Impact of high spinal anesthesia in pediatric congenital heart surgery on postoperative recovery: a retrospective propensity score-matched study.

Background: High spinal anesthesia (HSA) has been utilized in cardiac surgery; however, there is limited evidence on its impact on facilitating postoperative recovery. This study aimed to evaluate the impact of HSA in pediatric congenital heart surgery on postoperative recovery.

Methods: A single center, propensity score-matched retrospective cohort study was designed using data from pediatric patients under 18 years old, who underwent congenital heart surgeries classified as Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) score 3 or less.

Exposure to PCB52 (2,2',5,5'-tetrachlorobiphenyl) blunts induction of the gene for uncoupling protein 1 (UCP1) in white adipose.

Polychlorinated biphenyls (PCBs) are linked to cancer, learning disabilities, liver and cardiovascular disease, and diabetes. Older schools often contain high levels of PCBs, and inhalation is a major source of exposure. Technical PCB mixtures, called Aroclors, and individual dioxin-like PCBs impair adipocyte function, which can lead to type II diabetes.

Neurocardiology: Major mechanisms and effects.

Neurocardiology is a broad interdisciplinary specialty investigating how the cardiovascular and nervous systems interact. In this brief introductory review, we describe several key aspects of this interaction with specific attention to cardiovascular effects. The review introduces basic anatomy and discusses physiological mechanisms and effects that play crucial roles in the interaction of the cardiovascular and nervous systems, namely: the cardiac neuraxis, the taxonomy of the nervous system, integration of sensory input in the brainstem, influences of the autonomic nervous system (ANS) on heart and vasculature, the neural pathways and functioning of the arterial baroreflex, receptors and ANS effects in the walls of blood vessels, receptors and ANS effects in excitable cells in the heart, ANS effects on heart rate and sympathovagal balance, endo-epicardial inhomogeneity, ANS effects with a balanced vagal and sympathetic stimulation, sympathovagal interaction, arterial baroreflex, baroreflex sensitivity and heart rate variability, arrhythmias and the arterial baroreflex, the cardiopulmonary baroreflex, the exercise pressor reflex, exercise-recovery hysteresis, mental stress, cardiac-cardiac reflexes, the cardiac sympathetic afferent reflex (CSAR), and neuromodulation.

Evaluating a Child Presenting with Symptoms Concerning for Sjögren Disease: A Clinical Tool for Practice.

Background/purpose: Childhood Sjögren disease (cSjD) is a rare disease. There are no widely accepted diagnostic or classification criteria for cSjD. To fill this gap, members from CARRA Sjogren Workgroup and the International cSjD Workgroup created a clinical diagnostic algorithm.

Trigeminal nerve blocks attenuate afferent input to the puff-induced blink reflex in chronic orbital pain assessed by high-speed video.

Purpose: To assess the trigeminal blink reflex in chronic orbital pain and its modification by orbital anesthetic injections using a novel blink reflexometer.

Methods: The EyeStat (Generation 3, Blinktbi, Inc. Charleston, SC) is a device that triggers and analyzes the carbon dioxide puff-evoked trigeminal blink response.

Poor bone health in Duchenne muscular dystrophy: a multifactorial problem beyond corticosteroids and loss of ambulation.

Duchenne muscular dystrophy (DMD) is a progressive, fatal muscle wasting disease caused by X-linked mutations in the dystrophin gene. Alongside the characteristic muscle weakness, patients face a myriad of skeletal complications, including osteoporosis/osteopenia, high susceptibility to vertebral and long bone fractures, fat embolism post-fracture, scoliosis, and growth retardation. Those skeletal abnormalities significantly compromise quality of life and are sometimes life-threatening.

GLP-1R agonist promotes proliferation of neuroendocrine neoplasm cells expressing GLP-1 receptors.

Objectives: Semaglutide is a glucagon-like peptide 1 (GLP-1) analog that binds to GLP-1 receptors (GLP-1R) on beta-cells and neuronal cells and is used for treating type 2 diabetes and obesity. Insulin-secreting pancreatic neuroendocrine neoplasms have been reported to express high levels of GLP-1R protein, raising the possibility that GLP-1 receptor agonists could promote tumor growth. Our goal was to quantify GLP-1R expression levels in 6 neuroendocrine neoplasm cellular models and determine their proliferative response to semaglutide treatment.

Structure and assembly of the dystrophin glycoprotein complex.

The dystrophin glycoprotein complex (DGC) has a crucial role in maintaining cell membrane stability and integrity by connecting the intracellular cytoskeleton with the surrounding extracellular matrix. Dysfunction of dystrophin and its associated proteins results in muscular dystrophy, a disorder characterized by progressive muscle weakness and degeneration. Despite the important roles of the DGC in physiology and pathology, its structural details remain largely unknown, hindering a comprehensive understanding of its assembly and function.

Kidney Outcomes in Children Receiving Extracorporeal Membrane Oxygenation: A Single-Center Acute Cohort From 2009 to 2019, Followed to 2021.

Objectives: Long-term kidney outcomes after extracorporeal membrane oxygenation (ECMO) are little quantified and understood. We aimed to describe the frequency of kidney dysfunction screening during follow-up and the prevalence of long-term kidney disease.

