A multi-centre UK-based survey on angioedema secondary to acquired C1 inhibitor deficiency.

Background: Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH) is very rare compared to its prototype, hereditary angioedema. An updated characterisation of the AAE-C1-INH cohort in UK is required to inform management.

Objectives: To describe the disease burden of AAE-C1-INH, long-term prophylaxis (LTP) and the clinical, immunochemical and treatment profiles of AAE-associated diseases in UK.

The Year in Cardiothoracic Transplant Anesthesia: Selected Highlights From 2022 Part I: Lung Transplantation.

These highlights focus on the research in lung transplantation (LTX) that was published in 2022 and includes the assessment and optimization of candidates for LTX, donor optimization, the use of organs from donation after circulatory death, and outcomes when using marginal or novel donors; recipient factors affecting LTX, including age, disease, the use of extracorporeal life support; and special situations, such as coronavirus disease2019, pediatric LTX, and retransplantation. The remainder of the article focuses on the perioperative management of LTX, including the perioperative risk factors for acute renal failure (acute kidney injury); the incidence and management of phrenic nerve injury, delirium, and pain; and the postoperative management of hyperammonemia, early postoperative infections, and the use of donor-derived cell-free DNA to detect rejection.

All the king's men (and women): Fluoro-less leadless pacemaker implant in a severely obese patient.

Introduction: Severe obesity presents significant challenges in imaging and delivery of therapy, including pacemaker implant.

Methods And Result: We present our experience implanting a leadless pacemaker (LP) in a severely obese man presenting with heart block. We describe our multidisciplinary approach using right internal jugular venous access and transesophageal imaging in lieu of fluoroscopy which failed to provide workable images in this instance.

"Blocking-like" effects in attentional set-shifting: Redundant cues facilitate shifting in male rats with medial prefrontal cortex inactivation.

Without a functioning prefrontal cortex, humans and other animals are impaired in measures of cognitive control and behavioral flexibility, including attentional set-shifting. However, the reason for this is unclear with evidence suggesting both impaired and enhanced attentional shifting. We inhibited the medial prefrontal cortex (mPFC) of rats while they performed a modified version of an attentional set-shifting task to explore the nature of this apparent contradiction.

Synthesis and Thermodynamic Evaluation of Sialyl-Tn MUC1 Glycopeptides Binding to Macrophage Galactose-Type Lectin.

Interactions between the tumor-associated carbohydrate antigens of Mucin 1 (MUC1) and the carbohydrate-binding proteins, lectins, often lead to the creation of a pro-tumor microenvironment favoring tumor initiation, progression, metastasis, and immune evasion. Macrophage galactose binding lectin (MGL) is a C-type lectin receptor found on antigen-presenting cells that facilitates the uptake of carbohydrate antigens for antigen presentation, modulating the immune response homeostasis, autoimmunity, and cancer. Considering the crucial role of tumor-associated forms of MUC1 and MGL in tumor immunology, a thorough understanding of their binding interaction is essential for it to be exploited for cancer vaccine strategies.

Feasibility and acceptability of a pilot studying investigating multi-family parent-only guided self-help family-based treatment for adolescent anorexia nervosa.

Objective: Family-based treatment (FBT) is an efficacious treatment for adolescent anorexia nervosa (AN). A parent-only guided self-help version of FBT (GSH-FBT) demonstrated preliminary efficacy in pilot investigations. To address challenges in access to care, we adapted GSH-FBT into a parent-only multi-family group format (MF-GSH-FBT) delivered via videoconferencing.

Ophthalmoplegia Associated With Extramedullary Multiple Myeloma: Case Series From a Tertiary Cancer Center.

Background: Multiple myeloma (MM) is a malignant disorder of plasma cells that results in tumor cells replacing the bone marrow. In extramedullary MM (EMM), however, tumor cells proliferate outside the bone marrow. EMM may produce ophthalmoplegia through direct invasion of the superior orbital fissure, cavernous sinus, and/or sphenoidal sinus.

An Uncommon Case of Multiple, Recurrent Cerebrospinal Fluid Venous Fistulas.

We present a clinical case to discuss the use of computed tomography (CT) spine myelograms alongside a complete history to diagnose multiple cerebrospinal fluid (CSF) venous fistulas (CVFs). The goal of this study is to familiarize clinicians with this challenging diagnosis and the utility of these studies in localizing leaks. A 63-year-old male patient with a history of cervical spinal stenosis, intermittent double vision, and sinus pressure managed with intermittent steroids presented to the clinic.

Ocular Toxicity Profile of Targeted Cancer Therapy (TCT) at a US Tertiary Cancer Center.

Purpose:  Targeted cancer therapy (TCT) is a significant advancement in oncology with promising survival improvement in patients with cancer and remarkable effects on various cancers. There is evidence suggesting a connection between specific TCT classes and the occurrence of immune-related adverse events (irAEs). Our study aims to investigate the potential ocular toxicities of different classes of TCT, provide a better understanding of these toxicities, and aid in the future development of screening and management recommendations for ocular irAEs.

Investigating the Effect of Lewis Acid Co-catalysts on Photosensitized Visible-Light De Mayo Reactions.

