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[Hemophilia: is a revolution in treatment options taking place?] [血友病:治疗方案正在发生革命?]
Pub Date : 2024-10-01 Epub Date: 2024-08-30 DOI: 10.1007/s00108-024-01712-4
Michael Spannagl

Prophylactic replacement therapy for hemophilia A (hereditary factor VIII deficiency) is a success story of the production of coagulation factor concentrates from donor plasma. Recombinant factor concentrates, which are also produced with modified gene constructs for coagulation factor VIII in order to improve pharmacological properties, have since proven their worth. This successful development over many years of factor concentrates for the successful treatment of hemophilia patients has now been followed by the innovation of a factor VIII mimetic in the form of a monoclonal antibody, which was developed in Japan already some years back. Emicizumab is a humanized, bispecific monoclonal antibody for therapeutic use in hemophilia A. With this therapeutic agent, the treatment of the hereditary coagulation defect is based, for the first time, on a completely new active principle. The specific antibody simulates the properties of coagulation factor VIII as a cofactor for the formation of the tenase complex with the coagulation factors IX and X. As a result under steady state conditions almost normal thrombin and thus fibrin formation can be achieved.

血友病 A(遗传性第八因子缺乏症)的预防性替代疗法是利用供体血浆生产凝血因子浓缩物的成功案例。重组因子浓缩物也是用改良的凝血因子 VIII 基因构建物生产的,目的是改善药理特性。在浓缩因子多年来成功治疗血友病患者的基础上,日本早在几年前就开发出了单克隆抗体形式的 VIII 因子模拟物。Emicizumab 是一种用于治疗 A 型血友病的人源化双特异性单克隆抗体。特异性抗体模拟凝血因子 VIII 的特性,作为辅助因子与凝血因子 IX 和 X 形成tenase 复合物。
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引用次数: 0
[Equity and social determinants of health over the life course]. [生命过程中的公平和健康的社会决定因素]。
Pub Date : 2024-10-01 Epub Date: 2024-09-10 DOI: 10.1007/s00108-024-01771-7
Franz Kolland, Rebekka Rohner

Background: Socio-structural and socio-cultural change in Western societies is increasingly challenging healthcare institutions to take good care of people's health and dignity. Further and sustainable progress in health care is increasingly influenced by socio-cultural conditions. If these conditions are insufficiently taken into account, further medical progress is jeopardized.

Aim of the paper: The aim of this paper is to elucidate the significance of social conditions of health over the life course and thus to shed more light on one of the four ethical principles in medicine, namely equity.

Material: The question is addressed by a literature review, whereby the literature was reviewed from a structural theory perspective.

Results: If people feel discriminated against in terms of their age, gender, or migration background, this not only has an impact on their self-esteem, but also on their health and recovery from illness. Unfavorable economic living conditions have a negative impact on health behavior. Experiences of discrimination in the healthcare system can reduce satisfaction with treatment and contribute to non-compliance with treatment instructions.

Discussion: The socio-cultural effects mentioned above can be influenced not only by individual changes in behavior but in particular by structural and institutional change processes. There is a need for "habitus sensitivity" in both clinical and private practice, i.e., it is also part of the responsibility of doctors in the healthcare system to eliminate discrimination.

背景:西方社会的社会结构和社会文化变革正日益挑战医疗保健机构对人们的健康和尊严的关爱。医疗保健的进一步和可持续发展越来越受到社会文化条件的影响。如果不充分考虑这些条件,医疗的进一步发展就会受到损害:本文旨在阐明生命过程中健康的社会条件的重要性,从而进一步阐明医学四大伦理原则之一,即公平原则:材料:本文通过文献综述来探讨这一问题,从结构理论的角度对文献进行了综述:结果:如果人们感到在年龄、性别或移民背景方面受到歧视,这不仅会影响他们的自尊,还会影响他们的健康和疾病康复。不利的经济生活条件会对健康行为产生负面影响。在医疗系统中遭受歧视的经历会降低对治疗的满意度,并导致不遵守治疗指示:上述社会文化影响不仅会受到个人行为变化的影响,而且尤其会受到结构和制度变革过程的影响。临床和私人诊所都需要 "习惯敏感性",也就是说,消除歧视也是医疗系统中医生的责任之一。
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引用次数: 0
[An STI rarely comes alone-An overview of sexually transmitted infections]. [性传播感染很少单独出现--性传播感染概述]。
Pub Date : 2024-10-01 Epub Date: 2024-09-13 DOI: 10.1007/s00108-024-01775-3
Carola Horn-Magar, Clara Lehmann

Sexually transmitted infections (STI) or sexually transmitted diseases (STD) can be caused by bacteria, viruses, fungi, protozoa and arthropods. The most frequent bacterial STIs include Chlamydia infections, syphilis and gonorrhea; viral STIs include herpes genitalis (herpes simplex virus 1 and 2), human papillomavirus (HPV) and human immunodeficiency virus (HIV). An empathetic medical history taking in cases of a clinical suspicion can provide important information for adequate diagnostics. When an STI is detected, a complete screening for other STIs should be carried out. The STIs result in a wide variety of symptoms and it is often difficult to make a clear diagnosis to a certain pathogen based on the clinical symptoms. To avoid reinfections, it is recommended to also test recent sexual partners. For certain pathogens highly effective postexposure prophylaxis or preventive vaccinations are available.

