Respirology Case ReportsPub Date : 2025-03-24eCollection Date: 2025-03-01DOI: 10.1002/rcr2.70146
Sona Vekaria, Grace Kavanagh, Siobhain Mulrennan
{"title":"Personalised CFTR Modulator Treatment Initiation and Monitoring in CF-Related Liver Disease: When Less Is More.","authors":"Sona Vekaria, Grace Kavanagh, Siobhain Mulrennan","doi":"10.1002/rcr2.70146","DOIUrl":"10.1002/rcr2.70146","url":null,"abstract":"<p><p>Hepatotoxicity due to Elexacaftor/Tezacaftor/Ivacaftor (ETI) use has been well documented. There are no dose adjustments or increased-frequency monitoring algorithms recommended for people who experience elevated transaminases without cirrhosis, only suggested treatment interruption or withdrawal depending on the severity of the derangement. Here we describe a patient with non-cirrhotic hepatic steatosis who experienced persistently elevated liver function tests due to modulator therapy but demonstrated a remarkable response to a notably low dose of ETI.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70146"},"PeriodicalIF":0.8,"publicationDate":"2025-03-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11932953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143711497","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Allergic Bronchopulmonary Mycosis Caused by <i>Schizophyllum commune</i> Successfully Treated With Mepolizumab.","authors":"Mitsuki Kakiuchi, Yuki Takigawa, Ken Sato, Hiromi Watanabe, Kenichiro Kudo, Akiko Sato, Keiichi Fujiwara, Takuo Shibayama","doi":"10.1002/rcr2.70161","DOIUrl":"10.1002/rcr2.70161","url":null,"abstract":"<p><p>Allergic bronchopulmonary mycosis (ABPM) is a complication of bronchial asthma (BA) and can lead to lung damage and respiratory failure. The standard treatment for ABPM is steroid therapy. Nonetheless, this treatment increases the risk of infections and osteoporosis. Alternative therapies include immunomodulatory biologics and mucus plug removal by bronchoscopy. This report describes a case of a 70-year-old man diagnosed with BA in 2014 and treated with inhaled corticosteroids/long-acting beta-agonists, which were later discontinued for unknown reasons. He was presented to our department with a BA attack, including wheezing and desaturation. Computed tomography and laboratory examinations suggested ABPM; the patient underwent mucus plug removal. The final diagnosis was ABPM, and steroids and itraconazole were initiated. However, BA symptoms did not improve, and the mucus plug and desaturation persisted. Mepolizumab (100 mg/month) subcutaneously cleared the plug and improved BA. This case demonstrates that mepolizumab can treat patients with ABPM and refractory BA.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70161"},"PeriodicalIF":0.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11925694/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Respirology Case ReportsPub Date : 2025-03-20eCollection Date: 2025-03-01DOI: 10.1002/rcr2.70163
Ali Gohar, Bilal Ahmed, Asim Ali, Maryam Ilyas, Momina Masroor, Ayesha Ayman, Masab Ali, Muhammad Husnain Ahmad
{"title":"Primary Ciliary Dyskinesia Complicated by Stroke in an Elderly Male: A Case Report.","authors":"Ali Gohar, Bilal Ahmed, Asim Ali, Maryam Ilyas, Momina Masroor, Ayesha Ayman, Masab Ali, Muhammad Husnain Ahmad","doi":"10.1002/rcr2.70163","DOIUrl":"10.1002/rcr2.70163","url":null,"abstract":"<p><p>Kartagener syndrome (KS) is an uncommon hereditary disorder, featuring situs inversus, chronic sinusitis, and bronchiectasis. Our case report presents a 60-year-old Asian male with KS who was incidentally diagnosed with KS upon presenting with an ischemic cerebrovascular accident (CVA). The patient had a longstanding history of poorly controlled type 2 diabetes mellitus and hypertension, presenting with acute right-sided weakness and speech impairment. His history of recurrent respiratory infections and infertility, combined with family findings of dextrocardia and male infertility, suggested KS. On physical examination, the patient exhibited signs of dextrocardia. Imaging confirmed situs inversus and dextrocardia, while neurological evaluation revealed an embolic stroke in the left middle cerebral artery territory. Certain cardiovascular anomalies in KS may affect stroke risk; however, their co-occurrence in the patient appears to be coincidental, given the patient's baseline risk for stroke. A high-resolution chest CT demonstrated bronchiectasis and carotid Doppler ultrasound identified atherosclerotic plaques, likely contributing to the stroke. This case highlights the need for heightened awareness of neurological events, such as stroke, in patients with KS. Cardiovascular risk factors, compounded by the complexity of the syndrome, require prompt evaluation and multidisciplinary care to prevent severe complications.