Clinical Care for Cardiovascular Disease in Patients With Williams-Beuren Syndrome.

Williams-Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries.

Allostatic Load/Chronic Stress and Cardiovascular Outcomes in Patients Diagnosed With Breast, Lung, or Colorectal Cancer.

Background: Cardiovascular disease and cancer share a common risk factor: chronic stress/allostatic load (AL). A 1-point increase in AL is linked to up to a 30% higher risk of major cardiac events (MACE) in patients with prostate cancer. However, AL's role in MACE in breast cancer, lung cancer, or colorectal cancer remains unknown.

Temporal trends in early-onset colorectal cancer incidence (2000-2020) by age group and five geographic regions in the state of Georgia.

The increase of early-onset colorectal cancer (CRC) among younger adults is a major public health concern. However, little is known about variations in CRC incidence across different age groups within small geographic areas in Georgia. We examined temporal trends of CRC incidence in Clayton, East Central, West Central, Northeast, and Southeast regions, by age groups.

Acquired von Willebrand syndrome in a patient with monoclonal gammopathy of unknown significance: A case report.

Monoclonal gammopathy of uncertain significance associated acquired von Willebrand syndrome is a serious bleeding condition driven by immunological clearance of von Willebrand factor and has limited treatment options. We present a patient who achieved durable remission through eradication of the monoclonal paraprotein with clonal directed therapy with bortezomib.

Patterns of Aortic Dilation in Tetralogy of Fallot: An Analysis of 100 Fetal Echocardiograms Compared With Matched Controls.

Background Although aortic dilation is common in tetralogy of Fallot (TOF), its progression and risk of dissection are not well understood. The mechanism of dilation is primarily attributed to increased flow in utero; an alternative is unequal septation of the truncus arteriosus resulting in a larger aorta and inherently hypoplastic pulmonary artery (PA). If the latter is true, we hypothesize the aorta to PA ratio in TOF is stable throughout gestation, and sums of great artery dimensions are similar to controls.

Plasma ceramides relate to mild cognitive impairment in middle-aged men: The Maastricht Study.

Introduction: There is an urgent need for biomarkers identifying individuals at risk of early-stage cognitive impairment. Using cross-sectional data from The Maastricht Study, this study included 197 individuals with mild cognitive impairment (MCI) and 200 cognitively unimpaired individuals aged 40 to 75, matched by age, sex, and educational level.

Methods: We assessed the association of plasma sphingolipid and ceramide transfer protein (CERT) levels with MCI and adjusted for potentially confounding risk factors.

The current status of nanotechnological approaches to therapy and drug delivery in otolaryngology: A contemporary review.

Objectives/hypothesis: To summarize the current standing of nanomedicine-based technology, particularly nanoparticles (NPs), for drug delivery and diagnostic mechanisms in otolaryngology and the otolaryngology subspecialties.

Methods: Literature searches were performed using PubMed and Ovid MEDLINE from 2010 to 2022. The search focused on original articles describing developments and applications of nanotechnology and drug delivery in otology, neurotology, cranial base surgery, head and neck oncology, laryngology, bronchoesophagology, and rhinology.

Pediatric basilar skull fractures from multi-level falls: A systematic review and retrospective analysis.

Objective: Multi-level fall (MLF) accounts for 26.5%-37.7% of traumatic pediatric basilar skull fractures (BSFs).

Rapid response to mycophenolate mofetil in combination with romiplostim in a case of severe refractory immune thrombocytopenia post COVID-19 vaccination.

Vaccine mediated immune mediated thrombocytopenia (ITP) is exceedingly rare. We present a case of a young female who developed severe refractory ITP with multiple bleeding sites post second dose of COVID-19 vaccination. She was treated with a combination of romiplostim and mycophenolate mofetil that resulted in rapid platelet count recovery.

Longitudinal cognitive performance of Alzheimer's disease neuropathological subtypes.

Introduction: Alzheimer's disease (AD) neuropathological subtypes (limbic predominant [lpAD], hippocampal sparing [HpSpAD], and typical [tAD]), defined by relative neurofibrillary tangle (NFT) burden in limbic and cortical regions, have not been studied in prospectively characterized epidemiological cohorts with robust cognitive assessments.

Methods: Two hundred ninety-two participants with neuropathologically confirmed AD from the Religious Orders Study and Memory and Aging Project were categorized by neuropathological subtype based on previously specified diagnostic criteria using quantitative regional NFT counts. Rates of cognitive decline were compared across subtypes using linear mixed-effects models that included subtype, time, and a subtype-time interaction as predictors and four cognitive domain factor scores (memory, executive function, language, visuospatial) and a global score as outcomes.

