Amyloidosis in Childhood: A Review of Clinical Features and Comparison with Adult Forms.

Amyloidosis is a rare multisystem disorder characterized by extracellular accumulation of insoluble fibrils in various organs and tissues. The most common subtype in the pediatric population is systemic reactive amyloidosis, typically developing secondary to chronic inflammatory conditions and resulting in deposition of serum amyloid A protein in association with apolipoprotein HDL3. Clinical presentation is highly variable and is mostly influenced by specific organs involved, precursor protein type, and extent of amyloid deposition, often closely reflecting clinical features of the underlying disease.

Insulin Delivery Technology for Treatment of Infants with Neonatal Diabetes Mellitus: A Systematic Review.

Neonatal diabetes mellitus is a rare disorder of glucose metabolism with onset within the first 6 months of life. The initial treatment is based on insulin infusion. The technologies for diabetes treatment can be very helpful, even if guidelines are still lacking.

Community-based diet and obesity-related policy, system, and environmental interventions for obesity prevention during the first 1000 days: A scoping review.

Community-based policy, systems, and environmental interventions have the potential to reduce modifiable risk factors for obesity early in life. The purpose of this scoping review was to characterize the breadth, generalizability, and methodological quality of community-based diet and obesity-related policy, system, and environmental interventions during the first 1000 days of life, from pregnancy to 24 months of age. Eight databases were searched, and 83 studies (122 references) were included.

Temperature and ST-segment morphology remote monitoring: new perspectives for implantable cardiac monitors in Brugada syndrome.

Introduction: Patients with Brugada syndrome (BrS) face an increased risk of ventricular arrhythmias and sudden cardiac death. Implantable cardiac monitors (ICMs) have emerged as effective tools for detecting arrhythmias in BrS. Technological advancements, including temperature sensors and improved subcutaneous electrocardiogram (subECG) signal quality, hold promise for further enhancing their utility in this population.

Predicting ventricular arrhythmia inducibility in ajmaline-induced Brugada type I pattern: Validation of the dST-Tiso interval.

Introduction: The dST-Tiso is a newly proposed electrocardiographic (ECG) marker during Brugada (BrS) type I pattern, that predicts the likelihood of ventricular arrhythmia (VA) inducibility in patients with ajmaline-induced pattern. The objective of this study was to validate the effectiveness of this criterion using an independent data set.

Methods: Consecutive patients exhibiting a BrS type I ECG pattern following ajmaline administration underwent programmed ventricular stimulation (PVS).

Non-invasive cardiac activation mapping and identification of severity of epicardial substrate in Brugada Syndrome: a case report.

Introduction: It has recently been shown that electrocardiographic imaging (ECGi) can be employed in individuals undergoing an ajmaline test who have Brugada Syndrome (BrS), to evaluate the extent of substrate-involved arrhythmia in the right ventricular overflow tract (RVOT). For the first time, we stratify the risk of sudden cardiac death (SCD) in BrS during ajmaline testing using the dST-Tiso interval (a robust predictor of the inducibility of ventricular arrhythmias (VAs) in the presence of drug-induced BrS type-1 pattern) in combination with ECGi technology.

Case Presentation: We studied a 48-year-old man with BrS ECG type-2 pattern and presence of J-wave without a family history of SCD but with a previous syncope.

Late-stage Attentional Bias towards Food Cues Varies According to Weight Status.

The Current food environment has become increasingly obesogenic, with rates of obesity and related conditions continually rising. Advertisements for energy-dense foods are abundant and promote unhealthy eating behaviors by capitalizing on one's attentional bias towards food cues, a cognitive process resulting from the sensitization of highly reinforcing food. A heightened awareness towards food cues may promote overconsumption of energy-dense foods.

Investigating Deep Sedation With Intravenous Ketamine in Spontaneous Respiration During Pulsed-Field Ablation.

Objectives: The authors report their experience of a protocol for deep sedation with ketamine in spontaneous respiration during the pulsed-field ablation (PFA) of atrial fibrillation (AF).

Design: Observational, prospective, nonrandomized fashion.

Setting: Single-center hospitalized patients.

Sedation strategies for pulsed-field ablation of atrial fibrillation: focus on deep sedation with intravenous ketamine in spontaneous respiration.

Aims: A standardized sedation protocol for pulsed-field ablation (PFA) of atrial fibrillation (AF) through irreversible cellular electroporation has not been well established. We report our experience of a protocol for deep sedation with ketamine in spontaneous respiration during the PFA of AF.

Methods And Results: All consecutive patients undergoing PFA for AF at our center were included.

Two Decades of Wildlife Pathogen Surveillance: Case Study of and Its Wild Reservoir .

The Costa Rican pygmy rice rat () is the primary reservoir of (CHOV), the causal agent of hantavirus disease, pulmonary syndrome, and fever in humans in Panama. Since the emergence of CHOV in early 2000, we have systematically sampled and archived rodents from >150 sites across Panama to establish a baseline understanding of the host and virus, producing a permanent archive of holistic specimens that we are now probing in greater detail. We summarize these collections and explore preliminary habitat/virus associations to guide future wildlife surveillance and public health efforts related to CHOV and other zoonotic pathogens.

