A novel genetic variant associated with progressive familial intrahepatic cholestasis type 3: A case series.

Progressive familial intrahepatic cholestasis type 3 (PFIC-3) is a rare disorder characterized by chronic cholestasis usually progressing to end-stage liver disease (ESLD) within the first two decades of life. PFIC-3 is caused by pathogenic genetic variants of the ATP-binding cassette 4 (ABCB4) gene with variable inheritance; the most common is autosomal recessive. We present two cases of PFIC-3 with genetic testing confirming a novel genetic variant in ABCB4 with homozygous genotype c.

Discovering novel inhibitors of RfaH from Klebsiella pneumoniae to combat antimicrobial resistance.

RfaH is a crucial protein involved in anti-termination of transcription, which is necessary for spreading virulence in certain types of bacteria, such as Klebsiella pneumoniae and Escherichia coli. RfaH works by interacting directly with RNA polymerase and ribosomes, which activates the production of certain components needed for the bacteria's survival. Targeting RfaH offers a novel approach to hindering bacterial transcription and virulence.

Atrial Fibrillation in Young Patients: Insights From the National Inpatient Sample.

Background: The burden of atrial fibrillation (AF) hospitalizations in young patients is not well characterized. We aimed to study the burden, comorbidities, outcomes, and resource utilization of AF hospitalizations in young patients.

Methods: We conducted a retrospective analysis of all primary AF hospitalizations in patients 18-45 years of age using the National Inpatient Sample data from January 1, 2008 to December 31, 2019.

Mortality in Recipients of Durable Left Ventricular Assist Devices Undergoing Ventricular Tachycardia Ablation.

Background: Left ventricular assist device (LVAD) recipients have a higher incidence of ventricular tachycardia (VT). However, the role of VT ablation in this population is not well-established.

Objectives: This single-center retrospective cohort study sought to examine the impact of post-LVAD implant VT ablation on survival.

Inequities in atrial fibrillation trials: An analysis of participant race, ethnicity, and sex over time.

Background: Despite the importance of racial and ethnic representation in clinical trials, limited data exist about the enrollment trends of these groups in atrial fibrillation (AF) trials over time.

Objective: The purpose of this study was to examine the characteristics of contemporary AF clinical trials and to evaluate their association with race and ethnicity over time.

Methods: We performed a systematic search of all completed AF trials registered in ClinicalTrials.

Shear bond strength between conventional composite resin and alkasite-based restoration used in sandwich technique: An study.

Aim: The success of layered restorations necessitates the utilization of an improved restorative material compatible with composite restorations. Therefore, in this line of research, the strength of adhesion of conventional resin-based dental composite to different filling materials was tested.

Materials And Methods: Conventional composite resin was bonded to four restorative materials (Group I: conventional glass ionomer cement (GIC), Group II: resin-modified glass ionomer cement, Group III: flowable composite, and Group IV: Cention-N) received no surface treatment (Subgroup A: control), sandblasting using 50-µm aluminum oxide particles (Subgroup B), sandblasting and resin adhesive (Subgroup C), acid etch and resin adhesive (Subgroup D), or self-etch resin adhesive (Subgroup E).

Utilization and perspectives of weight loss medications in pediatric metabolic dysfunction-associated steatotic liver disease.

Metabolic dysfunction-associated steatotic liver disease (MASLD) is increasing globally in pediatric populations. Currently, MASLD management primarily relies on lifestyle interventions, which pose challenges in sustaining long-term weight loss. This study investigated the use of weight loss medications in MASLD care through an international survey of 166 pediatric gastroenterologists and hepatologists.

Characteristics of contemporary atrial fibrillation clinical trials and their association with industry sponsorship.

Background: Industry sponsorship is an important source of funding for atrial fibrillation (AF) clinical trials, the implications of which have not been analyzed.

Objective: The purpose of this study was to determine the characteristics of contemporary AF clinical trials and to evaluate their association with funding source.

Methods: We systematically assessed all completed AF trials registered in the ClinicalTrials.

