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News Estimated Worldwide Prevalence of Population with AAT Deficiency with MZ Alleles

Alpha-1-antitrypsin deficiency (AATD) with the MZ genotype was until recently associated with negligible or low risk for lung and liver tissue damage. However, growing evidence suggests that carriers of MZ alleles are at increased risk of organ damage when exposed to tobacco smoke, polluted air, or hepatotoxic substances. Moreover, according to a global epidemiological study, the prevalence of the MZ genotype is very common, especially among Caucasians in Europe and other continents.
Source: Deficiency of Alpha-1-Antitrypsin 14. 11. 2022

News Repeated Episodes of Respiratory Failure Due to Undiagnosed AAT Deficiency –⁠ Case Report

The case report from authors in Naples shows that alpha-1-antitrypsin deficiency (AATD) should be considered when respiratory symptoms occur at any age. It also illustrates the diagnostic procedure in such cases and confirms the effectiveness of subsequently initiated therapy.
Source: Deficiency of Alpha-1-Antitrypsin 14. 11. 2022

News Persistent Symptom Control with s.c. Supplementation of C1 Inhibitor in Prophylactic Treatment of HAE

The open-label extension of the COMPACT study with subcutaneous C1 inhibitor in the prophylaxis of hereditary angioedema (HAE) has brought fresh results, shedding light on the treatment's impact on the frequency of attacks and reducing the need for rescue medication.
Source: Hereditary Angioedema 14. 11. 2022

News Inhibitor of Activated Factor XII Garadacimab in Prophylaxis of HAE Attacks

Hereditary angioedema (HAE) is an autosomal dominant inherited disease characterized by a deficiency of C1 inhibitor. This condition leads to excessive production of bradykinin, which is responsible for the development of soft tissue edema. Garadacimab represents a human antibody, the first of its class, aimed at inhibiting activated factor XII, a key initiator of bradykinin formation. The phase II study presented below, whose results were recently published, evaluated the efficacy and safety of this innovative HAE therapy.
Source: Hereditary Angioedema 14. 11. 2022

News Good news for sleep in patients with axial spondyloarthritis –⁠ ixekizumab effectively reduces night-time back pain

The targeted treatment of axial spondyloarthritis currently relies on the anti-inflammatory effects of tumor necrosis factor (TNF) inhibitors, interleukin IL-17 inhibitors, and Janus kinase (JAK) inhibitors. Besides the objective efficacy of these targeted treatments, it is crucial to describe their influence on subjective symptoms for decision-making in clinical practice. The evaluation of the analgesic effect of ixekizumab (IXE) was provided by the randomized controlled trial COAST-V.
Source: Biological Treatment 14. 11. 2022

News To what extent can improvement of nutritional status through parenteral nutrition affect the results of oncological treatment?

Just like cancer patients and their caregivers, doctors often overlook the risk of malnutrition. Good nutrition can be a predictor of survival in patients with malignancies.
Source: Parenteral Nutrition 14. 11. 2022

News Does SGLT2 Inhibitor Administration Benefit Diabetics with Low Cardiorenal Risk?

There is growing evidence of the cardiorenal protective effects of sodium-glucose cotransporter 2 inhibitors (SGLT2i, also known as gliflozins), which were developed as antihyperglycemic drugs for the treatment of type 2 diabetes. These antidiabetic medications are recommended for patients with heart or renal disease or at high risk of these complications. However, is it worthwhile to consider their use in diabetics as part of long-term primary cardiorenal prevention? Israeli authors sought the answer to this question.
Source: Diabetes 14. 11. 2022

News 6 Possible Benefits of Adding Empagliflozin to Metformin

Currently, there is intense discussion about the advantages of sodium-glucose co-transporter 2 inhibitors (SGLT2i, also known as gliflozins) in terms of their cardio and nephroprotective properties. Below, we summarize the proven beneficial effects specifically associated with adding empagliflozin to metformin and mention the indications for empagliflozin, which are reimbursed.
Source: Diabetes 14. 11. 2022

News Benefits of Physical Activity for Individuals with Hemophilia –⁠ Recommendations of the MEMO Group

Regular physical activity can increase joint stability and function in individuals with hemophilia, reduce the risk of injury, improve quality of life, and have other positive effects. However, according to recent findings, a regular exercise program is not sufficiently emphasized in comprehensive hemophilia therapy. Therefore, a group of Italian experts has created a consensus summarizing current knowledge about the benefits of exercise for individuals with hemophilia and recommendations for physical activity.
Source: Hemophilia with Movement 25. 11. 2022

News The Importance of Proper Protein and Calorie Intake in Critically Ill Patients –⁠ Insights from Recent Comparisons

The issue of nutrition, especially proteins, is a crucial aspect affecting the overall health and prognosis of critically ill patients. Two recent studies published in the journals Critical Care and Journal of Parenteral and Enteral Nutrition focused on nutrient intake.
Source: Parenteral Nutrition 14. 11. 2022

News Impact of Early Administration of High Protein Dose on Mortality of Critically Ill Patients

Energy intake and the composition of individual macronutrients in patients in intensive care units (ICUs) can influence treatment outcomes in terms of the incidence of complications, duration of mechanical ventilation, or mortality.
Source: Parenteral Nutrition 14. 11. 2022

News MDT Board: a unique project for doctors caring for patients with lung diseases and other thoracic pathologies

A unique concept of a cooperative thoracic group has been created in the Czech Republic, involving specialists from several leading Prague clinics. The primary purpose of the so-called board is to provide the best diagnostic and treatment plan for a patient with lung disease and other pathologies in the thoracic area, shared by all members of the multidisciplinary team and based on current knowledge. The MDT Board project, which operates on the principle of online indication seminars, is intended for doctors of patients with common and rare lung diseases.
Source: MDT Board 14. 11. 2022

News Population Pharmacokinetic Model in Patients with von Willebrand Disease

Dutch authors presented a potential population pharmacokinetic model describing levels of von Willebrand factor and coagulation factor VIII during and after surgical procedures in patients with von Willebrand disease.
Source: Von Willebrand Disease 14. 11. 2022

News Pharmacokinetics of Concentrates for the Treatment of von Willebrand’s Disease

Von Willebrand's disease (vWD) is characterized by a deficiency or reduced functional capacity of von Willebrand factor (vWF) and, in more severe cases, by a deficiency of coagulation factor VIII (FVIII). Treatment usually requires repeated infusions of vWF and FVIII concentrate, with available products having different factor ratios. Repeated infusions of concentrates can lead to different FVIII exposures when using different products. The pharmacokinetics of two products with varying vWF and FVIII ratios have been compared in experimental preclinical and clinical studies.
Source: Von Willebrand Disease 14. 11. 2022

News New Therapeutic Options in Light of Current Knowledge on the Pathogenesis of Atopic Dermatitis

Atopic dermatitis (AD) is a chronic inflammatory skin disease characterized by intense itching and recurrent eczematous lesions. AD is diagnosed in 2 out of 10 children and often persists into adulthood. In developed countries, it is the most common skin disease with a lifetime prevalence exceeding 15%. However, it is also very diverse due to a wide range of clinical phenotypes reflecting various pathophysiological mechanisms and interactions between genetics, the immune system, and the environment. So, what have we learned about atopic dermatitis from recent research, and how has this translated into advances in its treatment?
Source: Biological Treatment 14. 11. 2022

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