the allergenicity of foods. Modification to allergen properties through processing can enable dietary liberations. Reduced allergenicity may also allow for lower risk immunotherapy approaches.

Cardiac growth patterns and metabolism before and after birth in swine: Role of miR in proliferation, hypertrophy and metabolism.

The adult mammalian heart is unable to undergo cardiac repair, limiting potential treatment options after cardiac damage. However, the fetal heart is capable of cardiac repair. In preparation for birth, cardiomyocytes (CMs) undergo major maturational changes that include exit from the cell cycle, hypertrophic growth, and mitochondrial maturation.

Impact of obesity on outcome in children diagnosed with cancer in Canada: A report from Cancer in Young People in Canada.

Background: Childhood obesity can result in adverse health outcomes. The objectives of this study were to describe the prevalence of obesity and determine the association between obesity at cancer diagnosis and event-free survival (EFS) and overall survival (OS) in children diagnosed with cancer in Canada.

Methods: The authors conducted a retrospective cohort study using the Cancer in Young People in Canada database, including all children with newly diagnosed cancer aged 2-18 years across Canada from 2001 to 2020.

Staged Approach: The Role of Delayed Repair Following Complete Unifocalization of Major Aortopulmonary Collateral Arteries with Ventricular Septal Defect and Pulmonary Atresia.

The presentation of pulmonary vasculature in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCA) is highly variable-as is the number, size and position of the MAPCAs and their relationship with the native pulmonary artery system. The priority in the management of this disease should be attaining timely and complete unifocalization, as opposed to single-stage full repair in every case. The merit of early unifocalization is that it secures the pulmonary vascular bed by (a) avoiding loss of lung segments from progressive stenosis/atresia of MAPCA origins, (b) preventing lung injury from high pressure/flow in areas fed by large, unobstructed MAPCAs, and (c) restoring central continuity of the pulmonary vasculature.

Does using the Lung Clearance Index (LCI) inform clinical decisions in children with cystic fibrosis?

Introduction: The Lung Clearance Index (LCI) is an established research test, but its role in clinical decision-making is not well defined. This study estimated the proportion of treatment decisions that are changed or supported by the added information provided by LCI.

Methods: A mixed methods prospective observational study was conducted in North America.

Right ventricular remodeling in complex congenital heart disease.

In congenital heart diseases (CHD) of moderate to great complexity involving the right ventricle (RV), the morphologic RV can be exposed to significant stressors across the lifespan either in a biventricular circulation in a sub-pulmonary or sub-aortic position, or as part of a univentricular circulation. These include pressure and/or volume overload, hypoxia, ischemia, and periprocedural surgical stress leading to remodeling, maladaptation, dilation hypertrophy and dysfunction. This review examines the macroscopic remodeling of the RV in various forms of CHD and explores remodeling trajectories, along with the effects of surgeries and residual lesion repair, in tetralogy of Fallot, Ebstein anomaly, congenitally corrected transposition of the great arteries, transposition of the great arteries with atrial switch surgery, and single ventricle palliated by Fontan.

Home oxygen therapy for Thai preterm infants with bronchopulmonary dysplasia. What are the predictive factors for successful weaning: a 20-year review.

Background: Consequences of lung injury and inflammation in preterm infants with bronchopulmonary dysplasia (BPD) contribute to prolonged oxygen requirements. Home oxygen therapy (HOT) is an alternative way of respiratory support in infant with BPD. However, there is no consensus on weaning guidelines.

The association between self-reported total gestational weight gain by pre-pregnancy body mass index and moderate to late preterm birth.

Background: Inadequate and excessive gestational weight gain (GWG) defined by the Institute of Medicine (IOM) has been associated with preterm birth. However, studies demonstrate inconsistent associations.

Objectives: We examined the associations between categorical and continuous total GWG and moderate to late preterm birth (32-<37 weeks), and evaluated differences in these associations by pre-pregnancy BMI.

Systematic Analysis of the Design, Methodology and Patient Population Characteristics of the Pediatric Direct Oral Anticoagulant (DOAC) Trials of Venous Thromboembolism Treatment.

Introduction: The pediatric direct oral anticoagulation (DOAC) trials provide an opportunity to evaluate and characterize challenges in their design and execution to inform future antithrombotic trials.

Objective: To perform a systematic review of pediatric DOAC trials for the treatment of venous thromboembolism to critically appraise their methodology and understand the feasibility and challenges.

Methods: Systematic search of MEDLINE, EMBASE, the Cochrane Library and ClinicalTrials.

Left-Right Brain-Wide Asymmetry of Neuroanatomy in the Mouse Brain.

Left-right asymmetry of the human brain is widespread through its anatomy and function. However, limited microscopic understanding of it exists, particularly for anatomical asymmetry where there are few well-established animal models. In humans, most brain regions show subtle, population-average regional asymmetries in thickness or surface area, alongside a macro-scale twisting called the cerebral petalia in which the right hemisphere protrudes anteriorly past the left.

LINE1 elements at distal junctions of rDNA repeats regulate nucleolar organization in human embryonic stem cells.

The nucleolus is a major subnuclear compartment where ribosomal DNA (rDNA) is transcribed and ribosomes are assembled. In addition, recent studies have shown that the nucleolus is a dynamic organizer of chromatin architecture that modulates developmental gene expression. rDNA gene units are assembled into arrays located in the p-arms of five human acrocentric chromosomes.