Anti-spike antibody durability after SARS-CoV-2 vaccination in adolescent solid organ transplant recipients.

Background: Adolescent solid organ transplant recipients (aSOTRs) who received three doses of the COVID-19 mRNA vaccine experience high seroconversion rates and antibody persistence for up to 3 months. Long-term antibody durability beyond this timeframe following three doses of the SARS-CoV-2 mRNA vaccine remains unknown. We describe antibody responses 6 months following the third vaccine dose (D3) of the BNT162b2 mRNA vaccination among aSOTRs.

Omicron Infections in Vaccinated Pediatric Solid Organ Transplant Recipients.

SARS-CoV-2 infection during the Omicron period was frequent amongst a cohort of vaccinated pediatric solid organ transplant recipients (pSOTRs) despite robust anti-receptor-binding domain (anti-RBD) antibody response, suggesting poor neutralizing capacity against Omicron subvariants. Breakthrough infections among pSOTRs were overall limited in severity.

Immunosuppression minimization is safe and associated with good long-term success in pediatric recipients of liver transplant.

Immunosuppression reduction after liver transplant is an important strategy to mitigate long-term medication side effects. We describe our center's experience with immunosuppression minimization to once-daily calcineurin inhibitor dosing. Success was defined as continuing daily calcineurin inhibitor monotherapy with normal transaminases and no rejection.

Exploring polysaccharide and protein-enriched decellularized matrix scaffolds for tendon and ligament repair: A review.

Tissue engineering (TE) has become a primary research topic for the treatment of diseased or damaged tendon/ligament (T/L) tissue. T/L injuries pose a severe clinical burden worldwide, necessitating the development of effective strategies for T/L repair and tissue regeneration. TE has emerged as a promising strategy for restoring T/L function using decellularized extracellular matrix (dECM)-based scaffolds.

Incidence, Risk Score Performance, and In-Hospital Outcomes of Postoperative Atrial Fibrillation After Cardiac Surgery.

Background: Postoperative atrial fibrillation (POAF) frequently complicates cardiac surgery. Predicting POAF can guide interventions to prevent its onset. This study assessed the incidence, risk factors, and related adverse outcomes of POAF after cardiac surgery.

Frailty Status Modifies the Efficacy of ICD Therapy for Primary Prevention Among Patients With HF.

Background: Implantable cardioverter-defibrillator (ICD) therapy is recommended to reduce mortality risk in patients with heart failure with reduced ejection fraction (HFrEF). Frailty is common among patients with HFrEF and is associated with increased mortality risk. Whether the therapeutic efficacy of ICD is consistent among frail and nonfrail patients with HFrEF remains unclear.

Clinicopathologic Features of Severe Acute Hepatitis Associated With Adenovirus Infection in Children.

A recent increase in reports of severe acute hepatitis of unknown etiology in children is under investigation. Although adenovirus has been frequently detected, its role remains unclear, and systematic histopathologic analysis is lacking. We conducted a retrospective study of 11 children hospitalized between October 2021 and May 2022 with unexplained acute hepatitis and concurrent adenovirus infection.

Risk Stratification in Patients Who Underwent Percutaneous Left Atrial Appendage Occlusion.

Left atrial appendage occlusion (LAAO) is effective in preventing thromboembolism. Risk stratification tools could help identify patients at risk for early mortality after LAAO. In this study, we validated and recalibrated a clinical risk score (CRS) to predict risk of all-cause mortality after LAAO.

Living donor liver transplantation: A multi-disciplinary collaboration towards growth, consensus, and a change in culture.

Introduction: Living donor liver transplantation (LDLT) reduces liver transplant waitlist mortality and provides excellent long-term outcomes for persons with end stage liver disease. Yet, utilization of LDLT has been limited in the United States (US).

Methods: In October 2021, the American Society of Transplantation held a consensus conference to identify important barriers to broader expansion of LDLT in the US, including data gaps, and make recommendations for impactful and feasible mitigation strategies to overcome these barriers.