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Ion Channel Diseases as a Cause of Sudden Cardiac Death in Young People: Aspects of Their Diagnosis, Treatment, and Pathogenesis. 导致年轻人心脏性猝死的离子通道疾病:诊断、治疗和发病机制。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-10-04 DOI: 10.3238/arztebl.m2024.0130
Ibrahim El-Battrawy, Andreas Mügge, Ibrahim Akin, Huu Phuc Nguyen, Hendrik Milting, Assem Aweimer
{"title":"Ion Channel Diseases as a Cause of Sudden Cardiac Death in Young People: Aspects of Their Diagnosis, Treatment, and Pathogenesis.","authors":"Ibrahim El-Battrawy, Andreas Mügge, Ibrahim Akin, Huu Phuc Nguyen, Hendrik Milting, Assem Aweimer","doi":"10.3238/arztebl.m2024.0130","DOIUrl":"10.3238/arztebl.m2024.0130","url":null,"abstract":"<p><strong>Background: </strong>Sudden cardiac death (SCD) is the death of an apparently healthy person within one hour of the onset of symptoms, or within 24 hours of last being seen alive and well-with no evidence of an extra-cardiac cause. In autopsied cases, SCD is defined as the natural unexpected death of unknown or cardiac cause. The reported incidence figures for SCD vary widely.</p><p><strong>Methods: </strong>This review is based on clinical registry studies, metaanalyses, randomized controlled trials, systematic reviews, and current guidelines that were retrieved by a selective search in PubMed employing the key words \"channelopathy,\" \"Brugada syndrome,\" \"long QT syndrome,\" \"catecholaminergic polymorphic ventricular tachycardia,\" \"short QT syndrome,\" and \"early repolarization.\"</p><p><strong>Results: </strong>Approximately 18% of cases of SCD in young persons are associated with cardiac channelopathy. The most common ion channel diseases affecting the heart are long QT syndrome and Brugada syndrome. The diagnosis is established by specific ECG abnormalities in the absence of structural heart disease. These can be unmasked by various maneuvers, e.g., the administration of sodium-channel blockers in Brugada syndrome. Imaging studies such as echocardiography, coronary angiography, and computed tomography are used to rule out structural heart disease and coro nary artery disease. Long-term ECG and risk stratification scores can be useful aids to therapeutic decision-making. For some of these diseases, it is advisable for the patient to avoid particular triggers of ECG changes and cardiac arrhythmias in his or her everyday life. The near relatives of persons with congenital ion channel diseases should undergo clinical and gen etic screening to protect them from SCD.</p><p><strong>Conclusion: </strong>The affected families should be investigated systematically so that appropriate diagnoses and treatments can be established.</p>","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":" Forthcoming","pages":"665-672"},"PeriodicalIF":6.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141497423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Long-Term Cure of Patients With Hereditary Medullary Thyroid Carcinoma: 40 Years of Follow-Up in a Single Center. 遗传性甲状腺髓样癌患者的长期治愈:一个中心40年的随访研究
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-10-04 DOI: 10.3238/arztebl.m2024.0174
Friedhelm Raue, Thomas Bruckner, Karin Frank-Raue
{"title":"The Long-Term Cure of Patients With Hereditary Medullary Thyroid Carcinoma: 40 Years of Follow-Up in a Single Center.","authors":"Friedhelm Raue, Thomas Bruckner, Karin Frank-Raue","doi":"10.3238/arztebl.m2024.0174","DOIUrl":"10.3238/arztebl.m2024.0174","url":null,"abstract":"<p><strong>Background: </strong>The cure rate of patients with hereditary medullary thyroid carcionoma (MTC) can be decisively improved by screening for elevated calcitonin (Ctn) levels and RET gene mutations in patients from families affected by multiple endocrine neoplasia type 2 (MEN2), followed by prophylactic thyroidectomy in persons with mutated RET genes. In this long-term observational study, we investigated whether postoperative cures are indeed maintained decades after the procedure.</p><p><strong>Methods: </strong>From 1979 to 2021, 277 patients with MEN2 who underwent thyroidectomy were observed postoperatively for 14.4 ± 10.3 years (mean, standard deviation). They were classified as either cured or not cured depending on the last measured serum Ctn level (cured, Ctn < 10 pg/mL or < 2 pg/mL; not cured, Ctn ≥ 10 pg/mL). Depending on their RET mutation status, they were categorized as moderate, high, or highest risk (121, 130, and 26 patients, respec - tively).</p><p><strong>Results: </strong>154 patients (55.6%) obtained a long-term cure (Ctn <10 pg/mL). The median age at surgery was 27, 14, and 4 years in patients at moderate, high, and highest risk. All 52 patients who had undergone prophylactic thyroidectomy before the age of 6 years, 9 years, or 6 months had a Ctn level below 2 pg/mL and were cured at the end of the follow-up period. In a multivariable analysis, prognostic factors for a long-term cure were a lower tumor stage and, by tendency, classification as belonging to the moderate as opposed to the highest-risk group.</p><p><strong>Conclusion: </strong>In patients receiving an early diagnosis of MEN2 via family screening, prophylactic thyroidectomy taking into account the RET mutation risk group can achieve a long-term cure of MTC with undetectable serum Ctn levels.</p>","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":" Forthcoming","pages":"657-664"},"PeriodicalIF":6.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142281906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Long-Term Clinical Studies in the Outpatient Sector-Medullary Carcinoma of the Thyroid as an Example. 长期临床研究在门诊部门-甲状腺髓样癌为例。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-10-04 DOI: 10.3238/arztebl.m2024.0185
