{"title":"Case of pulmonary Langerhans cell histiocytosis presenting as ground-glass opacity in the lower lung","authors":"","doi":"10.1016/j.rmcr.2024.102095","DOIUrl":"10.1016/j.rmcr.2024.102095","url":null,"abstract":"<div><p>Nodules and cysts with upper lobe predominance on chest computed tomography (CT) are highly suggestive of pulmonary Langerhans cell histiocytosis (PLCH). Herein, we describe a case of PLCH that presented with the unusual CT findings of subpleural ground-glass opacity (GGO) and traction bronchiectasis mostly in both lower lungs. No nodules or cysts were observed in the upper or middle lung areas. Video-assisted thoracoscopic biopsies were performed at the right lower lobe. Biopsy specimens showed findings consistent with those of scarred PLCH. To the best of our knowledge, this is the first case of PLCH presenting as GGO in the lower lungs.</p></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213007124001187/pdfft?md5=5b8b74dc5d3298707f1a437ee7d76a28&pid=1-s2.0-S2213007124001187-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142098304","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":"Pulmonary manifestations associated with positive anti neutrophil cytoplasmic antibodies: The tip of the iceberg","authors":"","doi":"10.1016/j.rmcr.2024.102113","DOIUrl":"10.1016/j.rmcr.2024.102113","url":null,"abstract":"<div><h3>Background</h3><p>Even though anti-neutrophil cytoplasmic antibodies (ANCA) are frequently linked to ANCA associated vasculitis (AAV), it's important to understand that other illnesses, including lung diseases, can also manifest as ANCA positivity. Finding the incriminated pathology might be difficult.</p></div><div><h3>Aim</h3><p>To report four ANCA-associated cases with a diagnosis problem.</p></div><div><h3>Methods</h3><p>Four patients were recruited from the allergo-pneumology department of the Fattouma Bourguiba University Hospital of Monastir, Tunisia, over two years (2020–2022). Indirect immunofluorescence technique on neutrophil cells (Euroimmun, Germany) was used for ANCA screening with a positivity limit of 1/20. ANCA typing was carried out by a line-blot technique (Euroimmun, Germany).</p></div><div><h3>Results</h3><p>This case series reports four cases (age range: 41–67 years, sex ratio: 0.3) that presented with pulmonary manifestations associated with ANCA positivity. Two patients had perinuclear ANCA with anti-myeloperoxidase on typing, and two cases had cytoplasmic ANCA with one case of anti-leukocyte proteinase 3 on typing. Final diagnoses were pulmonary tuberculosis (case 1), systemic lupus erythematosus (case 2), bronchiolitis obliterans organizing pneumonia (case 3), and pulmonary aspergillosis with AAV (case 4).</p></div><div><h3>Conclusion</h3><p>A panel of diagnoses may be evoked in front of positive ANCA, making the diagnosis difficult to determine and requiring multidisciplinary interactions, with imaging and histological investigations having a crucial role in guiding the final decision.</p></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213007124001369/pdfft?md5=eebba7307aeb0f5cf020bd84b8b31ab2&pid=1-s2.0-S2213007124001369-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142272472","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":"Hermansky-Pudlak Syndrome: An unusual pattern of pulmonary fibrosis","authors":"","doi":"10.1016/j.rmcr.2024.102123","DOIUrl":"10.1016/j.rmcr.2024.102123","url":null,"abstract":"<div><div>Hermansky-Pudlak Syndrome is a rare genetic cause of pulmonary fibrosis, associated with albinism, nystagmus, and a bleeding diathesis. Histologically, Hermansky-Pudlak Syndrome Pulmonary Fibrosis (HPS-PF) typically resembles usual interstitial pneumonia (UIP), however radiologically this is not always the case with a range of features described in the current literature. HPS-PF typically occurs earlier in life than idiopathic pulmonary fibrosis (IPF) and there is limited evidence to support the use of antifibrotic therapy. Given the rarity and potential clinical outcomes of the disease, further research is required. This may be aided by the inclusion of patient with HPS-PF in registry databases.