Herein, we describe studies showing that Lewis acid co-catalysts can significantly broaden the scope of alkenes that can be incorporated into the photosensitized visible-light De Mayo reaction. Mechanistic studies suggest that the primary benefit of the Lewis acid is not on substrate sensitization but rather on bond-forming steps downstream of energy transfer, highlighting the diverse effects that Lewis acids can have on sensitized photoreactions.

The Year in Perioperative Echocardiography: Selected Highlights From 2022.

THIS SPECIAL article is part of an annual series for the Journal of Cardiothoracic and Vascular Anesthesia. The authors thank the editor-in-chief, Dr. Kaplan, and the Editorial Board for the opportunity to continue this series, which focuses on the past year's research highlights that pertain to perioperative echocardiography in relation to cardiothoracic and vascular anesthesia.

Highlights on Ocular Toxicity of Immune Checkpoint Inhibitors at a US Tertiary Cancer Center.

Introduction: Immune checkpoint inhibitors (ICIs) have improved prognosis in advanced malignancies; however, they may be associated with extensive ocular immune-related adverse events (irAEs) that are sight threatening. Our study aimed to identify the presentation, characteristics, management, and clinical outcomes of ocular irAEs.

Methods: In this retrospective, observational case series, we reviewed the medical records of 1280 patients at a large US tertiary cancer center between 2010 and 2020.

Selective Cross-Ketonization of Carboxylic Acids Enabled by Metallaphotoredox Catalysis.

Carboxylic acids are attractive building blocks for synthetic chemistry because they are chemically stable, abundant, and commercially available with substantial structural diversity. The process of combining two carboxylic acids to furnish a ketone is termed ketonization. This is a potentially valuable transformation that has been underutilized in organic synthesis due to the harsh reaction conditions generally required and the lack of selectivity obtained when coupling two distinct carboxylic acids.

Emergency treatment of anaphylaxis: concise clinical guidance.

Anaphylaxis is a serious systemic hypersensitivity reaction that is usually rapid in onset and may cause death. It is characterised by the rapid development of airway and/or breathing and/or circulation problems. Intramuscular adrenaline is the most important treatment, although, even in healthcare settings, many patients do not receive this intervention contrary to guidelines.

Dental management of a patient with systemic mastocytosis.

Mastocytosis is a term encompassing a group of clinical disorders characterised by clonal proliferation of abnormal mast cells (MCs) in organ systems of the body. Mastocytosis can be systemic (with or without skin involvement) or cutaneous, and can affect organs including bone marrow, liver, spleen, lymph nodes and mucosal surfaces. Patients with systemic mastocytosis (SM) are susceptible to triggers that could cause activation of abnormal MCs, resulting in multiorgan dysfunction and life-threatening anaphylactic reactions.

Case Report: Neuromyelitis Optica After Treatment of Uveal Melanoma With Nivolumab and Ipilimumab.

The development of immune checkpoint inhibitors (ICIs) has greatly improved survival of patients with advanced malignancies. ICIs can cause immune-related adverse effects (irAEs) involving any organ. Neurological irAEs are infrequent and have mostly been reported in patients with melanoma.

Accessing Unsymmetrically Linked Heterocycles through Stereoselective Palladium-Catalyzed Domino Cyclization.

A palladium-catalyzed strategy is presented to synthesize unsymmetrically linked heterocycles within stereoselective tetrasubstituted olefins. This reaction is proposed to occur via a vinyl-Pd intermediate capable of initiating the cyclization of various alkyne-tethered nucleophiles. Products are formed in up to 96 % yield and excellent stereoselectivities are obtained using low catalyst loadings.

Progress toward a comprehensive teaching approach to the FAIR data principles.

We evaluate recent efforts to further the effective teaching of FAIR data principles by examining existing and developing educational frameworks focused upon FAIR, training initiatives that have informed teaching on FAIR skills' topics, and a number of key sources for discovering FAIR training materials and how much those sources provide descriptive information about the materials. FAIR4S, providing a coherent description of skills and competencies, is analyzed by target audience using the description of actors found in a European Open Science Cloud ecosystem report and by comparison of the coverage and extent of description of educational and training materials available from the list of sources for finding such materials. Our analysis elucidates the importance of linking resources to FAIR-related educational frameworks, providing consistent descriptions of them using a community-based metadata scheme, and developing an instructor community of practice where ideas and methods can be shared on how to teach FAIR data skills.

Imaging of Trigeminal Neuralgia and Other Facial Pain.

We review and illustrate the radiology of facial pain, emphasizing trigeminal neuralgia, relevant anatomy, current classification, concepts about etiology, and the role of imaging and its influence on the choice of treatment. We discuss glossopharyngeal neuralgia, other neuropathic causes of facial pain, postinflammatory and neoplastic causes, and nociceptive (end-organ) causes of facial pain, as well as referred otalgia. Other conditions that may present with facial pain, including trigeminal autonomic cephalgias and giant cell arteritis, are reviewed briefly.

Dysphagia, Dysphonia, and Dysarthria Outcomes Among Adults Hospitalized With COVID-19 Across Ireland.

Objective: To investigate the presence, degree, predictors, and trajectory of dysphagia, dysphonia, and dysarthria among adults hospitalized with COVID-19 across the Republic of Ireland (ROI) during the first wave of the pandemic.

Study Design: Prospective observational cohort study.

Methods: Adults with confirmed COVID-19 who were admitted into 14 participating acute hospitals across ROI and referred to speech and language therapy between March 1st and June 30th 2020 were recruited.