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70163"},"PeriodicalIF":0.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11926249/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693936","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Respirology Case ReportsPub Date : 2025-03-20eCollection Date: 2025-03-01DOI: 10.1002/rcr2.70134
Jose Carlos Fraga, Felipe Colombo Holanda, Natalia Zanini Silva, Maria Fernanda Oliveira, Paola Santis Isolan, Elenara Fonseca Procianoy
{"title":"Bronchopleural Fistula Following COVID-19 and Necrotizing Pneumonia in Childhood: Treatment With Intrapleural Vacuum-Assisted Closure Therapy.","authors":"Jose Carlos Fraga, Felipe Colombo Holanda, Natalia Zanini Silva, Maria Fernanda Oliveira, Paola Santis Isolan, Elenara Fonseca Procianoy","doi":"10.1002/rcr2.70134","DOIUrl":"10.1002/rcr2.70134","url":null,"abstract":"<p><p>Bronchopleural fistula is an abnormal communication between the bronchial tree and the pleural space. Necrotizing pneumonia is a catastrophic infection of the lungs characterised by necrosis of the interstitial tissue. Although rarely reported, it has been described as a complication of COVID-19. The usual treatment for bronchopleural fistula involves endoscopic or surgical procedures. We present the case of a 5-year-old girl admitted with necrotizing pneumonia and difficult-to-control bronchopleural fistula, with a report of flu-like illness and a positive PCR test for COVID-19 2 months earlier. Over 38 days, several procedures were performed, including left-side tube drainage, thoracotomy, segmental resection, open window thoracotomy, and finally vacuum-assisted closure (VAC) therapy. Twenty days after the start of VAC therapy, complete closure of the bronchopleural fistula was achieved. At 26 months of follow-up, the child remained asymptomatic, with radiological examinations showing pulmonary expansion and absence of intrapleural air.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70134"},"PeriodicalIF":0.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11925471/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143671408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Aspergillus Bronchitis at Localised Mucus Plug in an Immunocompetent Patient.","authors":"Kai Yamazaki, Yukihiro Horio, Kazuhito Hatanaka, Takashi Yaguchi, Kozaburo Sadahiro, Kohei Umemoto, Shigeaki Hattori, Katsuyoshi Tomomatsu, Naoki Hayama, Yoko Ito, Tsuyoshi Oguma, Koichiro Asano","doi":"10.1002/rcr2.70104","DOIUrl":"10.1002/rcr2.70104","url":null,"abstract":"<p><p>Aspergillus tracheobronchitis is a form of invasive aspergillosis that primarily occurs in immunocompromised patients. We report a case of Aspergillus bronchitis in an immunocompetent 55-year-old woman with a mucus plug at the site of localised bronchiectasis. The mucus plug gradually enlarged over 9 years, when the patient exhibited submissive haemoptysis. Bronchial artery embolization, followed by partial lung resection was performed. Pathological and mycological examinations led to the diagnosis of AT caused by <i>Aspergillus udagawae</i>.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70104"},"PeriodicalIF":0.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11925472/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143671402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Respirology Case ReportsPub Date : 2025-03-20eCollection Date: 2025-03-01DOI: 10.1002/rcr2.70156
Joseph Derry, Harshana Bandara