性传播感染(STI)或性传播疾病(STD)可由细菌、病毒、真菌、原生动物和节肢动物引起。最常见的细菌性 STI 包括衣原体感染、梅毒和淋病;病毒性 STI 包括生殖器疱疹(单纯疱疹病毒 1 和 2)、人类乳头瘤病毒(HPV)和人类免疫缺陷病毒(HIV)。在临床怀疑的情况下,富有同情心的病史采集可以为充分诊断提供重要信息。当检测到性传播感染时,应进行其他性传播感染的全面筛查。性传播感染导致的症状多种多样,通常很难根据临床症状明确诊断某种病原体。为避免再次感染,建议同时对最近的性伴侣进行检测。对于某些病原体,可以采取高效的暴露后预防措施或接种预防性疫苗。
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引用次数: 0
[Situation-precise medicine : Ethical comments on the debate around precision medicine]. [情境精准医学:围绕精准医学辩论的伦理评论]。
Pub Date : 2024-10-01 Epub Date: 2024-08-28 DOI: 10.1007/s00108-024-01772-6
Andreas Lob-Hüdepohl

The label "precision medicine" appears to have the status of a "moral high-value word". Moral high-value words possess an ambivalent dialectic of light and dark. On the one hand, precision medicine promises an individual diagnosis and treatment of severe diseases focussed on the ill person with reduction of undesired side effects as far as possible. On the other hand, the label "precision medicine" rapidly leads to a gradual devaluation of everything that only seems "not precise", "vague" or simply "generally just unspecific". Precision medicine therefore nearly automatically promotes their prioritization and preference in the distribution of scarce resources, which is simultaneously associated with a deprioritization or inferiority in the distribution of scarce resources for the "not so valuable". As a further communicative weak point the limited understanding of "precision" catches the eye: precision medicine is often associated with technological high-performance medicine, which often reacts to a clear (severe) disease picture; however, many courses of diseases are still in an unclear stage or in a situation where there are clearly no (longer) perspectives for recovery and curative measures are no longer medically indicated. This article takes up on three ethical aspects, which should play an essential role in the debate on precision medicine: the uppermost criterion in the distribution of scarce resources, the relevance for patients as the uppermost aim of medical treatment and that of the patient-relevant inner understanding of the quality of life or well-being of patients.

"精准医学 "这一标签似乎具有 "道德高价值词 "的地位。道德高价值词具有明暗矛盾的辩证关系。一方面,"精准医疗 "承诺以病人为中心,对严重疾病进行个体化诊断和治疗,尽可能减少不良副作用。另一方面,"精准医学 "这一标签迅速导致一切看起来 "不精准"、"含糊不清 "或只是 "一般不具体 "的东西逐渐贬值。因此,"精准医学 "几乎自动地促进了稀缺资源分配中的优先级和优先权,而这同时又与稀缺资源分配中 "不那么有价值 "的优先级降低或劣势相关联。作为另一个传播弱点,对 "精准 "的有限理解引人注目:精准医疗通常与技术上的高效医疗联系在一起,它往往对明确(严重)的疾病情况做出反应;然而,许多疾病的病程仍处于不明确阶段,或处于明显没有(更长)康复前景的情况下,治疗措施在医学上已不再适用。本文从三个伦理方面展开论述,这三个方面应在有关精准医学的讨论中发挥重要作用:稀缺资源分配的首要标准、医疗的首要目标与患者的相关性以及患者对生活质量或福祉的内在理解。
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引用次数: 0
[GLP-1 receptor agonists for the treatment of patients with heart failure? : STEP-HFpEF and STEP-HFpEF DM]. [治疗心力衰竭患者的 GLP-1 受体激动剂:STEP-HFpEF 和 STEP-HFpEF DM]。
Pub Date : 2024-10-01 Epub Date: 2024-08-28 DOI: 10.1007/s00108-024-01763-7
Sophie Charlotte Schröder, Ulrich Laufs, Sirka Nitschmann
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引用次数: 0
[Recurrent hemolysis and iron overload of unclear origin]. [原因不明的复发性溶血和铁超载]。
Pub Date : 2024-10-01 DOI: 10.1007/s00108-024-01801-4
Laura Distelmaier, Christian Gebhard, Antje Holzäpfel, Michael von Bergwelt-Baildon, Sebastian Theurich, Holger Cario, Karsten Spiekermann

