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The Diagnosis and Treatment of Chronic Rhinosinusitis. 慢性鼻炎的诊断和治疗。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0167
Tanja Hildenbrand, Katrin Milger-Kneidinger, Ingo Baumann, Rainer Weber

Background: 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.

Methods: This review is based on relevant publications retrieved by a selective search of the literature, with particular attention to current national and international guidelines.

Results: 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.

Conclusion: CRS calls for individually adapted medical and/or surgical treatment.

背景:慢性鼻窦炎(CRS)是一种以鼻腔和副鼻窦粘膜局部慢性炎症为特征的异质性疾病。约有 5%的人口受其影响:方法:本综述基于通过选择性文献检索获得的相关出版物,尤其关注当前的国家和国际指南:结果:CRS 是根据症状和鼻内窥镜检查及影像学检查的客观结果综合定义和诊断的。CRS 严重影响患者的生活质量,并产生直接和间接的医疗费用。在 20%-45% 的病例中,CRS 与合并支气管哮喘有关,且罹患其他疾病的风险显著升高(如慢性阻塞性肺病,OR 1.73;抑郁症,HR 1.50;阻塞性睡眠呼吸暂停,OR 1.91;癌症,OR 1.14-5.30)。CRS 主要采用局部类固醇药物治疗(鼻部症状的标准化平均差异为 -0.63(95% 置信区间 [-0.89;-0.37];以 SNOT -22 衡量的生活质量的标准化平均差异为 -5.46 [-8.08;-2.84]),以及鼻腔灌洗和全身性类固醇药物(适当时还可使用抗生素)。如果适当的药物治疗未能带来充分和持续的改善,则应进行鼻旁窦内窥镜手术。这可以改善个别症状、总体症状评分和患者的生活质量。严重的难治性CRS合并鼻息肉可采用生物制剂治疗:结论:CRS 需要因人而异的药物和/或手术治疗。
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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 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0114
Omid Madadi-Sanjani, Vincent Rohrbacher, Marie Uecker
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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 Q1 MEDICINE, GENERAL & INTERNAL 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
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引用次数: 0
The Care of Children and Adolescents with Chronic Inflammatory Bowel Disease: A Cluster-Randomized Trial on Improving the Guideline Conformity of Treatment by the Use of the CEDATA-GPGE Patient Registry. 儿童和青少年慢性炎症性肠病患者的护理:利用 CEDATA-GPGE 患者登记册改善治疗指南一致性的分组随机试验》。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0168
Luisa Tischler, Aletta Boerkoel, Heiko Krause, Neeltje van den Berg, Jan de Laffolie

Background: For children and adolescents with chronic inflammatory bowel disease (IBD), treatment that is not in adequate conformity with the guidelines can adversely affect both the course of disease and the patients' development. The targeted use of digital patient registries may improve real-life adherence to the recommendations of evidence-based guidelines.

Methods: In a cluster-randomized, controlled trial (DRKS00015505), treatment providers for the intervention group (IG) documented the treatment of children and adolescents with IBD in the CEDATA-GPGE patient registry; they received automated feedback on the data they entered and on potential deviations of the documented treatment from recommendations contained in the guidelines (care deficits). Treatments providers for the control group (CG) documented treatments as previously, i.e., only in the patients' charts. At the end of a twelve-month observation period, the data from both groups at baseline and on follow-up were analyzed in an intergroup comparison. The primary endpoint was the number of care deficits at twelve months.

Results: 319 patients were recruited from 47 pediatric gas troen tero logical centers in Germany (IG: 21 centers and 160 subjects; CG: 26 centers and 159 subjects). Among the 146 subjects in the IG who were followed up at 12 months, there were an average (mean) of 0.17 care deficits per patient (95% confidence interval [0.10; 0.24]). Among the 134 subjects in the CG who were followed up at 12 months, there were an average (mean) of 0.55 [0.43; 0.66] identified care deficits per patient (p < 0.0001).

Conclusion: Registry-based feedback can help bring treatment and its documentation into better con for - mity with the relevant guidelines and thereby reduce or prevent care deficits in children and adolescents with IBD.