{"title":"A Spontaneous Pneumomediastinum During an Asthma Exacerbation.","authors":"Joseph Derry, Harshana Bandara","doi":"10.1002/rcr2.70156","DOIUrl":"10.1002/rcr2.70156","url":null,"abstract":"<p><p>This case highlights the rare possibility of having pneumomediastinum in patients with asthma without having other structural lung disease. This also highlights the importance of vigilant reading of the chest x-ray, including the proper inspection of the soft tissues areas.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70156"},"PeriodicalIF":0.8,"publicationDate":"2025-03-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11926253/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Cryoextraction of Subacute, Adhesive and Immobile Airway Blood Clot With Short Freezing Time: A Case Report.","authors":"Takashi Nishihara, Takayuki Takimoto, Eiji Sugimoto, Shojiro Minomo, Toru Arai","doi":"10.1002/rcr2.70155","DOIUrl":"10.1002/rcr2.70155","url":null,"abstract":"<p><p>We report a rare case of a patient experiencing persistent exertional dyspnoea due to a subacute, adhesive and immobile airway blood clot following haemoptysis. Cryoextraction with short freezing times, which fragmented the clot into small pieces, proved effective, despite previous reports on acute airway blood clots recommending longer freezing times.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70155"},"PeriodicalIF":0.8,"publicationDate":"2025-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11919481/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143659070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Case of Myasthenia Gravis and Stiff-Person Syndrome That Improved After Extended Thymectomy.","authors":"Shoji Okado, Tetsuya Mizuno, Yuta Kawasumi, Yuka Kadomatsu, Toyofumi Fengshi Chen-Yoshikawa","doi":"10.1002/rcr2.70149","DOIUrl":"10.1002/rcr2.70149","url":null,"abstract":"<p><p>In cases of thymoma with autoimmune disease, extended thymectomy may improve disease symptoms and medication effects. Myasthenia gravis (MG) is the most common complication, but thymoma with Stiff-Person syndrome (SPS) is rare, and there are few reports of surgery. In the present case, we performed robot-assisted extended thymectomy on a 74-year-old man with thymoma who had MG and SPS. At 6 months after surgery, his MG and SPS symptoms had improved and the dose of prednisolone had been reduced, confirming that surgical treatment contributed to disease control. Thus far, only two studies have been published on thymoma surgery in conjunction with MG and SPS, and both have documented the progression of neurological symptoms after surgery. To the best of our knowledge, this is the first report of a resected thymoma with MG and SPS, both of which were controlled after surgery.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70149"},"PeriodicalIF":0.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11913529/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651221","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Placement of a Customised Y-Shaped Silicone Stent via the Transthoracic Approach for Closing a Large Bronchial Stump Fistula.","authors":"Junqi Zhu, Chan Xu, Honglei Tao, Shunxin Xin, Weihua Xu","doi":"10.1002/rcr2.70152","DOIUrl":"10.1002/rcr2.70152","url":null,"abstract":"<p><p>Bronchial stump fistula occurs at the site of a lobectomy or segmentectomy and is a specific type of bronchopleural fistula. In addition to complications such as empyema and pneumothorax, a large bronchial stump fistula can result in decreased lung ventilation due to significant air leakage, particularly during general anaesthesia with endotracheal intubation, making closure of the large bronchial stump fistula challenging. The present paper reports on a case of a patient with a large right bronchial stump fistula who had difficulty maintaining normal oxygenation when using conventional tracheal intubation for mechanical ventilation. We utilised left-lung single-lung ventilation during the procedure to ensure adequate oxygenation and inserted a customised Y-shaped silicone stent via the transthoracic approach into the right-side fistula to occlude the bronchial stump fistula. A satisfactory outcome was achieved in this case.</p>","PeriodicalId":45846,"journal":{"name":"Respirology Case Reports","volume":"13 3","pages":"e70152"},"PeriodicalIF":0.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11913528/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651232","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}