Matthias Kroiß
{"title":"Long-Term Clinical Studies in the Outpatient Sector-Medullary Carcinoma of the Thyroid as an Example.","authors":"Matthias Kroiß","doi":"10.3238/arztebl.m2024.0185","DOIUrl":"https://doi.org/10.3238/arztebl.m2024.0185","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 20","pages":"655-656"},"PeriodicalIF":6.5,"publicationDate":"2024-10-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142969982","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Necrotizing Soft Tissue Infection Due to Vibrio vulnificus After Bathing in the Baltic Sea. 在波罗的海沐浴后因弧菌引起的坏死性软组织感染》(Necrotizing Soft Tissue Infection Due to Vibrio vulnificus After Bathing in the Baltic Sea)。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0058
Daniela Tominski, Dinah von Schöning, Martin Franz
{"title":"Necrotizing Soft Tissue Infection Due to Vibrio vulnificus After Bathing in the Baltic Sea.","authors":"Daniela Tominski, Dinah von Schöning, Martin Franz","doi":"10.3238/arztebl.m2024.0058","DOIUrl":"10.3238/arztebl.m2024.0058","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 19","pages":"654"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741544/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869414","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Recognizing Statistical Problems in Reports of Clinical Trials: a Readers' Aid. Part 33 of a Series on Evaluation of Scientific Publications. 认识临床试验报告中的统计问题:读者指南》。科学出版物评估系列丛书第 33 部分。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0113
Anna Suling, Anika Großhennig
{"title":"Recognizing Statistical Problems in Reports of Clinical Trials: a Readers' Aid. Part 33 of a Series on Evaluation of Scientific Publications.","authors":"Anna Suling, Anika Großhennig","doi":"10.3238/arztebl.m2024.0113","DOIUrl":"10.3238/arztebl.m2024.0113","url":null,"abstract":"<p><strong>Background: </strong>Readers of clinical trial reports should be able to critically evaluate the design, results, and conclusions of the trial. There are internationally accepted guidelines that define methodological standards for trial planning, statistical methods, and the display and interpretation of the results. Publications may nonetheless contain erroneous findings and interpretations.</p><p><strong>Methods: </strong>Statistical errors can arise in the planning of the trial, the analysis and display of the results, as well as in the interpretation of p-values and treatment effects in experimental and observational clinical trials. A useful aid for readers of medical publications should include a description of the potential statistical problems without complex theoretical background information. With this aim, we discuss certain major types of statistical error that the reader should be familiar with in order to be able to interpret the conclusions of these publications more easily.</p><p><strong>Results: </strong>Statistical errors can already arise at an early stage through the choice of the wrong question to be addressed or the wrong population to be analyzed; such errors will inevitably have consequences. Before the start of any clinical trial, a primary endpoint must be defined, the sample size must be calculated, and the trial must be appropriately registered (among other requirements). With regard to the analysis, readers should for example take into account whether a statistical analysis plan with an intention-to-treat analysis existed for the study in question. They must be able to recognize erroneous methods of displaying and comparing data, confounding, as well as incorrect interpretations of p-values, and should take these problems into account when interpreting the findings. The problem of invalid causal inferences is not restricted to observational studies.</p><p><strong>Conclusion: </strong>Statistical errors do, indeed, arise. They should be detected as early as possible in various test instances. Nonetheless, readers should be able to judge independently whether the published clinical trial reflects meticulous and correct trial planning, appropriate display of the trial's results, and a proper, reasoned interpretation of the findings. The published checklists are a good aid for this purpose.</p>","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":" Forthcoming","pages":"634-638"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741548/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141455885","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Splenic Rupture After Colonoscopy. 结肠镜检查后脾破裂。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0041
Karsten Müssig, Otfried Sauer, Petr Ziegler
