Management of iron overload: lessons from transfusion-dependent hemoglobinopathies.

Before the advent of effective iron chelation, death from iron-induced cardiomyopathy and endocrine failure occurred in the second decade in patients with thalassemia major, and this experience has driven expectation of poor outcomes and caused anxiety in all disorders associated with iron loading to this day. To be clear, severe iron overload still causes significant morbidity and mortality in many parts of the world, but current understanding of iron metabolism, noninvasive monitoring of organ-specific iron loading in humans, and effective iron chelators have dramatically reduced morbidity of iron overload. Furthermore, clinical experience in hemoglobinopathies supports iron biology learned from animal studies and identifies common concepts in the biology of iron toxicity that inform the management of iron toxicity in several human disorders.

Kinetic Modeling of In Vivo K Distribution and Fluxes with Stable K Isotopes: Effects of Dietary K Restriction.

Maintaining extracellular potassium (K) within narrow limits, critical for membrane potential and excitability, is accomplished through the internal redistribution of K between extracellular fluid (ECF) and intracellular fluid (ICF) in concert with the regulation of renal K output to balance K intake. Here we present evidence from high-precision analyses of stable K isotopes in rats maintained on a control diet that the tissues and organs involved in the internal redistribution of K differ in their speed of K exchange with ECF and can be grouped into those that exchange K with ECF either rapidly or more slowly ("fast" and "slow" pools). After 10 days of K restriction, a compartmental analysis indicates that the sizes of the ICF K pools decreased but that this decrease in ICF K pools was not homogeneous, rather occurring only in the slow pool (15% decrease, < 0.

Cerebrospinal Fluid Shunt Infections.

Cerebrospinal fluid (CSF) shunt infections are a particularly challenging clinical problem. This review article addresses epidemiology and microbiology of CSF shunt infections. Clinical care is reviewed in detail, including recent guidelines and systematic review articles.

Backtracking childhood leukaemia to birth: A battle of addition.

Paediatric leukaemia has a long tail of driver mutations each of which must be 'backtracked' to samples taken at birth to identify the prenatal origin of a subtype. Presently, Bardini et al. describe the first successful backtracking of an NUTM1 rearrangement, which sheds light on the biology of this particular alteration.

A prospective longitudinal investigation of the trajectory of obsessive-compulsive symptoms during adolescence.

Obsessive-compulsive symptoms (OCS) increase with age during childhood and adolescence, and subthreshold OCS in childhood associate with a higher probability of obsessive-compulsive disorder (OCD) diagnosis in adulthood. Additionally, average age of onset for OCD is in adolescence, with the majority of OCD cases emerging by early adulthood. Despite these trends, the specific course of OCS development in adolescence is relatively unknown.

Status of LGBTQ+ Inclusion Using a Multi-Institution Assessment of US Radiology Residencies.

Introduction: An inclusive residency program is crucial to the recruitment and retention of competitive and diverse applicants. The radiology lesbian, gay, bisexual, transgender, queer or questioning, or another diverse gender identity (LGBTQ+) inclusion audit was published in 2022, which provided a road map for assessing the inclusivity of a program's policies, facilities, culture, and engagement. In this multi-institutional trial, we detail the results of the LGBTQ+ inclusion audit for nine US radiology residency programs.

Contributions of each of the BAFF receptors to the lymphocyte profiles in C57BL/6 mice.

BAFF, a vital B cell survival and differentiation factor, has three receptors: B-cell maturation antigen (BCMA), transmembrane activator and CAML interactor (TACI) and BR3. Although B cells are greatly reduced in B6.Baff (which harbour no BAFF) and B6.

Randomized pilot trial of cell phone support to improve medication adherence among adolescents and young adults with chronic health conditions.

Introduction: Adolescents and young adults (AYA) living with chronic medical conditions often struggle to develop medication adherence skills. This pilot trial evaluated the impact of a mobile health coaching intervention, Cell Phone Support (CPS), on medication adherence.

