Pemphigus Vulgaris With Esophageal Involvement in an Atopic Child Successfully Treated With Dupilumab.

We report the case of a 3-year-old boy who was diagnosed with childhood pemphigus vulgaris having developed oral lesions, gastrointestinal symptoms with esophageal involvement, and failure to thrive. He had a markedly increased total serum IgE level and peripheral blood eosinophilia. The pemphigus was recalcitrant to conventional therapies and, based on the coexisting characteristics of Th2 immune deviation, he was treated with dupilumab and has had sustained clinical improvement since starting treatment.

Malaria parasites require a divergent heme oxygenase for apicoplast gene expression and biogenesis.

Malaria parasites have evolved unusual metabolic adaptations that specialize them for growth within heme-rich human erythrocytes. During blood-stage infection, parasites internalize and digest abundant host hemoglobin within the digestive vacuole. This massive catabolic process generates copious free heme, most of which is biomineralized into inert hemozoin.

The effect of body mass index at diagnosis on survival of patients with squamous cell head and neck carcinoma.

The aim of this study is to investigate the prognostic role of body mass index (BMI) on survival from head and neck cancer (HNC). We performed a pooled analysis of studies included in the International Head and Neck Cancer Epidemiology consortium. We used Cox proportional hazards models to estimate the adjusted hazard ratios (HR) for overall survival and HNC-specific survival, and we stratified the results according to cancer site.

Cardiac bridging integrator 1 gene therapy rescues chronic non-ischemic heart failure in minipigs.

Heart failure (HF) is a major cause of mortality and morbidity worldwide, yet with limited therapeutic options. Cardiac bridging integrator 1 (cBIN1), a cardiomyocyte transverse-tubule (t-tubule) scaffolding protein which organizes the calcium handling machinery, is transcriptionally reduced in HF and can be recovered for functional rescue in mice. Here we report that in human patients with HF with reduced ejection fraction (HFrEF), left ventricular cBIN1 levels linearly correlate with organ-level ventricular remodeling such as diastolic diameter.

Turner syndrome: fertility, familial clustering, and cancer risk.

Study Question: Is there an increased risk of reproductive or colon cancer in women with Turner syndrome and their family members?

Summary Answer: Our data suggest that there is an increased risk for sigmoid colon cancer in women with Turner syndrome and an increased prostate cancer risk in second- and third-degree male relatives.

What Is Known Already: Turner syndrome has been associated with lower risk of breast cancer, but increased risk of gonadoblastoma and colon cancer in some, but not all studies. There is also evidence for a genetic predisposition to sex chromosome aneuploidy, which may indicate a predisposition to Turner syndrome and the associated cancer risk in family members.

Impact of COVID-19 on newborn hearing screening (NBHS) and early hearing detection and intervention (EHDI) services: A statewide analysis of differences and policy implications.

Introduction: This study investigates the pandemic's impact on newborn hearing screening (NBHS) and access to hearing services for children in Utah. Specifically, it explores the differences in NBHS rates, diagnostic hearing testing, early intervention enrollment, and congenital cytomegalovirus (cCMV) screening before and during the pandemic.

Methods: Utilizing a comprehensive statewide Early Hearing Detection and Intervention (EHDI) database, we analyzed data from January 2017 to December 2021, excluding a 6-month period preceding March 16, 2020, to eliminate potential confounders related to pandemic onset.

Impact of clinician feedback reports on antibiotic use in children hospitalized with community-acquired pneumonia.

Background: Feedback reports summarizing clinician performance are effective tools for improving antibiotic use in the ambulatory setting, but the effectiveness of feedback reports in the hospital setting is unknown.

Methods: Quasi-experimental study conducted between December 2021 and November 2023 within a pediatric health system measuring the impact of clinician feedback reports delivered by email and reviewed in a monthly meeting on appropriate antibiotic use in children hospitalized with community-acquired pneumonia (CAP). We used an interrupted time series analysis (ITSA) to estimate the immediate change and change over time in the proportion of CAP encounters adherent to validated metrics of antibiotic choice and duration, then used Poisson regression to estimate intervention effect as a rate ratio (RR).

Creating a plasma coordination center to support COVID-19 outpatient trials across a national network of hospital blood banks.

Introduction: In response to the COVID-19 pandemic, we rapidly implemented a plasma coordination center, within two months, to support transfusion for two outpatient randomized controlled trials. The center design was based on an investigational drug services model and a Food and Drug Administration-compliant database to manage blood product inventory and trial safety.

Methods: A core investigational team adapted a cloud-based platform to randomize patient assignments and track inventory distribution of control plasma and high-titer COVID-19 convalescent plasma of different blood groups from 29 donor collection centers directly to blood banks serving 26 transfusion sites.