Lenalidomide maintenance fails to overcome the unfavourable prognosis of low NK-cell counts in rituximab-chemotherapy responsive elderly DLBCL patients: A LYSA group study.

Low baseline NK-cell counts (NKCCs) in patients with diffuse large B-cell lymphoma (DLBCL) are associated with a poor prognosis. The REMARC phase III trial (NCT01122472) showed that lenalidomide maintenance prolonged PFS in rituximab-chemotherapy responders. We conducted a REMARC ancillary study analysing the impact of lenalidomide maintenance on the prognostic value of low NKCCs.

Assessment of the Hawthorne effect during central venous catheter manipulation.

Objectives: To describe the compliance to the practices of hand hygiene and hub disinfection before manipulation of the central venous catheter in two moments: before and after educational intervention. Adherence to hand hygiene was assessed with two methods: direct observation and video camera.

Methods: Before and after study conducted with the nursing team in an adult intensive care unit, in São Paulo, Brazil, including 180 observations using video cameras and direct observation.

Rhythm- or rate-control strategies according to 4S-AF characterization scheme and long-term outcomes in atrial fibrillation patients: the FAMo (Fibrillazione Atriale in Modena) cohort.

The 4S-AF scheme [Stroke risk, Symptom severity, Severity of atrial fibrillation (AF) burden, Substrate severity] was recently proposed to characterize AF patients. In this post hoc analysis we evaluated the agreement between the therapeutic strategy (rate or rhythm control, respectively), as suggested by the 4S-AF scheme, and the actual strategy followed in a patients cohort. Outcomes of interest were as follows: all-cause death, a composite of all-cause death/any thromboembolism/acute coronary syndrome, and a composite of all-cause death, any thrombotic/ischemic event, and major bleeding (net clinical outcome).

Key characteristics for effective acute pulmonary vein isolation when using a novel cryoballoon technology: insights from the CHARISMA registry.

Purpose: A new cryoballoon (CB) technology (POLARx™; Boston Scientific) for pulmonary vein (PV) isolation in patients with atrial fibrillation (AF) has recently been introduced. The aim of this study was to evaluate procedural and biophysical parameters resulting in acute PV isolation when using this new CB.

Methods: We assessed the first 69 consecutive patients indicated for AF ablation who underwent PV isolation by means of a novel CB system.

dST-Tiso Interval, a Novel Electrocardiographic Marker of Ventricular Arrhythmia Inducibility in Individuals With Ajmaline-Induced Brugada Type I Pattern.

The aim of this study was to investigate the reliability of a novel electrocardiographic (ECG) marker in predicting ventricular arrhythmia (VA) inducibility in individuals with drug-induced Brugada syndrome (BrS) type I pattern. Consecutive patients with drug-induced type I BrS pattern underwent programmed ventricular stimulation (PVS) and, according to their response, were divided into 2 groups. Clinical characteristics and 12-lead ECG intervals before and after ajmaline infusion were compared between the 2 groups.

Disentangling environmental drivers of circadian metabolism in desert-adapted mice.

Metabolism is a complex phenotype shaped by natural environmental rhythms, as well as behavioral, morphological and physiological adaptations. Metabolism has been historically studied under constant environmental conditions, but new methods of continuous metabolic phenotyping now offer a window into organismal responses to dynamic environments, and enable identification of abiotic controls and the timing of physiological responses relative to environmental change. We used indirect calorimetry to characterize metabolic phenotypes of the desert-adapted cactus mouse (Peromyscus eremicus) in response to variable environmental conditions that mimic their native environment versus those recorded under constant warm and constant cool conditions, with a constant photoperiod and full access to resources.

Establishing Interrater Reliability Within the Advanced Practice Nurse Peer Review Process.

Background And Purpose: The Advanced Practice Nurse (APN) Council refined the APN peer review to an objective, data-driven process. The purpose of the study was to assess the interrater reliability of APN peer reviews using the APN Rubric based on Hamric, Spross & Hanson's Model of Advanced Practice Nursing.

Methods: A quantitative single-site study with a convenience sample of 80 APN Portfolios.

Leveraging natural history biorepositories as a global, decentralized, pathogen surveillance network.

The Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic reveals a major gap in global biosecurity infrastructure: a lack of publicly available biological samples representative across space, time, and taxonomic diversity. The shortfall, in this case for vertebrates, prevents accurate and rapid identification and monitoring of emerging pathogens and their reservoir host(s) and precludes extended investigation of ecological, evolutionary, and environmental associations that lead to human infection or spillover. Natural history museum biorepositories form the backbone of a critically needed, decentralized, global network for zoonotic pathogen surveillance, yet this infrastructure remains marginally developed, underutilized, underfunded, and disconnected from public health initiatives.