Rupture of a calcified right ventricle to pulmonary artery homograft by balloon dilation- emergency rescue by venus P-Valve.

Background: Percutaneous pulmonary valve implantation (PPVI) is a recognized alternative treatment to surgery for patients with dysfunctional right ventricular outflow tracts. Patient selection is essential to avoid serious complications from attempted treatment, such as rupture or dissection, especially of the calcified outflow tracts. We describe a case with an unexpected rupture of a calcified homograft valve and main pulmonary artery, which was treated successfully by emergency implantation of a self-expanding Venus P-Valve (Venus MedTech, Hangzhou, China) without the need for pre-stenting with a covered stent.

Unintended Consequences of Immune Therapy for Immune-Mediated Diseases: Paradoxical Psoriasis and Dupilumab-Associated Musculoskeletal Syndrome.

Two presentations at the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) 2023 annual meeting focused on unintended consequences of immunomodulatory therapy for psoriasis (PsO). Dr. Elizabeth Wallace presented on unintended consequences of tumor necrosis factor inhibitors for treating PsO and other inflammatory disorders.

Regulatory T cells and immunoglobulin E: A new therapeutic link for autoimmunity?

Autoimmune diseases have a prevalence of approximately 7 to 9% and are classified as either organ-specific diseases, including type I diabetes, multiple sclerosis, inflammatory bowel disease and myasthenia gravis, or systemic diseases, including systemic lupus erythematosus, rheumatoid arthritis and Sjögren's syndrome. While many advancements have been made in understanding of the mechanisms of autoimmune disease, including the nature of self-tolerance and its breakdown, there remain unmet needs in terms of effective and highly targeted treatments. T regulatory cells (Tregs) are key mediators of peripheral tolerance and are implicated in many autoimmune diseases, either as a result of reduced numbers or altered function.

Molecular Pathways for Immune Recognition of Preproinsulin Signal Peptide in Type 1 Diabetes.

The signal peptide region of preproinsulin (PPI) contains epitopes targeted by HLA-A-restricted (HLA-A0201, A2402) cytotoxic T cells as part of the pathogenesis of β-cell destruction in type 1 diabetes. We extended the discovery of the PPI epitope to disease-associated and (risk) and and (protective) alleles, revealing that four of six alleles present epitopes derived from the signal peptide region. During cotranslational translocation of PPI, its signal peptide is cleaved and retained within the endoplasmic reticulum (ER) membrane, implying it is processed for immune recognition outside of the canonical proteasome-directed pathway.

T cell receptor β-chains display abnormal shortening and repertoire sharing in type 1 diabetes.

Defects in T cell receptor (TCR) repertoire are proposed to predispose to autoimmunity. Here we show, by analyzing >2 × 10 TCRB sequences of circulating naive, central memory, regulatory and stem cell-like memory CD4 T cell subsets from patients with type 1 diabetes and healthy donors, that patients have shorter TCRB complementarity-determining region 3s (CDR3), in all cell subsets, introduced by increased deletions/reduced insertions during VDJ rearrangement. High frequency of short CDR3s is also observed in unproductive TCRB sequences, which are not subjected to thymic culling, suggesting that the shorter CDR3s arise independently of positive/negative selection.

Understanding and preventing type 1 diabetes through the unique working model of TrialNet.

Type 1 diabetes is an autoimmune disease arising from the destruction of pancreatic insulin-producing beta cells. The disease represents a continuum, progressing sequentially at variable rates through identifiable stages prior to the onset of symptoms, through diagnosis and into the critical periods that follow, culminating in a variable depth of beta cell depletion. The ability to identify the very earliest of these presymptomatic stages has provided a setting in which prevention strategies can be trialled, as well as furnishing an unprecedented opportunity to study disease evolution, including intrinsic and extrinsic initiators and drivers.

[F]-Fluorodeoxyglucose Positron Emission Tomography in the Diagnosis, Treatment Stratification, and Monitoring of Patients with Retroperitoneal Fibrosis: A Prospective Clinical Study.

Background: The ability to distinguish malignant from benign retroperitoneal fibrosis (RPF) and to select patients who are likely to respond to steroid treatment using a noninvasive test would be a major step forward in the management of patients with RPF.

Objective: To prospectively evaluate the potential of [F]-fluorodeoxyglucose positron emission tomography (FDG-PET) to improve clinical decision-making and management of RPF.

Design, Setting, And Participants: A total of 122 RPF patients were assessed and managed by a multidisciplinary RPF service between January 2012 and December 2015.

Short-term outcome of periviable small-for-gestational-age babies: is our counseling up to date?

Objective: There are limited data for counseling on and management of periviable small-for-gestational-age (SGA) fetuses. We therefore aimed to investigate the short-term outcome of periviable SGA fetuses in relation to the likely underlying cause.

Methods: This was a retrospective study of data from three London tertiary fetal medicine centers obtained between 2000 and 2015.

Independent Loss of Methylthioadenosine Phosphorylase (MTAP) in Primary Cutaneous T-Cell Lymphoma.

Methylthioadenosine phosphorylase (MTAP) and the tumor suppressor genes CDKN2A-CDKN2B are frequently deleted in malignancies. The specific role of MTAP in cutaneous T-cell lymphoma subgroups, mycosis fungoides (MF) and Sézary syndrome (SS), is unknown. In 213 skin samples from patients with MF/SS, MTAP copy number loss (34%) was more frequent than CDKN2A (12%) in all cutaneous T-cell lymphoma stages using quantitative reverse transcription PCR.

A multifaceted intervention package to improve the diagnosis and management of delirium.

ABSTRACT Background: Delirium is a major cause of morbidity and mortality amongst hospital patients. Previous studies have shown that it is often poorly recognized and managed. We wanted to assess the impact of a multifaceted intervention on delirium management.

Blood and islet phenotypes indicate immunological heterogeneity in type 1 diabetes.

Studies in type 1 diabetes indicate potential disease heterogeneity, notably in the rate of β-cell loss, responsiveness to immunotherapies, and, in limited studies, islet pathology. We sought evidence for different immunological phenotypes using two approaches. First, we defined blood autoimmune response phenotypes by combinatorial, multiparameter analysis of autoantibodies and autoreactive T-cell responses in 33 children/adolescents with newly diagnosed diabetes.

Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart.

Aims: The 'hybrid' procedure is an alternative to the Norwood operation in classical hypoplastic left heart syndrome to support the systemic circulation until subsequent palliation. This approach has been extended to infants with the borderline development of left heart structures. We investigated whether or not a hybrid procedure for 'borderline' cases of underdevelopment of the left heart would lead to any improvement in the growth of those structures relative to body size or would impact on eventual repair.

The experience of agency nurses working in a London teaching hospital.

This Husserlian phenomenological study explored the lived experience of eight full-time agency nurses working in the environment of intensive care in London, United Kingdom. In-depth interviews were used to ascertain the participants' experiences of full-time agency nursing in intensive care. Colaizzi's [Colaizzi P.