Pub Date : 2024-06-01Epub Date: 2023-12-14DOI: 10.1055/a-2216-0404
Fabian Gleibs, Felix Döllinger, Martin Witzenrath, Ralf-Harto Huebner, Jacopo Saccomanno
A 24-year-old male patient, without further symptoms or comorbidities presented to the emergency room with acute dyspnea after heavy lifting two days before. On auscultation an attenuated vesicular breath was noticed on the right lung. In the initial chest radiograph a right-sided primary spontaneous pneumothorax with minor mediastinal shift was diagnosed. After insertion of a 12-French chest tube the patient's clinical condition deteriorated. The following chest radiograph and computed tomography of the thorax showed a reexpansion pulmonary edema in the right lung. The patient was admitted to the ICU and supportive treatment was initiated. Pulmonary reexpansion edema after drainage of a pneumothorax is a very rare complication with mortality rates reaching up to 20%. The exact pathophysiology remains unknown. Typical Symptoms include dyspnea, hypotension, and tachycardia. To minimize the risk of a pulmonary reexpansion edema, not more than 1200-1800 ml of air should be drained at once and the drainage should be stopped when the patient starts coughing.
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Pub Date : 2024-06-01Epub Date: 2024-02-13DOI: 10.1055/a-2182-1907
Carsten Schwarz, Jutta Bend, Helge Hebestreit, Michael Hogardt, Christian Hügel, Stephan Illing, Jochen G Mainz, Ernst Rietschel, Sebastian Schmidt, Bernhard Schulte-Hubbert, Helmut Sitter, Marc Oliver Wielpütz, Jutta Hammermann, Ingo Baumann, Frank Brunsmann, Doris Dieninghoff, Ernst Eber, Helmut Ellemunter, Patience Eschenhagen, Caroline Evers, Saskia Gruber, Assen Koitschev, Julia Ley-Zaporozhan, Uta Düesberg, Hans-Joachim Mentzel, Thomas Nüßlein, Felix C Ringshausen, Ludwig Sedlacek, Christina Smaczny, Olaf Sommerburg, Sivagurunathan Sutharsan, Ralf-Peter Vonberg, Ann-Katrin Weber, Jovita Zerlik
Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, Pseudomonas aeruginosa (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung function. There are many possibilities to treat PA-infection.This is a S3-clinical guideline which implements a definition for chronic PA-infection and demonstrates evidence-based diagnostic methods and medical treatment in order to give guidance for individual treatment options.
囊性纤维化（CF）是最常见的常染色体隐性遗传多系统疾病。在德国，至少有 8000 人患有此病。该病是由囊性纤维化跨膜传导调节器（CFTR）基因突变导致跨膜氯离子通道 CFTR 功能障碍引起的。即使高效的 CFTR 调节器疗法已经问世多年，CF 患者的年龄也比以前大得多，但气道的反复和慢性感染以及肺部恶化仍时有发生。在成年 CF 患者中，铜绿假单胞菌（PA）是肺部定植和慢性感染的最主要病原体，会导致肺功能进一步丧失。治疗 PA 感染的方法有很多种。这是一份 S3 级临床指南，其中对慢性 PA 感染进行了定义，并展示了循证诊断方法和药物治疗，以便为个体治疗方案提供指导。
{"title":"[CF Lung Disease - a German S3 Guideline: Pseudomonas aeruginosa].","authors":"Carsten Schwarz, Jutta Bend, Helge Hebestreit, Michael Hogardt, Christian Hügel, Stephan Illing, Jochen G Mainz, Ernst Rietschel, Sebastian Schmidt, Bernhard Schulte-Hubbert, Helmut Sitter, Marc Oliver Wielpütz, Jutta Hammermann, Ingo Baumann, Frank Brunsmann, Doris Dieninghoff, Ernst Eber, Helmut Ellemunter, Patience Eschenhagen, Caroline Evers, Saskia Gruber, Assen Koitschev, Julia Ley-Zaporozhan, Uta Düesberg, Hans-Joachim Mentzel, Thomas Nüßlein, Felix C Ringshausen, Ludwig Sedlacek, Christina Smaczny, Olaf Sommerburg, Sivagurunathan Sutharsan, Ralf-Peter Vonberg, Ann-Katrin Weber, Jovita Zerlik","doi":"10.1055/a-2182-1907","DOIUrl":"10.1055/a-2182-1907","url":null,"abstract":"<p><p>Cystic Fibrosis (CF) is the most common autosomal recessive genetic multisystemic disease. In Germany, it affects at least 8000 people. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the airway epithelial lining fluid which leads to reduction of the mucociliary clearance.Even if highly effective, CFTR modulator therapy has been available for some years and people with CF are getting much older than before, recurrent and chronic infections of the airways as well as pulmonary exacerbations still occur. In adult CF life, <i>Pseudomonas aeruginosa</i> (PA) is the most relevant pathogen in colonisation and chronic infection of the lung, leading to further loss of lung function. There are many possibilities to treat PA-infection.This is a S3-clinical guideline which implements a definition for chronic PA-infection and demonstrates evidence-based diagnostic methods and medical treatment in order to give guidance for individual treatment options.</p>","PeriodicalId":20197,"journal":{"name":"Pneumologie","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2024-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139730370","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}
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