Innere Medizin (Heidelberg, Germany)最新文献

Background: Sepsis is defined as "being evoked as a life-threatening organ dysfunction caused by an inadequate host response to infection". The most recent German S3 guidelines were published in 2018 and the Surviving Sepsis Campaign (SSC) last published the current recommendations for the treatment of sepsis and septic shock in 2021.

Objective: This article explores and discusses which evidence in the treatment of sepsis and septic shock has been confirmed.

Material and methods: Discussion of the 2018 German S3 guidelines, supplementation of the content of the 2021 international guidelines and recent research results since 2021.

Results: The primary objective for managing sepsis and septic shock still includes rapid identification, early initiation of anti-infective treatment, and focus cleansing when feasible. In addition, the focus is on hemodynamic stabilization, including the early use of vasopressors for prevention of hypervolemia and, if necessary, the use of organ support procedures. Supportive treatment, such as the administration of corticosteroids and the use of apheresis, can be advantageous in specific scenarios. The focus is increasingly shifting towards post-intensive care unit (ICU) follow-up care, improving the quality of life after surviving sepsis and the close involvement of relatives of the patient.

Conclusion: Despite the fact that considerable progress has been made in understanding the pathophysiology and treatment of sepsis, the early administration of anti-infective agents, focus control, nuanced volume therapy and the use of catecholamines continue to be fundamental to sepsis management. New recommendations emphasize the early use of vasopressors (primarily norepinephrine) and the administration of corticosteroids, especially in cases of septic shock and pneumonia.

Cardiovascular diseases are still the leading global cause of mortality. Modifiable cardiovascular risk factors have been well-defined for many years and are amenable to multimodal treatment. The scope of pharmaceutical interventions targeting atherogenic lipoproteins in general and low-density lipoprotein (LDL)-cholesterol concentrations in particular, has significantly broadened over the last years, leading to an intensification of appropriate treatment targets, which are not sufficiently achieved in the clinical routine. Current prevention guidelines issued by the European Society for Cardiology (ESC) define patient cohorts according to their individual cardiovascular risk, which results in a risk-adapted lipid-lowering treatment. The aim is to avoid overtreatment of patients with a low or moderate risk and undertreatment of patients with a high or very high risk. In addition to secondary prevention in patients with an already established cardiovascular disease, primary prevention uses age-adapted risk stratification algorithms to assess the 10-year risk, e.g., systematic coronary risk evaluation 2 (SCORE2), SCORE2-older persons (SCORE2-OP) and the lifetime risk, e.g., lifetime-perspective cardiovascular disease model (LIFE-CVD) as the foundation for therapeutic decision making. Special patient groups with known elevated cardiovascular risk (familial hypercholesterolemia, diabetes mellitus, chronic kidney disease) are stratified by incorporating disease-specific parameters and are treated according to defined lipid-lowering targets.

Immune thrombocytopenia (ITP) is an acquired thrombocytopenia caused by an autoimmune reaction against platelets in the blood and against megakaryocytes in the bone marrow. The indication for treatment is based on the bleeding symptoms and patient-specific factors. This article presents the current 2023 expert report that also forms the basis for the updated version of the Onkopedia guideline, which is expected in the course of 2024. In addition to the background to pathogenesis and pathophysiology, the article discusses clinical manifestations and diagnostics, as well as first-, second- and third-line therapy, including the management of emergency situations. Practice-relevant recommendations are given on the use and side effects of glucocorticoids, as well as on therapy with thrombopoietin receptor agonists (TRA), including the withdrawal regimen, and on treatment with a syk inhibitor.

Fabry's disease is a rare X chromosome-linked inherited lysosomal storage disease characterized by insufficient metabolism of the substrate globotriaosylceramide (Gb₃) due to reduced alpha-galactosidase A (AGAL) activity. Lysosomal Gb₃ accumulation causes a multisystemic disease which, if untreated, reduces the life expectancy in females and males by around 10 and 20 years, respectively, due to progressive renal dysfunction, hypertrophic cardiomyopathy, cardiac arrhythmia and early occurrence of cerebral infarction. The diagnosis is confirmed by determining the reduced AGAL activity in leukocytes in males and molecular genetic detection of a -mutation causing the disease in females. The treatment comprises enzyme replacement therapy (ERT), agalsidase alfa, 0.2 mg/kg body weight (BW), agalsidase beta 1.0 mg/kg BW or pegunigalsidase alfa 1.0 mg/kg BW every 2 weeks i.v. or oral chaperone therapy (one capsule of migalastat 123 mg every other day) in the presence of amenable mutations. This article summarizes the data on the treatment of Fabry's disease and on complications in practice. The current guideline recommendations are addressed and new study results that could expand the therapeutic repertoire in the future are discussed.

Background: Iron deficiency is worldwide the most frequently occurring deficiency of a trace element. Meanwhile, the indications are increasing that iron deficiency plays a relevant role in many cardiovascular diseases and that treatment is accessible with intravenous administration of iron.

Objective and methods: The aim of this article is to elucidate the clinical comorbidities, diagnostic dilemmas and treatment possibilities of iron deficiency in cardiovascular diseases. The study situation on iron deficiency and iron substitution in heart failure, aortic valve stenosis, atrial fibrillation and pulmonary hypertension (PH) is summarized.

Results: The diagnostic criteria of iron deficiency in cardiovascular diseases are not finally decided. The guidelines of the European Society of Cardiology recommend either ferritin below 100 ng/ml alone or ferritin between 100 and 299 ng/ml with a transferrin saturation (TSAT) < 20 %. Some authors consider the determination of TSAT as sufficient as the only diagnostic criterion for iron deficiency in heart failure. Most studies on iron substitution in heart failure showed an improvement in the physical capacity and a reduction of the probability of a heart failure-related hospitalization by the substitution of an existing iron deficiency; however, it has been determined that a relevant proportion of patients show no response to iron substitution and that the cause for this is ultimately unclear. Whether the diagnostic criteria for iron deficiency in heart failure can be transferred to other cardiovascular symptoms, cannot be clearly answered due to the lack of data from prospective interventional studies.

Conclusion: The substitution of iron deficiency is one of very few possibilities to improve the physical capability in heart failure. The pivotal point of the discussion on iron deficiency and substitution in cardiovascular diseases is the correct identification of patients who benefit from treatment.

Rare kidney diseases encompass a wide range of congenital, inherited and acquired conditions. Two million Europeans are affected by rare kidney diseases. The European Rare Kidney Disease Reference Network (ERKNet) aims to improve the clinical management of patients with these diseases. ERKNet encompasses 95 highly specialized adult and pediatric nephrology units at 72 sites in 24 European Union (EU) member states, as well as a group of patient advocates (European Patient Advocacy Group, ePAG). ERKNet centers care for more than 65,000 rare kidney disease patients and pursues a variety of activities. An online consultation service helps improve the management of complex cases. Expert working groups develop clinical practice guidelines for individual groups of rare kidney diseases. In a 3-year postgraduate program, junior physicians at ERKNet centers are trained by experts in the diagnosis and treatment of rare kidney diseases through webinars and case-based eLearning modules. Information brochures and online texts on rare kidney diseases for patients, relatives and the general public are produced and disseminated. Clinical research is supported by a European Registry for Rare Kidney Diseases (ERKReg), which provides important information on demographics and disease progression and facilitates the identification of patient cohorts for therapeutic studies. In addition, the registry provides clinical performance statistics of reference centers and allows benchmarking to promote the harmonization and standardization of care for rare kidney disease patients across Europe.