The case of a 33-year-old male with recurrent icterus and hemolysis since childhood that was long mistaken for Gilbert disease is presented. Subsequently, the patient also developed splenomegaly and gallstones together with iron overload. Genetic testing revealed the diagnosis of hereditary xerocytosis, which is an erythrocyte membrane disorder causing recurrent hemolysis. Xerocytosis is often challenging to diagnose and the frequency of the condition might be underestimated as there are often no typical findings in the microscopic differential blood count, and Eosin-5-maleimide dye (EMA) test, which is used to diagnose other erythrocyte membrane disorders, is normal. In cases of splenomegaly, iron overload and recurrent hemolysis, or in the case of a clinical diagnosis of Gilbert disease together with one of the above-mentioned symptoms, further investigations and possibly also genetic testing should be considered.

本病例是一名 33 岁的男性,自幼反复出现黄疸和溶血,长期以来被误认为是吉尔伯特病。随后,患者还出现了脾肿大和胆结石,并伴有铁超载。基因检测显示,患者被诊断为遗传性红细胞增多症,这是一种导致反复溶血的红细胞膜疾病。由于显微鉴别血细胞计数通常没有典型的发现,而且用于诊断其他红细胞膜疾病的 Eosin-5 马来酰亚胺染料(EMA)试验也正常,因此红细胞增多症的诊断往往具有挑战性,而且该病的发病率可能被低估。如果出现脾脏肿大、铁超载和反复溶血,或临床诊断为吉尔伯特病并伴有上述症状之一,则应考虑进行进一步检查,也可能进行基因检测。
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引用次数: 0
[Periaortic gas and signs of dissection of a mycotic aneurysm in the descending thoracic aorta due to Clostridium septicum]. [败血梭菌引起的降胸主动脉霉菌性动脉瘤的主动脉周围气体和夹层迹象]。
Pub Date : 2024-09-27 DOI: 10.1007/s00108-024-01800-5
Monica Maria Novoa Usme, Iskandar Atmowihardjo, Sebastian Spencker

The case of 92-year-old male with a history of sepsis due to Clostridium septicum is described. Since the bacteria were also found at the site of joint aspiration, the patient underwent knee prosthesis removal. Due to the association between colorectal cancer and Clostridium septicum, a colonoscopy was performed. An adenocarcinoma was extracted in situ. At 5 months, the patient presented again with shortness of breath, acute retrosternal pain and fever. CT revealed a mycotic aneurysm of the descending aorta with Stanford B dissection and periaortic gas. Due to the fragility of the patient, conservative treatment was initiated.

本病例描述了一名 92 岁男性因败血梭菌引起败血症的病史。由于在关节抽吸处也发现了细菌,患者接受了膝关节假体切除手术。由于结肠直肠癌与败血梭菌之间存在关联,患者接受了结肠镜检查。原位摘除了一个腺癌。5 个月后,患者再次出现呼吸急促、急性胸骨后疼痛和发烧。CT 显示降主动脉有一个霉菌性动脉瘤,伴有 Stanford B 夹层和主动脉周围气体。由于患者身体虚弱,医生开始对其进行保守治疗。
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引用次数: 0
[A rare cause of acute abdomen in a young immunocompetent woman]. [免疫功能正常的年轻女性急性腹部的罕见病因]。
Pub Date : 2024-09-01 Epub Date: 2024-05-15 DOI: 10.1007/s00108-024-01713-3
Paul Witte, Marco Rossi, Henning Fischer, Michael Christ

Streptococcus pyogenes is a human pathogenic, gram positive bacterium that primarily leads to pharyngitis or soft tissue infections. Primary peritonitis caused by S. pyogenes infection is rare and there are only a few published cases worldwide. Primary peritonitis due to other pathogens occurs in immunosuppressed conditions such as HIV or other chronic diseases. However, younger, healthy women are more likely to be affected by S. pyogenes peritonitis. At present, the underlying molecular mechanisms can only be speculated on. One possibility is that, similar to the clinical picture of streptococcal toxic shock syndrome (STSS), a specific serotype of the M protein in combination with inhibition of the cell response of neutrophil granulocytes could play a role. In addition to peritonitis, the clinical picture may include other organ manifestations such as acute kidney damage or circulatory dysregulation. In terms of treatment, rapid pathogen-directed empirical antibiotic therapy is the treatment of choice. If there is no indication of secondary peritonitis, diagnostic laparoscopy can be dispensed with in the further diagnostic work-up.