背景:对于患有慢性炎症性肠病(IBD)的儿童和青少年来说,如果治疗不完全符合指南的要求,就会对病程和患者的发展产生不利影响。有针对性地使用数字患者登记册可改善现实生活中对循证指南建议的遵守情况:在一项分组随机对照试验(DRKS00015505)中,干预组(IG)的治疗提供者在 CEDATA-GPGE 患者登记册中记录了对患有 IBD 的儿童和青少年的治疗情况;他们会收到关于所输入数据的自动反馈,以及关于所记录治疗与指南中建议的潜在偏差(护理缺陷)的自动反馈。对照组(CG)的治疗提供者则像以前一样记录治疗,即只在患者病历中记录。在为期 12 个月的观察期结束时,对两组基线数据和随访数据进行组间比较分析。主要终点是十二个月时护理缺陷的数量:德国 47 家儿科胃肠病中心共招募了 319 名患者(IG:21 家中心,160 名受试者;CG:26 家中心,159 名受试者)。在随访 12 个月的 146 名 IG 受试者中,平均每名患者的护理缺陷为 0.17(平均值)(95% 置信区间 [0.10; 0.24])。在随访12个月的134名CG受试者中,每名患者平均(平均)发现0.55 [0.43; 0.66]个护理缺陷(P < 0.0001):以登记为基础的反馈有助于使治疗及其记录更加符合相关指南,从而减少或预防儿童和青少年 IBD 患者的护理缺陷。
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引用次数: 0
The Incidence of Endometriosis, 2014–2022. An Analysis of Nationwide Claims Data From Physicians in Private Practice. 2014-2022 年子宫内膜异位症的发病率。对全国私人诊所医生索赔数据的分析。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-20 DOI: 10.3238/arztebl.m2024.0160
Claudia Kohring, Manas K Akmatov, Jakob Holstiege, Iris Brandes, Sylvia Mechsner

Background: The epidemiological characterization of endometri - osis, particularly with regard to its incidence, has been inadequate to date both in Germany and other countries. The goal of this study was to determine trends in the incidence of diagnosed endometri - osis and changes in age structure at the time of first diagnosis over the period 2014-2022.

Methods: Nationwide claims data from physicians in private practice, obtained according to relevant German law (§ 295 SGB V), were used to identify the population at risk for a first assured diagnosis of endometriosis (ICD-10-GM: N80) during each year of the study period, consisting of women and girls aged 10-52 who were insured by the statutory health insurance system and for whom at least two years of prior observation were possible. Patients were defined as incident if they were documented as having received a first confirmed diagnosis of endometriosis, according to the case definition, during the study year. The case definition comprised multiple options for validating the diagnosis.

Results: The incidence of diagnosed endometriosis rose over the period of the study from 2.8 per 1000 persons at risk in 2014 to 4.1 per 1000 in 2022, corresponding to a 44% relative increase. There was also a marked shift in agespecific incidence toward higher values at younger ages: the median age at diagnosis fell from 37 years (2014) to 34 (2022).

Conclusion: This is the first study providing nationwide population-based data on the incidence of endometriosis in Germany. The observed rise in newly diagnosed cases is presumably mainly due to an increased awareness of endometriosis and to the growing recognition of the disease.

背景:迄今为止,德国和其他国家对子宫内膜异位症的流行病学特征,尤其是其发病率方面的研究尚不充分。本研究旨在确定 2014-2022 年间确诊子宫内膜异位症发病率的趋势以及首次确诊时年龄结构的变化:根据德国相关法律(§ 295 SGB V)从私人执业医师处获得的全国性理赔数据,用于确定研究期间每年首次确诊子宫内膜异位症的高危人群(ICD-10-GM:N80),这些人群包括年龄在 10-52 岁之间、参加了法定医疗保险系统且至少有两年之前的观察记录的妇女和女孩。根据病例定义,在研究年度内首次确诊子宫内膜异位症的患者被定义为病例。病例定义包括多个确诊选项:在研究期间,确诊子宫内膜异位症的发病率从2014年的每1000名高危人群中2.8例上升到2022年的每1000名高危人群中4.1例,相对增幅为44%。年龄特异性发病率也明显转向年轻时的较高值:确诊年龄中位数从 37 岁(2014 年)下降到 34 岁(2022 年):这是第一项提供德国子宫内膜异位症发病率全国人口数据的研究。新诊断病例的增加可能主要是由于人们对子宫内膜异位症的认识有所提高,以及对该疾病的认识不断加深。
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引用次数: 0
The Conservative and Operative Treatment of Carpal Fractures. 腕骨骨折的保守和手术治疗。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-06 DOI: 10.3238/arztebl.m2024.0102
Adrian Cavalcanti Kußmaul, Titus Kuehlein, Martin F Langer, Ali Ayache, Steffen Löw, Frank Unglaub

Background: Carpal fractures (incidence: 30-60 per 100 000 persons per year) are one of the more commonly overlooked fracture types. They can have serious consequences, as the use of the hand is indispensable in everyday life. In the following article, we present the elements of the diagnosis and treatment of fractures of the carpal bones.