{"title":"Splenic Rupture After Colonoscopy.","authors":"Karsten Müssig, Otfried Sauer, Petr Ziegler","doi":"10.3238/arztebl.m2024.0041","DOIUrl":"10.3238/arztebl.m2024.0041","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 19","pages":"654"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741545/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869415","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Daptomycin-Induced Eosinophilic Pneumonitis. 达托霉素诱发的嗜酸性细胞性肺炎
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0101
Polyxeni Vlachea, Florian Herrmann
{"title":"Daptomycin-Induced Eosinophilic Pneumonitis.","authors":"Polyxeni Vlachea, Florian Herrmann","doi":"10.3238/arztebl.m2024.0101","DOIUrl":"10.3238/arztebl.m2024.0101","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 19","pages":"626"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741547/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869412","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The Diagnosis and Treatment of Chronic Rhinosinusitis. 慢性鼻炎的诊断和治疗。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0167
Tanja Hildenbrand, Katrin Milger-Kneidinger, Ingo Baumann, Rainer Weber
{"title":"The Diagnosis and Treatment of Chronic Rhinosinusitis.","authors":"Tanja Hildenbrand, Katrin Milger-Kneidinger, Ingo Baumann, Rainer Weber","doi":"10.3238/arztebl.m2024.0167","DOIUrl":"10.3238/arztebl.m2024.0167","url":null,"abstract":"<p><strong>Background: </strong>Chronic rhinosinusitis (CRS) is a heterogeneous condition characterized by local chronic inflammation of the mucous membranes of the nose and paranasal sinuses. It affects approximately 5% of the population.</p><p><strong>Methods: </strong>This review is based on relevant publications retrieved by a selective search of the literature, with particular attention to current national and international guidelines.</p><p><strong>Results: </strong>CRS is defined by, and diagnosed on the basis of, a combination of symptoms and objective findings of nasal endoscopy and imaging studies. It markedly impairs quality of life and gives rise to both direct and indirect health care costs. In 20-45% of cases, CRS is associated with comorbid bronchial asthma and a significantly elevated risk of further diseases (e.g., COPD, OR 1.73; depression, HR 1.50; obstructive sleep apnea, OR 1.91; carcinoma, OR 1.14-5.30). CRS is primarily treated medically with topical steroids (standardized mean difference of nasal symptoms, -0.63 (95% confidence interval [-0.89; -0.37]; standardized mean difference of quality of life as measured by SNOT -22, -5.46 [-8.08; -2.84]), as well as with nasal lavage and, as an option, systemic steroids (and antibiotics where appropriate). If appropriate medical treatment fails to bring about adequate and sustained improvement, endoscopic sinus surgery is indicated. This improves the individual symptoms, the overall symptom score, and patients' quality of life. Severe refractory CRS with nasal polyposis can be treated with biological agents.</p><p><strong>Conclusion: </strong>CRS calls for individually adapted medical and/or surgical treatment.</p>","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":" Forthcoming","pages":"643-653"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741551/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142035529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cystic Biliary Atresia—A Single-Center, Retrospective Analysis. 胆囊胆道闭锁:单中心回顾性分析。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0114
Omid Madadi-Sanjani, Vincent Rohrbacher, Marie Uecker
{"title":"Cystic Biliary Atresia—A Single-Center, Retrospective Analysis.","authors":"Omid Madadi-Sanjani, Vincent Rohrbacher, Marie Uecker","doi":"10.3238/arztebl.m2024.0114","DOIUrl":"10.3238/arztebl.m2024.0114","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 19","pages":"641-642"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741552/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869411","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Detection of Elevated Risk for Drug-Related Problems in the Hospital—The AMTS2 Risk Score. 检测医院内药物相关问题的高风险--AMTS2 风险评分。
IF 6.5 2区 医学
Deutsches Arzteblatt international Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0115
Lea Jung-Poppe, Barbara Pfistermeister, Hagen Fabian Nicolaus, Anna Roggenhofer, Anna Altenbuchner, Wahram Andrikyan, Armin Ströbel, Christine Schnitzer, Harald Dormann, Renke Maas
{"title":"Detection of Elevated Risk for Drug-Related Problems in the Hospital—The AMTS2 Risk Score.","authors":"Lea Jung-Poppe, Barbara Pfistermeister, Hagen Fabian Nicolaus, Anna Roggenhofer, Anna Altenbuchner, Wahram Andrikyan, Armin Ströbel, Christine Schnitzer, Harald Dormann, Renke Maas","doi":"10.3238/arztebl.m2024.0115","DOIUrl":"10.3238/arztebl.m2024.0115","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 19","pages":"639-640"},"PeriodicalIF":6.5,"publicationDate":"2024-09-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11741550/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142869413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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