Association of Serum Polyunsaturated Fatty Acids (PUFAs) and Children's Dietary Inflammatory Index (C-DII) with Recurrent Respiratory Infections in Children: A Cross-Sectional Study.

To assess the associations between serum and dietary polyunsaturated fatty acids (PUFAs), as well as the inflammatory potential of diet measured by the Children's Dietary Inflammatory Index (C-DII), and recurrent respiratory infections (RRIs) in children. We enrolled 44 children aged 3-16 years with RRIs and 44 healthy controls. Dietary intake was assessed using a 7-day food record from which PUFA intake and C-DII were calculated.

From Cellular to Metabolic: Advances in Imaging of Inherited Retinal Diseases.

: Inherited retinal diseases (IRDs) are a genetically complex group of disorders, usually resulting in progressive vision loss due to retinal degeneration. Traditional imaging methods help in structural assessments, but limitations exist in early functional cellular-level detection that are crucial for guiding new therapies. : This review includes a systematic search of PubMed and Google Scholar for studies on advanced imaging techniques for IRDs.

Extracorporeal Membrane Oxygenation in Pediatric Patients with Congenital Heart Disease: Surgical Considerations.

The use of extracorporeal membrane oxygenation (ECMO) in the pediatric population has increased over time, with the ability to rescue pulmonary and cardiovascular deterioration. ECMO can be utilized by neonates and children with congenital heart disease in both the preoperative and post-operative setting to improve survival and minimize morbidity. ECMO cannulation strategy must be tailored to age, weight and physiological state of the patient.

Multiomics dissection of human RAG deficiency reveals distinctive patterns of immune dysregulation but a common inflammatory signature.

Human recombination-activating gene (RAG) deficiency can manifest with distinct clinical and immunological phenotypes. By applying a multiomics approach to a large group of -mutated patients, we aimed at characterizing the immunopathology associated with each phenotype. Although defective T and B cell development is common to all phenotypes, patients with hypomorphic variants can generate T and B cells with signatures of immune dysregulation and produce autoantibodies to a broad range of self-antigens, including type I interferons.

Flexed-Neck Flexible Nasolaryngoscopy for Evaluation of the Subglottis and Trachea in Children.

Objective: Determine if a flexed-neck posture during flexible nasolaryngoscopy (FNL) improves visualization of the subglottis.

Study Design: Retrospective review of children undergoing FNL in the neutral (FNL) and flexed-neck (FN-FNL) positions.

Setting: Tertiary children's hospital.

Oversized cells activate global proteasome-mediated protein degradation to maintain cell size homeostasis.

Proliferating animal cells maintain a stable size distribution over generations despite fluctuations in cell growth and division size. Previously, we showed that cell size control involves both cell size checkpoints, which delay cell cycle progression in small cells, and size-dependent regulation of mass accumulation rates (Ginzberg et al., 2018).

Maternal Chronic Physical Conditions and Alcohol and Substance Use Disorders in the Preconception and Perinatal Periods.

Chronic physical conditions (CPC) and alcohol and substance use disorders (SUD) frequently co-occur, but this has not been examined perinatally. We explored the combined effects of CPC and prepregnancy SUD on perinatal SUD-related adverse events and outpatient care. This population-based study comprised 77,474 people with and 664,751 without CPC with a birth in Ontario, Canada, 2013-2020.

Functional genotype classification groups distinguish disease severity in recessive dystrophic epidermolysis bullosa.

Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic disorder due to pathogenic variants in the COL7A1 gene. In this study we determined the association between different categories of COL7A1 variants and clinical disease severity in 236 RDEB patients in North America. Published reports or in-silico predictions were used to assess the impact of pathogenic variants in COL7A1 on type VII collagen (C7) protein function.

Major ABO Incompatibility in Non-Myeloablative Hematopoietic Stem Cell Transplant for Sickle Cell Disease-Not an Insurmountable Obstacle.

With advances in conditioning strategies and graft-versus-host disease (GvHD) prevention, hematopoietic stem cell transplantation (HSCT) is a safe, curative treatment option for pediatric patients with sickle cell disease (SCD). However, donor options have been limited in non-myeloablative matched sibling donor (MSD) setting by excluding recipients with major ABO blood group incompatible donors due to concern of the risk of significant complications such as pure red cell aplasia (PRCA). We present three cases of successful HSCT with major ABO incompatibility with their donors, and discuss strategies to safely expand the donor pool to include these donors.

"Blindness" is not a contraindication for voretigene neparvovec-rzyl treatment-a review of 9 cases.

Objective: Biallelic RPE65 pathogenic variants may cause Leber congenital amaurosis (LCA). Voretigene neparvovec-rzyl (VN, Luxturna) is the only approved subretinal gene therapy that demonstrated benefit and safety. The eligibility criteria are vague and variable between centres.

Prenatal intervention for a large ureterocele in a male fetus causing lower urinary tract obstruction "Society of Fetal Urology Case Reports, Fall 2024".

A male fetus at 20 weeks of gestation presented with severe bilateral hydroureteronephrosis and distended bladder due to a large ureterocele. A percutaneous ureterocele puncture at 21+3 weeks aimed to decompress the ureterocele, unclog the lower urinary tract, and prevent renal damage. Post-puncture, the ureterocele decompressed, with improved left hydronephrosis and a normal right kidney.