Design: Retrospective cohort of pediatric ECMO patients with estimated glomerular filtration rate (eGFR) (mL/min/1.

Artificial intelligence-guided design of lipid nanoparticles for pulmonary gene therapy.

Ionizable lipids are a key component of lipid nanoparticles, the leading nonviral messenger RNA delivery technology. Here, to advance the identification of ionizable lipids beyond current methods, which rely on experimental screening and/or rational design, we introduce lipid optimization using neural networks, a deep-learning strategy for ionizable lipid design. We created a dataset of >9,000 lipid nanoparticle activity measurements and used it to train a directed message-passing neural network for prediction of nucleic acid delivery with diverse lipid structures.

Supplementary oxygen efficacy for chronic pulmonary disorders and exertion desaturation.

Introduction: Exertion-induced desaturation (EID) is a common complication of numerous pulmonary disorders and often treated with supplementary oxygen during exertion. We performed a systematic review and meta-analysis of randomised clinical trials (RCTs) to evaluate the efficacy of supplementary oxygen for EID in pulmonary disorders.

Material And Methods: Medline and Embase were systematically searched from July 2022 to June 2023 following PRISMA guidelines.

No Evidence of Early Developmental Delay in Juvenile-Onset Huntington's Disease Patients.

Background: Previous studies suggest that early developmental delay is a common feature of Juvenile-Onset Huntington's disease (JOHD), with highest incidence in those with very high CAG repeats (> 80). However, all reports of developmental delay in JOHD are exclusively based on retrospective review of medical charts. Comprehensive assessment of birth history metrics may provide better insight into the question of early life development in JOHD.

Heavy menstrual bleeding clinics for adolescents.

Heavy menstrual bleeding (HMB) is a common symptom in adolescence, often leading to significant disruptions in daily life, such as school absences, shame caused by the stigma surrounding menstruation, and symptoms from iron deficiency. Further, HMB may be the first and/or only sign of an underlying bleeding disorder. Navigating the symptoms, effects, and treatments of HMB during adolescence requires a collaborative approach between the patient, caregivers, and healthcare providers.

Glial cell deficits are a key feature of schizophrenia: implications for neuronal circuit maintenance and histological differentiation from classical neurodegeneration.

Dysfunctional glial cells play a pre-eminent role in schizophrenia pathophysiology. Post-mortem studies have provided evidence for significantly decreased glial cell numbers in different brain regions of individuals with schizophrenia. Reduced glial cell numbers are most pronounced in oligodendroglia, but reduced astrocyte cell densities have also been reported.

The ZFHX3 GGC Repeat Expansion Underlying Spinocerebellar Ataxia Type 4 has a Common Ancestral Founder.

Background: The identification of a heterozygous exonic GGC repeat expansion in ZFHX3 underlying spinocerebellar ataxia type 4 (SCA4) has solved a 25-year diagnostic conundrum. We used adaptive long-read sequencing to decipher the pathogenic expansion in the index Utah family and an unrelated family from Iowa of Swedish ancestry. Contemporaneous to our discovery, other groups identified the same repeat expansion in affected individuals from Utah, Sweden, and Germany, highlighting the current pivotal time for detection of novel repeat expansion disorders.

Predicting Nephrotoxic Acute Kidney Injury in Hospitalized Adults: A Machine Learning Algorithm.

Rationale And Objective: Acute kidney injury (AKI) is a common complication among hospitalized adults, but AKI prediction and prevention among adults has proved challenging. We used machine learning to update the nephrotoxic injury negated by just-in time action (NINJA), a pediatric program that predicts nephrotoxic AKI, to improve accuracy among adults.

Study Design: A retrospective cohort study.

Utilizing an Opportunistic Clinical Study and Population-Based Pharmacokinetic Models to Identify Rational Empiric Dosing Regimens for Piperacillin-Tazobactam in Critically Ill Patients.

Determining an effective dosing regimen for piperacillin-tazobactam in critically ill patients is challenging due to substantial pharmacokinetic variability caused by complex pathophysiological changes. To address this need, a prospective clinical study was conducted, which enrolled 112 critically ill patients and employed an opportunistic sampling strategy. Population modeling and simulation were performed to characterize the pharmacokinetics (PK) and probability of target attainment (PTA) of piperacillin-tazobactam under various dosing regimens.

Dynll1-PI31 Interaction Enhances Proteolysis via the Proteasome, Representing a Novel Therapeutic Target for INF2-Related FSGS.

Background: The p.Arg218Gln (R218Q) mutation in the inverted formin 2 (INF2) gene causes podocytopathy prone to focal segmental glomerulosclerosis (FSGS). This mutation disrupts the ability of INF2 to sequester DYNLL1, thus promoting dynein-mediated mistrafficking of the slit diaphragm protein, nephrin, to proteolytic pathways.

β-Blocker Use and Delayed Onset and Progression of Huntington Disease.

Importance: Huntington disease (HD) is characterized by motor, cognitive, and psychiatric decline. β-Blockers may play a therapeutic role by decreasing enhanced sympathetic tone in HD.

Objective: To evaluate the impact of β-blockers on the timing of motor diagnosis onset and progression of HD symptoms.