</div></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142327726","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":"Adult-onset Williams-Campbell syndrome: Clinical presentations, diagnostic challenges, and treatment approaches","authors":"","doi":"10.1016/j.rmcr.2024.102126","DOIUrl":"10.1016/j.rmcr.2024.102126","url":null,"abstract":"<div><div>Williams-Campbell syndrome (WCS), traditionally recognized in childhood, is a rare congenital disorder characterized by subsegmental bronchial cartilage deficiency, leading to bronchiectasis. However, its occurrence in adults presents unique diagnostic complexities. We present two cases: Case 1, a 53-year-old male with recurrent rhinosinusitis and COVID-19-associated lung cysts; Case 2, a 59-year-old cyclist with pulmonary bullae. Diagnostic evaluations included pulmonary function tests and imaging studies. Both cases underwent extensive diagnostic evaluations before WCS diagnosis. Management was focused on symptom alleviation and pneumococcal vaccination. Adult-onset WCS poses diagnostic challenges, often mimicking other respiratory conditions. Pathology confirmation is a gold standard for definitive diagnosis; however, in the case of WCS, the literature supports a diagnostic approach primarily based on clinical and radiological findings. Early recognition and tailored management strategies are essential to enhance patient outcomes.</div></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142359398","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":"RET mutated non-small cell lung cancer (NSCLC) in association with patients from Central America","authors":"","doi":"10.1016/j.rmcr.2024.102118","DOIUrl":"10.1016/j.rmcr.2024.102118","url":null,"abstract":"<div><h3>Background</h3><div>The rearranged during transfection (RET) gene is on chromosome 10 (10q11.2) and normally encodes a receptor tyrosine kinase that plays a role in the development of the kidney, nervous, and respiratory systems.</div><div>Aberrant RET gene activation can occur through gene mutations, gene fusions, or over expression leading to uncontrolled cell growth and the development of malignancy. The RET gene was first identified in 1985 as a protooncogene playing a role in the pathogenesis of lymphoma, and mutations are now associated with numerous cancers including lung, thyroid, breast, colon, prostate, and kidney. Activating RET mutations are estimated to occur in 1–2% of NSCLC cases.</div><div>Our center (University Medical Center New Orleans) serves a diverse patient population, seeing approximately forty-five new diagnoses of advanced lung cancer per year. All patients with a new diagnosis of lung cancer have tumor material tested for mutations with use of high throughput next generation sequencing (NGS). The typical patient with a RET-mutated malignancy is a younger patient, nonsmoker, with adenocarcinoma histology. However, ethnicity has not been found to be associated with this driver mutation, unlike, for example, EGFR-mutated lung adenocarcinoma and its association with Asian women. In this case series we describe three patients identified as having a RET mutated lung adenocarcinoma, all of whom were originally from Honduras, presenting over the course of three years (3/2022, 5/2023, 6/2020).</div></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142359395","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":"Primary pulmonary extranodal NK/T-cell lymphoma diagnosed by thoracoscopic lung biopsy: A case report","authors":"","doi":"10.1016/j.rmcr.2024.102127","DOIUrl":"10.1016/j.rmcr.2024.102127","url":null,"abstract":"<div><div>A 63-year-old Japanese female presented with fever. Computed tomography showed multiple nodules in both lungs. Corticosteroids and antibiotics were administered to treat suspected organizing and bacterial pneumonia, resulting in no improvement and respiratory failure worsened. Surgical lung biopsy revealed infiltration of CD3, CD56, Granzyme B, and EBV-encoded RNA-ISH-positive atypical lymphocytes. She was diagnosed with primary pulmonary extranodal NK/T-cell lymphoma (ENKL) and died two months after diagnosis with only a temporary effectiveness of chemotherapy. We should consider the possibility of ENKL and perform prompt and appropriate biopsy for early diagnosis in cases where empiric therapy is ineffective for suspected pneumonia.</div></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142359394","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 pulmonary mucosa-associated lymphoid tissue lymphoma diagnosed by detecting t(11;18)(q21;q21) in bronchial lavage fluid","authors":"","doi":"10.1016/j.rmcr.2024.102133","DOIUrl":"10.1016/j.rmcr.2024.102133","url":null,"abstract":"<div><div>A 76-year-old woman was diagnosed with mucosa-associated lymphoid tissue (MALT) lymphoma by upper gastrointestinal endoscopy. She underwent further investigation for concomitant bilateral pleural effusions and right pulmonary consolidation. MALT lymphoma with the t(11; 18)(q21; q21) translocation and <em>API2-MALT1</em> were detected in pleural fluid. Lymphoma was not histopathologically diagnosed by lung biopsies, but the same translocation was identified in bronchial lavage. MALT lymphoma is often difficult to diagnose by bronchoscopy because of only mild dysplasia. However, present report on using chromosomal translocation analysis from bronchial lavage indicates that such testing may serve as a useful diagnostic adjunct in MALT lymphoma with lung involvement.