Methods: This review is based on meta-analyses and randomized controlled trials (RCTs) published from 2013 to 2023 that were retrieved by a structured literature search, supplemented by guideline recommendations and expert consensus statements. In addition, data on the administrative prevalence of carpal fractures were obtained from the German Association of Statutory Health Insurance Physicians (Kassenärztliche Vereinigung, KV) and from the German Statutory Accident Insurance (Deutsche Gesetzliche Unfallversicherung, DGUV).

Results: The administrative prevalence of carpal fractures in 2022 was 44 496 outpatient cases (KV, DGUV) in one year. After clinical history-taking, physical examination and x-ray have been performed, thin-slice computed tomography is recommended as part of the diagnostic evaluation. Treatment recommendations are based on evidence of levels II to IV. Multiple RCTs have been carried out on the treatment of scaphoid fractures, and a clinical guideline exists. Proximal, dislocated and unstable scaphoid fractures should be treated surgically. Non-displaced or minimally displaced fractures of the middle third of the scaphoid bone require a shorter period of immobilization with surgical treatment (2-4 weeks) than with conservative treatment (6-8 weeks). The use of plaster casts that do not hinder elbow and thumb mobility yields healing rates similar to those obtained with the immobi - lization of both of these joints. Failure to treat an unrecognized scaphoid fracture can lead to pseudarthrosis, avascular bony necrosis, and misalignment. Other, rarer types of carpal fractures must be managed on an individual basis, as the available ev idence is limited to expert consensus.

Conclusion: Early recognition and appropriate treatment of carpal fractures lead to healing in more than 90% of cases. Although the available evidence on their proper treatment is growing, many questions are subject to expert consensus, and decisions about treatment must be made individually.

背景:腕骨骨折(发病率:每年每 10 万人中有 30-60 例)是较常被忽视的骨折类型之一。由于手的使用在日常生活中不可或缺,因此腕骨骨折可能造成严重后果。在下面的文章中,我们将介绍腕骨骨折的诊断和治疗要点:本综述以 2013 年至 2023 年发表的荟萃分析和随机对照试验(RCT)为基础,通过结构化文献检索,并辅以指南建议和专家共识声明。此外,还从德国法定医疗保险医生协会(Kassenärztliche Vereinigung,KV)和德国法定意外保险(Deutsche Gesetzliche Unfallversicherung,DGUV)获得了腕骨骨折行政流行率的数据:2022 年腕骨骨折的行政发病率为 44 496 例门诊病例(KV、DGUV)。在进行临床病史采集、体格检查和 X 光检查后,建议将薄层计算机断层扫描作为诊断评估的一部分。治疗建议以 II 至 IV 级证据为基础。针对肩胛骨骨折的治疗已进行了多项研究,并制定了临床指南。近端、脱位和不稳定的肩胛骨骨折应进行手术治疗。与保守治疗(6-8 周)相比,手术治疗(2-4 周)所需的固定时间更短。使用不妨碍肘部和拇指活动的石膏模型,其愈合率与固定这两个关节的愈合率相近。如果未能及时治疗肩胛骨骨折,可能会导致假关节、无血管性骨坏死和错位。其他罕见类型的腕骨骨折必须根据个体情况进行治疗,因为现有证据仅限于专家共识:结论:腕骨骨折的早期识别和适当治疗可使 90% 以上的病例痊愈。结论:早期识别和适当治疗可使 90% 以上的病例痊愈。尽管有关适当治疗的现有证据越来越多,但许多问题仍有待专家达成共识,因此必须根据个人情况做出治疗决定。
{"title":"The Conservative and Operative Treatment of Carpal Fractures.","authors":"Adrian Cavalcanti Kußmaul, Titus Kuehlein, Martin F Langer, Ali Ayache, Steffen Löw, Frank Unglaub","doi":"10.3238/arztebl.m2024.0102","DOIUrl":"10.3238/arztebl.m2024.0102","url":null,"abstract":"<p><strong>Background: </strong>Carpal fractures (incidence: 30-60 per 100 000 persons per year) are one of the more commonly overlooked fracture types. They can have serious consequences, as the use of the hand is indispensable in everyday life. In the following article, we present the elements of the diagnosis and treatment of fractures of the carpal bones.</p><p><strong>Methods: </strong>This review is based on meta-analyses and randomized controlled trials (RCTs) published from 2013 to 2023 that were retrieved by a structured literature search, supplemented by guideline recommendations and expert consensus statements. In addition, data on the administrative prevalence of carpal fractures were obtained from the German Association of Statutory Health Insurance Physicians (Kassenärztliche Vereinigung, KV) and from the German Statutory Accident Insurance (Deutsche Gesetzliche Unfallversicherung, DGUV).</p><p><strong>Results: </strong>The administrative prevalence of carpal fractures in 2022 was 44 496 outpatient cases (KV, DGUV) in one year. After clinical history-taking, physical examination and x-ray have been performed, thin-slice computed tomography is recommended as part of the diagnostic evaluation. Treatment recommendations are based on evidence of levels II to IV. Multiple RCTs have been carried out on the treatment of scaphoid fractures, and a clinical guideline exists. Proximal, dislocated and unstable scaphoid fractures should be treated surgically. Non-displaced or minimally displaced fractures of the middle third of the scaphoid bone require a shorter period of immobilization with surgical treatment (2-4 weeks) than with conservative treatment (6-8 weeks). The use of plaster casts that do not hinder elbow and thumb mobility yields healing rates similar to those obtained with the immobi - lization of both of these joints. Failure to treat an unrecognized scaphoid fracture can lead to pseudarthrosis, avascular bony necrosis, and misalignment. Other, rarer types of carpal fractures must be managed on an individual basis, as the available ev idence is limited to expert consensus.</p><p><strong>Conclusion: </strong>Early recognition and appropriate treatment of carpal fractures lead to healing in more than 90% of cases. Although the available evidence on their proper treatment is growing, many questions are subject to expert consensus, and decisions about treatment must be made individually.</p>","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":" Forthcoming","pages":"594-600"},"PeriodicalIF":6.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11661489/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141305672","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
In Reply. 回复中。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-06 DOI: 10.3238/arztebl.m2024.0093
Daniel Kotz
{"title":"In Reply.","authors":"Daniel Kotz","doi":"10.3238/arztebl.m2024.0093","DOIUrl":"10.3238/arztebl.m2024.0093","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 18","pages":"612-613"},"PeriodicalIF":6.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11661476/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142827371","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
Unfortunate Terminology. 不幸的术语。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-06 DOI: 10.3238/arztebl.m2024.0161
Thomas Dietz, Nicola Riedel-Kusch, Christian Tschirch
{"title":"Unfortunate Terminology.","authors":"Thomas Dietz, Nicola Riedel-Kusch, Christian Tschirch","doi":"10.3238/arztebl.m2024.0161","DOIUrl":"10.3238/arztebl.m2024.0161","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 18","pages":"618"},"PeriodicalIF":6.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11661478/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142827311","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
Hereditary Hemorrhagic Telangiectasia (Osler's Disease): Systemic, Interdisciplinary, Relatively Common—and Often Missed. 遗传性出血性远端血管扩张症(奥斯勒氏病):系统性、跨学科、相对常见且经常被忽视。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-06 DOI: 10.3238/arztebl.m2024.0111
Urban W Geisthoff, Andreas H Mahnken, Ulrike W Denzer, André Kemmling, Christopher Nimsky, Boris A Stuck