Contributions of Pediatric Hematology/Oncology to the Diagnosis, Treatment, and Cure of Acute Lymphoblastic Leukemia-Part 2b (Numbers 16 to 20).

This offering represents part 2b of a second set of 5 additional contributions of pediatric hematology/oncology to the diagnosis, treatment, and potential cure of precursor B-cell acute lymphoblastic leukemia. It contains numbers 16 to 20 and includes (16) allogeneic hematopoietic stem cell transplantation, newer immunotherapies including (17) blinatumomab, and (18) inotuzumab ozogamicin, (19) ploidy, and (20) creation of the "day hospital" to administer outpatient care to children with acute lymphoblastic leukemia and other cancers. These and the other reviewed contributions have had a significant role in improving the quality and duration of the lives of children, most of whom faced tragic and painful death back in the 1950s and 1960s.

Evaluation of patient and encounter decision aid interventions for atrial fibrillation: Baseline characteristics of the RED-AF study - A Randomized Controlled Trial.

Background: The Randomized Evaluation of Decision Support Interventions for Atrial Fibrillation (RED-AF) trial is a multi-site, randomized controlled clinical trial examining the effectiveness of a patient decision aid and an encounter decision aid in promoting shared decision-making (SDM) during a clinical encounter for patients with atrial fibrillation (AF). We sought to describe baseline characteristics of patients and clinicians in the trial and compare them to the demographics of the larger AF population. We also conducted an analysis of possible predictors of attrition rates at baseline, 6 and 12 months.

Comparison of Race-neutral Versus Race-specific Spirometry Equations for Evaluation of Child Asthma.

Rationale: Race-based estimates of pulmonary function in children could influence the evaluation of asthma in children from racial and ethnic minoritized backgrounds.

Objectives: To determine if race-neutral (GLI-Global) versus race-specific (GLI-Race-Specific) reference equations differentially impact spirometry evaluation of childhood asthma.

Methods: The analysis included 8,719 children aged 5 to <12 years from 27 cohorts across the United States grouped by parent-reported race and ethnicity.

Evaluations of State Medical Cannabis Programs in the USA: A Narrative Review.

Background: Medical cannabis (MC) use is increasing across the USA, with functional MC programs now operating in 38 states. While program policies and practices vary widely, little is known about whether and how states evaluate their programs. Better characterization of state MC program evaluation to date could inform states, program officials, and providers about best practices and provide a roadmap for future program evaluation.

Community Disparities in Out-of-Hospital Cardiac Arrest Prehospital Antiarrhythmic Practices.

Objectives: Antiarrhythmic administration is an important treatment for out-of-hospital cardiac arrest (OHCA) with a shockable rhythm, but a minimal amount is known about disparities in such antiarrhythmic practices. We sought to investigate the association between community race/ethnicity and prehospital antiarrhythmic administration for OHCA.

Methods: We conducted a retrospective study of a national prehospital database, National Emergency Medical Services Information System (NEMSIS), linked to Census data.

Oral Health, Inflammation, and Cardiometabolic Factors in the VA Million Veteran Program.

Introduction: Associations between cardiometabolic comorbidities and self-reported oral health (OH) are often underexplored in large biobank datasets. While these associations are unaffected by dental care access, they could be mediated by immune responses and inflammation.

Objectives: This study assessed the associations between cardiometabolic comorbidities and self-reported OH, periodontitis, and tooth loss using the (ICD) codes in participants from the U.

Receptor tyrosine kinase inhibition leads to regression of acral melanoma by targeting the tumor microenvironment.

Background: Acral melanoma (AM) is an aggressive melanoma variant that arises from palmar, plantar, and nail unit melanocytes. Compared to non-acral cutaneous melanoma (CM), AM is biologically distinct, has an equal incidence across genetic ancestries, typically presents in advanced stage disease, is less responsive to therapy, and has an overall worse prognosis.

Methods: An independent analysis of published sequencing data was performed to evaluate the frequency of receptor tyrosine kinase (RTK) ligands and adapter protein gene variants and expression.

Bilateral Shoulder Infections and Delayed Recognition of Spinal Discitis in an Adult Male With Mild COVID-19 and Staphylococcus aureus Bacteremia: Case Report and Literature Review.

Coronavirus disease 2019 (COVID-19) has been associated with numerous complications beyond the respiratory tract, including spinal and joint co-infections and secondary infections. However, we could not locate any reported cases of bilateral shoulder infections with additional spinal infections associated with mild (like our patient) COVID-19 or with more severe cases of COVID-19. We report the case of a healthy 62-year-old male who presented with mild COVID-19 and concurrent methicillin-sensitive (MSSA) bacteremia associated with multiple musculoskeletal sites of deep-seated infection.