Clinical Features and Outcomes of Pediatric -Related Dilated Cardiomyopathy.

Background: Although genetic variants in are the most frequent cause of pediatric genetic dilated cardiomyopathy (DCM), there are no studies available describing this entity. We sought to describe clinical features, analyze variant location, and explore predictors of bad prognosis in pediatric -related DCM.

Methods And Results: We evaluated clinical records from 44 patients (24 men; median age at diagnosis, 0.

Longitudinal Assessment of T1 Mapping Trends Disease in Pediatric Patients With Heart Transplant.

Background: Surveillance for heart transplant rejection by endomyocardial biopsy is invasive and may yield false negatives. T1 and T2 mapping from cardiac magnetic resonance can demonstrate elevations with rejection. We sought to evaluate longitudinal changes in T1 and T2 mapping in pediatric patients with heart transplant.

Demographic and socioeconomic characteristics of patients diagnosed with autism through the Rapid Interactive screening Test for Autism in Toddlers.

We evaluated the integration of the Rapid Interactive screening Test for Autism in Toddlers (RITA-T) model in a community, comparing autism spectrum disorder (ASD) toddlers' demographic and socioeconomic characteristics. Of 394 ASD toddlers, 323 were screened with RITA-T. Those screened were from more deprived areas, traveled farther and were diagnosed earlier.

Is there a role for extracorporeal membrane oxygenation in children with acute liver failure? A retrospective analysis of the Extracorporeal Life Support Organization Registry.

Unlabelled: Data describing the use of extracorporeal membrane oxygenation (ECMO) in pediatric acute liver failure (PALF) are scarce. Thus, we aimed to describe the use of ECMO in patients with PALF using the data from the Extracorporeal Life Support Organization (ELSO) Registry between January 1, 2010, to December 31, 2022. We described patients' characteristics at ECMO initiation, outcome, and factors associated with mortality.

From defense to dysfunction: Autophagy's dual role in disease pathophysiology.

Autophagy is a fundamental pillar of cellular resilience, indispensable for maintaining cellular health and vitality. It coordinates the meticulous breakdown of cytoplasmic macromolecules as a guardian of cell metabolism, genomic integrity, and survival. In the complex play of biological warfare, autophagy emerges as a firm defender, bravely confronting various pathogenic, infectious, and cancerous adversaries.

Fight or flee, a vital choice for .

is a leading cause of healthcare-associated infections, causing billions of economic losses every year. Its symptoms range from mild diarrhea to life-threatening damage to the colon. Transmission and recurrence of infection (CDI) are mediated by the metabolically dormant spores, while the virulence of is mainly due to the two large clostridial toxins, TcdA and TcdB.

Extracorporeal Membrane Oxygenation After Liver Transplant in Children: A Review of the ELSO Registry.

There are minimal data describing use of extracorporeal membrane oxygenation (ECMO) in pediatric patient after a liver transplantation (LT). This study aimed at describing ECMO run in this specific situation using the data from Extracorporeal Life Support Organization (ELSO) Registry between January 1, 2010, to December 31, 2022. We described patients' characteristics at ECMO initiation, outcome and mortality risk factors.

Social, economic, and health indexes affecting epidemiology of preterm birth: A cross-sectional study.

Background And Aim: Preterm birth (PTB) accompanies with morbidities and mortality among newborns. This study aimed to show that different factors such as economic (adjusted net national income and gross domestic product [GDP] per capita), human developmental (human developmental index), and health (overall health performance, pregnancy prenatal care rate, and modeled estimated maternal mortality rate) indexes might influence the prevalence of PTB.

Methods: To this, the top 10 countries with the highest and lowest prevalence of PTB were extracted from the Global Burden of Disease report for PTB.

A novel RNF125 variant associated with Tenorio syndrome alters ubiquitin chain binding.

A key signalling pathway required for clearance of viruses from host cells relies on the receptor protein, retinoic acid-inducible gene I (RIG-I). The activity of RIG-I is tightly controlled, and once bound to viral dsRNA, addition of lysine 63-linked ubiquitin chains activates signalling. Meanwhile, the addition of lysine 48-linked ubiquitin chains to RIG-I is required to terminate signalling when the infection has been resolved.

Storytelling workshop to encourage stakeholder engagement with the Global Initiative for Childhood Cancer.