化脓性链球菌是一种人类致病性革兰氏阳性细菌,主要导致咽炎或软组织感染。由化脓性链球菌感染引起的原发性腹膜炎非常罕见,全世界仅有几例公开发表的病例。其他病原体引起的原发性腹膜炎多发生在免疫抑制的情况下,如艾滋病毒或其他慢性疾病。然而,年轻、健康的女性更容易受到化脓性葡萄球菌腹膜炎的影响。目前,只能推测其潜在的分子机制。一种可能是,与链球菌中毒性休克综合征(STSS)的临床表现类似,M 蛋白的特定血清型与抑制中性粒细胞的细胞反应相结合,可能在其中发挥了作用。除腹膜炎外,临床表现还可能包括其他器官表现,如急性肾损伤或循环失调。在治疗方面,以病原体为导向的快速经验性抗生素治疗是首选疗法。如果没有继发性腹膜炎的迹象,在进一步的诊断检查中可以不进行诊断性腹腔镜检查。
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引用次数: 0
[Catheter-assisted local lysis therapy for submassive pulmonary embolism]. [导管辅助局部溶栓疗法治疗亚大块肺栓塞]。
Pub Date : 2024-09-01 Epub Date: 2024-07-09 DOI: 10.1007/s00108-024-01736-w
Rainer Waßmer, Marie Koch, Evelyn Trips, Matthias Filz, Claudia Bräsel, Martin Meister, Xina Grählert, Torsten Fuß

Background: Pulmonary embolism is the third most common cardiovascular disease. Interventional treatment options as an alternative to systemic lysis therapy of hemodynamically stable, submassive pulmonary embolisms have received an unprecedented boost in innovation in recent years. The treatment options are heterogeneous and can be roughly divided into local thrombolysis and local thrombectomy. For years in our center we have been carrying out catheter-assisted, locoregional lysis therapy with side-hole lysis catheters and a cumulative dose per pulmonary branch of 10 mg alteplase over 15 h for hemodynamically stable, submassive pulmonary emboli.

Aim: The aim of this retrospective study was to review this therapeutic concept and to collect data on clinical endpoints and possible complications.

Methods: The study included data from 01/2018-03/2023. For this purpose, the patients were selected based on the OPS codes (8.838.60 and 1‑276.0), and the data was collected using the medical records. Biometric data, data on previous illnesses and vital parameters, laboratory chemistry data, CT diagnostic data, echocardiographic data, data on drug treatment and data on complications were collected anonymously.

Results: There was a significant reduction in the strain on the right heart. Peripheral oxygen saturation also improved significantly and heart rate decreased significantly. The complication rate remained low and was almost exclusively limited to access-related problems.

Conclusion: Catheter-assisted, locoregional lysis therapy is a safe and effective treatment method for submassive pulmonary embolism.

背景:肺栓塞是第三大常见心血管疾病:肺栓塞是第三大常见心血管疾病。近年来,作为全身溶栓治疗的替代方案,介入治疗在治疗血流动力学稳定的亚肿块型肺栓塞方面得到了前所未有的创新。治疗方法多种多样,大致可分为局部溶栓和局部血栓切除术。多年来,我们中心一直使用侧孔溶栓导管进行导管辅助局部溶栓治疗,并在15小时内对血流动力学稳定的亚严重肺栓塞患者每个肺动脉分支累计注射10毫克阿替普酶:研究包括2018年1月至2023年3月的数据。为此,根据 OPS 编码(8.838.60 和 1-276.0)选择患者,并通过病历收集数据。生物特征数据、既往疾病和生命参数数据、实验室化学数据、CT 诊断数据、超声心动图数据、药物治疗数据和并发症数据均以匿名方式收集:右心负荷明显减轻。结果:右心负荷明显减轻,外周血氧饱和度明显改善,心率明显下降。并发症发生率仍然很低,而且几乎仅限于与入路相关的问题:结论:导管辅助局部溶栓疗法是治疗亚大块肺栓塞的一种安全有效的方法。
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引用次数: 0
[New treatment options for primary biliary cholangitis]. [原发性胆汁性胆管炎的新治疗方案]。
Pub Date : 2024-09-01 Epub Date: 2024-08-21 DOI: 10.1007/s00108-024-01730-2
Verena Keitel-Anselmino, Sirka Nitschmann
{"title":"[New treatment options for primary biliary cholangitis].","authors":"Verena Keitel-Anselmino, Sirka Nitschmann","doi":"10.1007/s00108-024-01730-2","DOIUrl":"10.1007/s00108-024-01730-2","url":null,"abstract":"","PeriodicalId":73385,"journal":{"name":"Innere Medizin (Heidelberg, Germany)","volume":" ","pages":"962-964"},"PeriodicalIF":0.0,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142019734","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
Innere Medizin (Heidelberg, Germany)
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