Methods: Interventions in this randomized trial were CPS delivered by phone calls (CPS-C), CPS delivered by text messages (CPS-T), or automated text message reminders (ATR).

Effects of a fruit-ice combination flavor on appeal and sensory experience of vaping and moderation by preexisting e-cigarette flavor preference.

E-cigarettes with ice flavors, which are products with a cooling agent added to a characterizing flavor (e.g., grape-ice), are widely sold.

Perinatal arterial ischemic stroke diagnosed in infants receiving therapeutic hypothermia for hypoxic-ischemic encephalopathy.

Background: Both perinatal arterial ischemic stroke (PAIS) and hypoxic-ischemic encephalopathy (HIE) can present with neonatal encephalopathy. We hypothesized that among infants undergoing therapeutic hypothermia, presence of PAIS is associated with a higher risk of seizures and a lower risk of persistent encephalopathy after rewarming.

Methods: We studied 473 infants with moderate or severe HIE enrolled in the HEAL Trial who received a brain MRI.

Human-centered design approach to building a transition readiness mHealth intervention for early adolescents.

Objective: Mobile health (mHealth) interventions may be an efficacious strategy for promoting health behaviors among pediatric populations, but their success at the implementation stage has proven challenging. The purpose of this article is to provide a blueprint for using human-centered design (HCD) methods to maximize the potential for implementation, by sharing the example of a youth-, family-, and clinician-engaged process of creating an mHealth intervention aimed at promoting healthcare transition readiness.

Method: Following HCD methods in partnership with three advisory councils, we conducted semistructured interviews with 13- to 15-year-old patients and their caregivers in two phases.

A Review of COVID-19 Hotels in New York City During the Early 2020 Outbreak: An Alternative Care Model.

New York City (NYC) was the epicenter of the early US COVID-19 pandemic. From March to May 2020, overburdened healthcare centers precipitated an emergent need for non-traditional facilities to meet patient care demands. Given travel restrictions and NYC's underutilized tourist infrastructure, hotels were available to support emergency response needs.

Association of extreme hyperoxemic events and mortality in pediatric critical care: an observational cohort study.

Objective: Our aim was to confirm whether extreme hyperoxemic events had been associated with excess mortality in our diverse critical care population.

Methods: Retrospective analysis of 9 years of data collected in the pediatric and cardiothoracic ICUs in Children's Hospital Los Angeles was performed. The analysis was limited to those mechanically ventilated for at least 24 h, with at least 1 arterial blood gas measurement.

T cell Dissimilarities in B Cell Activating Factor-Deficient Versus B Cell Activating Factor Receptor 3-Deficient Systemic Lupus Erythematosus-Prone NZM 2328 Mice as Contributors to Their Divergent Clinical Outcomes.

Objective: We assessed the contributions of B cell and T cell subsets to the disparate clinical outcomes in NZM.Baff and NZM.Br3 mice.

Ethics of abortion in transplantation.

Purpose Of Review: The controversial medical procedure of abortion is the subject of this article. It argues that for transplant patients, including recipients, abortion is ethical.

Recent Findings: In June 2022, the United States Supreme Court overturned the long-standing decision of Roe v.

Epigenomic Dysregulation in Youth Vapers: Implications for Disease Risk Assessment.

Despite the ongoing epidemic of youth vaping, the long-term health consequences of electronic cigarette use are largely unknown. We report the effects of vaping smoking on the oral cell methylome of healthy young vapers and smokers relative to non-users. Whereas vapers and smokers differ in number of differentially methylated regions (DMRs) (831 2,863), they share striking similarities in the distribution and patterns of DNA methylation, chromatin states, transcription factor binding motifs, and pathways.

Ethical considerations in lung re-transplantation.

Purpose Of Review: Patients undergoing re-transplantation constitute a growing proportion of annual lung transplants. It is necessary to consider ethical considerations of re-transplantation in parallel with clinical progress.

Recent Findings: Most clinical data demonstrate patients undergoing re-transplantation have worse survival outcomes; however, there is limited discussion of the ethical principles surrounding re-transplantation.