Background: Hereditary hemorrhagic telangiectasia (HHT, Rendu- Osler-Weber disease, or Osler's disease for short) is a systemic disease that can severely impair the quality of life and that requires interdisciplinary treatment. Among rare diseases, it is relatively common, with a prevalence of approximately 1/5000.

Methods: This review is based on publications retrieved by a selective literature search, including the two international guidelines on clinically relevant aspects of HHT.

Results: On average, about two decades elapse between the initial symptoms and the diagnosis of HHT. 95% of patients have nosebleeds; these usually begin before age 20 but can occur at any time, from infancy to old age. The diagnosis is usually made on clinical grounds on the basis of the characteristic telangiectases, a positive family history, and possible involvement of the gastrointestinal tract, lungs, liver, and brain. Nosebleeds can sometimes be reduced by outpatient measures including counseling on keeping the nose moist (expert consensus), self-application of a nasal packing (which improves the quality of life, according to an online survey), and the prescription of tranexamic acid (reduction of nosebleeds from 17.3% [5.5; 27.6] to 54%). In particular, screening (expert consensus) for pulmonary vascular malformations (frequency 10-50%) can prevent many adverse outcomes. If pulmonary vascular malformations cannot be ruled out, antibiotic prophylaxis is recommended before medical procedures that can cause bacteremia (expert consensus).

Conclusion: Broad awareness of the condition, early diagnosis, and interdisciplinary treatment improve the quality of life and ultimate outcome of persons with HHT. Nevertheless, there are few options supported by good evidence for the appropriate treatment of this rare, often serious disease..