Storytelling can enhance stakeholder engagement and support the implementation of the World Health Organization and Pan American Health Organization's (PAHO) Global Initiative for Childhood Cancer, which aims to improve care globally for children with cancer. The Initiative aligns with the United Nations Sustainable Development Goals, addressing health, education, inequalities and international collaboration. This report describes the design and implementation of a workshop that used storytelling through film to encourage stakeholders in national cancer control plans to engage with the Initiative in its focal countries in Central America, the Dominican Republic and Haiti.

Transcatheter Ductal Stents Versus Surgical Systemic-Pulmonary Artery Shunts in Neonates With Congenital Heart Disease With Ductal-Dependent Pulmonary Blood Flow: Trends and Associated Outcomes From the Pediatric Health Information System Database.

Background Surgical systemic-to-pulmonary artery shunts have been the standard approach to establish stable pulmonary blood flow in neonates with congenital heart disease with ductal-dependent pulmonary blood flow. More recently, transcatheter ductal stents have been performed as an alternative, less invasive intervention. We aimed to characterize trends in the utilization of surgical shunts versus ductal stents and compare associated outcomes.

Mendelian Disorders in an Interstitial Cystitis/Bladder Pain Syndrome Cohort.

Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic pain disorder causing symptoms of urinary frequency, urgency, and bladder discomfort or pain. Although this condition affects a large population, little is known about its etiology. Genetic analyses of whole exome sequencing are performed on 109 individuals with IC/BPS.

Platelets in the neonate: Not just a small adult.

Neonates, particularly those born preterm, have a high incidence of thrombocytopenia and bleeding, most commonly in the brain. Because of this, it has historically been accepted that neonates should be transfused at higher platelet counts than older children or adults, to decrease their bleeding risk. However, a number of observational studies and a recent large, randomized trial found a higher incidence of bleeding and mortality in neonates who received more platelet transfusions.

Conceptualizing intubation sharing: A descriptive qualitative study of videolaryngoscopy for pediatric emergency airway management.

Background: This study characterizes clinical and educational practices around the use of videolaryngoscopy in pediatric emergency airway management through qualitative exploration.

Methods: This is a descriptive qualitative study using semi-structured interviews of emergency medicine physicians. Physicians were selected by theoretical sampling from urban, tertiary care pediatric hospitals across the United States until theoretical data saturation was achieved.

Magnetically Active Cardiac Patches as an Untethered, Non-Blood Contacting Ventricular Assist Device.

Patients suffering from heart failure often require circulatory support using ventricular assist devices (VADs). However, most existing VADs provide nonpulsatile flow, involve direct contact between the blood flow and the device's lumen and moving components, and require a driveline to connect to an external power source. These design features often lead to complications such as gastrointestinal bleeding, device thrombosis, and driveline infections.

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia.

Medium-Term Complications Associated With Coronary Artery Aneurysms After Kawasaki Disease: A Study From the International Kawasaki Disease Registry.

Background Coronary artery aneurysms (CAAs) may occur after Kawasaki disease (KD) and lead to important morbidity and mortality. As CAA in patients with KD are rare and heterogeneous lesions, prognostication and risk stratification are difficult. We sought to derive the cumulative risk and associated factors for cardiovascular complications in patients with CAAs after KD.

The re-occurrence of cardiomyopathy in propionic acidemia after liver transplantation.

Cardiomyopathy is a frequent complication of propionic acidemia (PA). It is often fatal, and its occurrence is largely independent of classic metabolic treatment modalities. Liver transplantation (LT) is a treatment option for severe PA as the liver plays a vital role in metabolism of the precursors that accumulate in patients with PA.

Patient and Institutional Characteristics Influence the Decision to Use Extracorporeal Cardiopulmonary Resuscitation for In-Hospital Cardiac Arrest.

BACKGROUND Outcomes from extracorporeal cardiopulmonary resuscitation (ECPR) are felt to be influenced by selective use, but the characteristics of those receiving ECPR are undefined. We demonstrate the relationship between individual patient and hospital characteristics and the probability of ECPR use. METHODS AND RESULTS We performed an observational analysis of adult inpatient cardiac arrests in the United States from 2000 to 2018 reported to the American Heart Association's Get With The Guidelines-Resuscitation registry restricted to hospitals that provided ECPR.