Presse Med
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Sickle Cell Disease (SCD) is a hereditary blood disorder characterized by the presence of abnormal hemoglobin, leading to the formation of sickle-shaped red blood cells, causing vaso-occlusion. Inflammation is a key component of the pathophysiology of SCD, contributing to the vascular complications and tissue damage. ⋯ Additionally, we will discuss the proposed mechanisms by which the complement system may contribute to tissue injury in this pathology. Finally, we will provide an overview of the available evidence concerning the effectiveness of therapeutic interventions aimed at blocking the complement system in the context of SCD and discuss the perspective of complement inhibition.
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Bronchiectasis is a final common pathway of a wide variety of underlying conditions including infectious, autoimmune, allergic, genetic and inflammatory conditions. Patients experience a chronic disease with variable clinical symptoms and course, but most experience cough, sputum production and recurrent exacerbations. Symptoms of bronchiectasis lead to poor quality of life and exacerbations are the major driver of morbidity and mortality. ⋯ Airway clearance is the mainstay of therapy for patients with symptoms of cough and sputum production. Frequently exacerbating patients may benefit from long term antibiotic or mucoactive therapies. Bronchiectasis is a heterogeneous disease and increasingly precision medicine approaches are advocated to target treatments most appropriately and to limit the emergence of antimicrobial resistance.
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Lymphangioleiomyomatosis (LAM) is an ultra-rare, slowly progressive neoplastic cystic disease, belonging to the group of PEComas. It can occur sporadically or associated to tuberous sclerosis complex disease and affects mainly women in child-birth age. ⋯ In the last decade, important advances in understanding molecular mechanisms underlying the LAM pathogenesis have been reached. It has allowed to obtain improvements in the research of novel biomarkers, treatment and a better management of the disease.
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Device therapy for heart failure has rapidly evolved over 2 decades. The knowledge of indications, assessment lead and device technology has expanded to include CRT, leadless pacing and conduction system pacing such as His bundle and left bundle branch area pacing. ⋯ Rapidity of therapy is associated with outcome which will be a challenge. If properly implemented devices and drugs will have a large positive affect of HF outcomes.
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Heart failure (HF) is a major public health problem affecting millions of adults worldwide. HF with preserved ejection fraction, i.e. > 50 %, (HFpEF) accounts for more than half of all HF cases, and its incidence and prevalence are increasing with the aging of the population and the growing prevalence of metabolic disorders such as obesity, diabetes and hypertension. ⋯ This complexity probably accounts for the lack of evidence-based medicine compared with HF with reduced EF. Nevertheless, significant progress has been made recently, with a high level of evidence obtained for the SGLT2 inhibitor class on the one hand, and promising data with new drugs targeting more specifically certain mechanisms such as obesity and inflammation on the other.