Deutsche medizinische Wochenschrift
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Dtsch. Med. Wochenschr. · Nov 2024
Historical Article[100 years thrombotic thrombocytopenic purpura (TTP) - lessons learned?].
100 years ago Dr. Eli Moschcowitz described the first case of thrombotic thrombocytopenic purpura. For many decades there were no recognized treatment options, and the mortality rate was extremely high. ⋯ Various publications from national iTTP cohorts improved the data and showed consistent reductions in the time until platelet normalization, a reduction in refractory courses and exacerbations (especially when therapy is controlled according to ADAMTS13-activity) as well as evidence of reduced mortality. Modern therapeutic options include strategies for preemptive therapy for ADAMTS13-relapse as well as plasma exchange-free treatment. The use of recombinant ADAMTS13 may also expand the therapeutic options in iTTP patients in the future.
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Renal infarction is a rare cause for an acute renal failure. At the time being, there is no sufficient correlation to the consumption of cannabis described. ⋯ The correlation between thrombo-embolic events and the consumption of cannabis has to be more explored because of its recent legalization in Germany. It should be considered as a cardiovascular risk factor. Until today there are no recommendations for the anticoagulants.
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Approximately one third of the German population has a migration background. According to the federal office for migration, in 2022 around 2,7 million people have taken refuge or have immigrated to Germany, causing major challenges for our health system. ⋯ Furthermore, new treatment options for hemoglobinopathies have been approved in the last couple of years, starting with the introduction of Luspatercept a few years ago for Beta-Thalassemias and in 2023 the introduction of Voxelotor for sickle cell disease. In 2024 the gene therapy with Exagamglogen-Autotemcel using the CRISPR/Cas molecular scissor was authorized as a new promising treatment for both conditions.
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A succinct summary of the 2023 guidelines on the management of infectious endocarditis of the European Society of Cardiology is presented. Main new aspects are (1) the importance of treating complicated endocarditis at a hospital with an endocarditis team and cardiac surgery, (2) a less restrictive recommendation for antibiotic prophylaxis, which now favors an individualized indication in patients with intermediary risk, e.g. bicuspid aortic valve and degenerative or rheumatic native valve disease, undergoing orodental interventions, while there is a clear recommendation for prophylaxis in high-risk patients (e.g. having prosthetic valves). (3) In patients with left-sided endocarditis caused by streptococci, enterococcus faecalis, staphylococcus aureus, or coagulase-negative staphylococci, who are stable after at least 10 days of in-hospital intravenous appropriate antibiotic therapy, step-down ambulatory (oral or parenteral) further antibiotic therapy is recommended.
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While genetic kidney diseases were long regarded as a rare cause of kidney failure, it has been shown in recent years that they account for a relevant proportion of cases. In cohorts of kidney transplant recipients, a monogenic cause is found in up to 30% of cases. Identifying the genetic cause of kidney disease has become much easier thanks to technological advances in DNA sequencing. ⋯ Centers of excellence with extensive experience in this area therefore play an important role in improving care. This also applies to the further training of colleagues in the field. In Germany, the National Action Alliance for People with Rare Diseases (NAMSE) and the nationwide establishment of - to date - 36 centers for rare diseases play an important role in this regard.