背景:遗传性出血性毛细血管扩张症(HHT,Rendu-Osler-Weber 病,简称奥斯勒病)是一种严重影响生活质量的系统性疾病,需要跨学科治疗。在罕见病中,该病较为常见,发病率约为 1/5000:方法:本综述基于选择性文献检索所检索到的出版物,包括两份关于高血压先天性心脏病临床相关方面的国际指南:从最初出现症状到确诊为 HHT,平均需要二十年的时间。95% 的患者会流鼻血,通常在 20 岁之前开始,但从婴儿期到老年期的任何时候都可能发生。诊断通常是根据特征性毛细血管扩张、阳性家族史以及胃肠道、肺部、肝脏和脑部可能受累等临床表现做出的。通过门诊措施有时可以减少鼻出血,这些措施包括保持鼻腔湿润的咨询(专家共识)、自行使用鼻腔填塞物(根据一项在线调查,这可以提高生活质量)以及处方氨甲环酸(鼻出血率从 17.3% [5.5; 27.6] 降至 54%)。尤其是肺血管畸形筛查(专家共识)(频率为 10%-50%)可以预防许多不良后果。如果不能排除肺血管畸形,建议在进行可能导致菌血症的医疗程序前使用抗生素预防(专家共识):结论:对该疾病的广泛认识、早期诊断和跨学科治疗可改善 HHT 患者的生活质量和最终结果。尽管如此,对于这种罕见的严重疾病的适当治疗,有充分证据支持的方案仍然很少。
{"title":"Hereditary Hemorrhagic Telangiectasia (Osler's Disease): Systemic, Interdisciplinary, Relatively Common—and Often Missed.","authors":"Urban W Geisthoff, Andreas H Mahnken, Ulrike W Denzer, André Kemmling, Christopher Nimsky, Boris A Stuck","doi":"10.3238/arztebl.m2024.0111","DOIUrl":"10.3238/arztebl.m2024.0111","url":null,"abstract":"<p><strong>Background: </strong>Hereditary hemorrhagic telangiectasia (HHT, Rendu- Osler-Weber disease, or Osler's disease for short) is a systemic disease that can severely impair the quality of life and that requires interdisciplinary treatment. Among rare diseases, it is relatively common, with a prevalence of approximately 1/5000.</p><p><strong>Methods: </strong>This review is based on publications retrieved by a selective literature search, including the two international guidelines on clinically relevant aspects of HHT.</p><p><strong>Results: </strong>On average, about two decades elapse between the initial symptoms and the diagnosis of HHT. 95% of patients have nosebleeds; these usually begin before age 20 but can occur at any time, from infancy to old age. The diagnosis is usually made on clinical grounds on the basis of the characteristic telangiectases, a positive family history, and possible involvement of the gastrointestinal tract, lungs, liver, and brain. Nosebleeds can sometimes be reduced by outpatient measures including counseling on keeping the nose moist (expert consensus), self-application of a nasal packing (which improves the quality of life, according to an online survey), and the prescription of tranexamic acid (reduction of nosebleeds from 17.3% [5.5; 27.6] to 54%). In particular, screening (expert consensus) for pulmonary vascular malformations (frequency 10-50%) can prevent many adverse outcomes. If pulmonary vascular malformations cannot be ruled out, antibiotic prophylaxis is recommended before medical procedures that can cause bacteremia (expert consensus).</p><p><strong>Conclusion: </strong>Broad awareness of the condition, early diagnosis, and interdisciplinary treatment improve the quality of life and ultimate outcome of persons with HHT. Nevertheless, there are few options supported by good evidence for the appropriate treatment of this rare, often serious disease..</p>","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":" Forthcoming","pages":"601-607"},"PeriodicalIF":6.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11661474/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141999573","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 Frequency of Food Allergens as Triggers of Severe Allergic Reactions—Data from the Anaphylaxis Registry. 食物过敏原引发严重过敏反应的频率——来自过敏反应登记处的数据。
IF 6.5 2区 医学 Q1 MEDICINE, GENERAL & INTERNAL Pub Date : 2024-09-06 DOI: 10.3238/arztebl.m2024.0110
Margitta Worm, Sabine Dölle-Bierke, Veronika Höfer
{"title":"The Frequency of Food Allergens as Triggers of Severe Allergic Reactions—Data from the Anaphylaxis Registry.","authors":"Margitta Worm, Sabine Dölle-Bierke, Veronika Höfer","doi":"10.3238/arztebl.m2024.0110","DOIUrl":"10.3238/arztebl.m2024.0110","url":null,"abstract":"","PeriodicalId":11258,"journal":{"name":"Deutsches Arzteblatt international","volume":"121 18","pages":"610-611"},"PeriodicalIF":6.5,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11661486/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142805807","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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