</div></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":0.8,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142527242","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":"Pulmonary Langerhan's cell histocystosis presenting with bilateral simultaneous pneumothoraces – Case report","authors":"Sophie Buckley , Emily O'Reilly , Deirdre Doyle , Desmond Murphy","doi":"10.1016/j.rmcr.2024.101999","DOIUrl":"https://doi.org/10.1016/j.rmcr.2024.101999","url":null,"abstract":"<div><p>We describe the case of a young male, with no significant medical history, who presented to the Emergency Department (ED) with severe respiratory compromise. He suffered a respiratory arrest shortly after presentation. An initial chest x-ray performed post intubation revealed bilateral pneumothoraces with evidence of abnormal underlying lungs. Through a series of investigations, he was diagnosed with Pulmonary Langerhans Cell Histocystosis. In this article, we outline the initial presentation, subsequent acute management and the clinical course pertaining to this man's presentation. We believe this is the first reported presentation of bilateral simultaneous pneumothoraces, with previously unknown Pulmonary Langerhans Cell histiocytosis (PLCH).</p></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213007124000224/pdfft?md5=65fcb9b58220331c1c889bcad1b1799b&pid=1-s2.0-S2213007124000224-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140067298","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":"Plastic bronchitis secondary to thoracotomy in an adult: A case report","authors":"Jia Wang , Long Tian , Bin Liu","doi":"10.1016/j.rmcr.2024.102002","DOIUrl":"https://doi.org/10.1016/j.rmcr.2024.102002","url":null,"abstract":"<div><p>This study presents a rare case of plastic bronchitis (PB) in a 57-year-old adult post-lung lobectomy, a condition predominantly found in post-Fontan children. The patient exhibited progressive dyspnea and complete atelectasis on the surgical side, revealing a gray rubbery bronchial cast obstructing the right main bronchus. Treatment involved repeated bronchoscopies, glucocorticoids, acetylcysteine, physiotherapy, and a low-fat diet, leading to gradual improvement. No similar cases have been reported, highlighting PB's diagnostic challenge. This underscores the need to consider rare conditions like PB in post-lobectomy complications. Timely examinations and bronchoscopies are essential for accurate diagnosis, ensuring prompt treatment and improving patient outcomes.</p></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S221300712400025X/pdfft?md5=fc9d790e35eb187dd3a88b7ef4ceb597&pid=1-s2.0-S221300712400025X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140113867","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}
Marco Ceccarelli , Manuel D'Onofrio , Vincenzo Ambrogi , Matteo Russo
{"title":"A numerical analysis of ventilation motion after chest surgery with a RESPIRholter device","authors":"Marco Ceccarelli , Manuel D'Onofrio , Vincenzo Ambrogi , Matteo Russo","doi":"10.1016/j.rmcr.2024.102005","DOIUrl":"10.1016/j.rmcr.2024.102005","url":null,"abstract":"<div><p>This case report presents a numerical evaluation of respiration in terms of biomechanical parameters of chest motion. This experimental evaluation is performed with RESPIRholter, a wearable device specifically developed to monitor the movement in the ribcage through the motion of the sixth rib whose characteristic motion is considered as representative of the motion of the thorax. Here we present test results acquired with a RESPIRholter device in a 6-h acquisition. These results characterize respiration biomechanics for diagnostic purposes in a chest surgery patient, highlighting the diagnostic utility of RESPIRholter in the identification of post-operation respiratory problem.</p></div>","PeriodicalId":51565,"journal":{"name":"Respiratory Medicine Case Reports","volume":null,"pages":null},"PeriodicalIF":1.1,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2213007124000285/pdfft?md5=013fc422914af832b493b6d4d0b317bf&pid=1-s2.0-S2213007124000285-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140270317","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}