Lower-Intensity Statins Contributing to Gaps in Care for Patients With Primary Severe Hypercholesterolemia.

Background Although severe hypercholesterolemia confers a 5-fold increased long-term risk for coronary artery disease, treatment guidelines may not be fully implemented, leading to underdiagnosis and suboptimal treatment. To further understand the clinical features and gaps in treatment approaches, we analyzed electronic medical record data from a midwestern US multidisciplinary healthcare system, between 2009 and 2020. Methods and Results We retrospectively assessed the prevalence, clinical presentation, and treatment characteristics of individuals currently treated with statin therapy having a low-density lipoprotein cholesterol (LDL-C) value that is either (1) an maximum electronic medical record-documented LDL-C ≥190 mg/dL (group 1, n=7542) or (2) an pretreatment LDL-C ≥190 mg/dL (group 2, n=7710).

Feasibility study for detection of retinal amyloid in clinical trials: The Anti-Amyloid Treatment in Asymptomatic Alzheimer's Disease (A4) trial.

Introduction: The retina and brain exhibit similar pathologies in patients diagnosed with neurodegenerative diseases. The ability to access the retina through imaging techniques opens the possibility for non-invasive evaluation of Alzheimer's disease (AD) pathology. While retinal amyloid deposits are detected in individuals clinically diagnosed with AD, studies including preclinical individuals are lacking, limiting assessment of the feasibility of retinal imaging as a biomarker for early-stage AD risk detection.

Defining an Ultrasound-guided Regional Anesthesia Curriculum for Emergency Medicine.

Objectives: Ultrasound-guided regional anesthesia (UGRA) can be a powerful tool in the treatment of painful conditions commonly encountered in emergency medicine (EM) practice. UGRA can benefit patients while avoiding the risks of procedural sedation and opioid-based systemic analgesia. Despite these advantages, many EM trainees do not receive focused education in UGRA and there is no published curriculum specifically for EM physicians.

Mixed Methods Study of Patient and Primary Care Provider Perceptions of Chronic Pain Treatment.

Objective: A mixed-method study involving patient focus groups and survey of primary care providers (PCPs) sought to compare perspectives about chronic pain (CP) and its treatment. Our goal was to identify needs and barriers for facilitating patient-centered care.

Methods: Two focus groups of CP patients from a single academic medical center explored interactions with PCPs and their understandings, experiences, and expectations of CP treatment.

Integrin-associated CD151 is a suppressor of prostate cancer progression.

Owing to the complexity of interacting molecular networks on the cell surface, integrin-associated tetraspanin CD151 remains controversial regarding its clinical importance and functional impact in prostate cancer. The current study evaluated dynamics and clinical importance of CD151 expression and its function in prostate cancer by IHC analysis of two independent patient cohorts (n=80, 181), bioinformatic interrogation of the TCGA database, and evaluation of gene knockdown effect at the cellular level. Our data showed that aside from high mRNA expression, CD151 was primarily localized to intercellular junctions at the plasma membrane in normal prostate glands or benign tissues, regardless of nature of antibodies used.

The Value of Innovation to Implementation Program (VIP): A strategic approach to aligning and leveraging academic research and clinical care missions.

Problem: Inefficient implementation of evidence-based care garners increasing attention as a source of suboptimal value of clinical care, and integration of quality improvement methodology into clinical practice represents a potential solution. Academic medical centers (AMCs) often have expertise in implementation science, yet it is not leveraged effectively to solve operational inefficiencies or to rapidly implement evidence-based practices (EBPs).

Approach: To leverage in-house research expertise, the University of Kentucky (UK) College of Medicine and Center for Health Services Research (CHSR) launched a pilot awards program-Value of Innovation to Implementation Program (VIP)-across its health system and six health professional colleges.

Effect of Intensive and Standard Clinic-Based Hypertension Management on the Concordance Between Clinic and Ambulatory Blood Pressure and Blood Pressure Variability in SPRINT.

Background Blood pressure ( BP ) varies over time within individual patients and across different BP measurement techniques. The effect of different BP targets on concordance between BP measurements is unknown. The goals of this analysis are to evaluate concordance between (1) clinic and ambulatory BP , (2) clinic visit-to-visit variability and ambulatory BP variability, and (3) first and second ambulatory BP and to evaluate whether different clinic targets affect these relationships.

Stabilization of PTGES by deubiquitinase USP9X promotes metastatic features of lung cancer via PGE signaling.

Early metastasis and local recurrence are the major causes of mortality and poor prognosis of non-small cell lung cancer (NSCLC). However, the underlying mechanisms of these processes are poorly understood. In this study, we aimed to investigate the roles of the PTGES/PGE pathway in lung cancer progression.