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Search on : Tracheobronchomegaly [Words]
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[PMID]: 29406234
[Au] Autor:Pacheco GG; Jones AM; Yao J; Kleiner DE; Taveira-DaSilva AM; Moss J
[Ad] Address:Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
[Ti] Title:Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis.
[So] Source:Chest;153(2):e19-e23, 2018 Feb.
[Is] ISSN:1931-3543
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:We present the case of a man with Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, who was referred to the National Institutes of Health Clinical Research Center because of a potential diagnosis of lymphangioleiomyomatosis (LAM), a rare condition in men. The patient was evaluated using ongoing protocols and provided written informed consent. The case demonstrates the presence of chronic inflammation surrounding the dilated airways and histologic changes of the lung parenchyma with emphysematouslike disruption in areas adjacent to the dilated airways. This finding suggests that damage to the lung parenchyma is an ongoing phenomenon in MKS. Moreover, our analysis of CT images indicates similar abnormalities in areas remote from the dilated airways. Finally, because of increased anatomic dead space, calculation of lung diffusion capacity by the single-breath method yielded abnormally low values that required making a correction for the large anatomic dead space, which can be measured by the single-breath nitrogen washout test.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1802
[Cu] Class update date: 180206
[Lr] Last revision date:180206
[St] Status:In-Data-Review

  2 / 261 MEDLINE  
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[PMID]: 29261580
[Au] Autor:Girit S; Senol E; Cag Y; Karatas O; Baysal T
[Ad] Address:Departments of Pediatrics, Division of Pediatric Pulmonology.
[Ti] Title:Ehlers-Danlos Syndrome Type IVB and Tracheobronchomegaly.
[So] Source:J Bronchology Interv Pulmonol;25(1):70-72, 2018 Jan.
[Is] ISSN:1948-8270
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Tracheobronchomegaly (TBM) is a rare disease of unknown etiology, characterized by dilatation of the trachea and main bronchi. Despite its rarity, TBM has been reported to accompany several conditions, particularly Ehlers-Danlos syndrome. Herein, we report on a 15-year-old patient who was diagnosed with TBM on the basis of radiologic and endoscopic findings and with Ehlers-Danlos syndrome type IVB presenting with marfanoid features, severe kyphoscoliosis, increased skin elasticity, and ongoing hypotonia since the neonatal period.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1712
[Cu] Class update date: 171220
[Lr] Last revision date:171220
[St] Status:In-Process
[do] DOI:10.1097/LBR.0000000000000416

  3 / 261 MEDLINE  
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[PMID]: 28891754
[Au] Autor:Quentin C; Lefevre N; Bodart E; Hanssens L
[Ad] Address:a Department of Pneumology , Hopital Universitaire des Enfants Reine Fabiola- Universite Libre de Bruxelles (ULB) , Brussels , Belgium.
[Ti] Title:Mounier Kuhn syndrome presenting with recurrent atelectasis.
[So] Source:Acta Clin Belg;:1-3, 2017 Sep 11.
[Is] ISSN:2295-3337
[Cp] Country of publication:England
[La] Language:eng
[Ab] Abstract:Objective and importance Mounier Kuhn syndrome is usually diagnosed in adulthood, and only a few cases have been described in childhood. Clinical presentation We present the case of a seven-year-old boy suffering from recurrent pneumonia and atelectasis. Intervention Previously performed chest X-rays showed bilateral hyperinflation and tracheobronchomegaly. Chest computed tomography (CT) confirmed the presence of distal enlargement of trachea and bronchi. Tracheobronchomegaly associated with recurrent respiratory tract infections is consistent with Mounier Kuhn syndrome. Pseudomonas aeruginosa was isolated from the sputum of the patient. He was then treated according to the guidelines for P. aeruginosa management in cystic fibrosis patients considering the similarities in clinical presentations and pathophysiology of both diseases. Antibiotic treatment resulted in a remarkable reduction of events of pulmonary exacerbation and hospitalizations. There are no specific guidelines for treatment options in case of pulmonary exacerbation of Mounier Kuhn syndrome. Case reports discussing the choice and efficiency of antibiotic treatment are random. Conclusion headings We share our experience of treating pulmonary exacerbation caused by P. aeruginosa in a patient with Mounier Kuhn syndrome suggesting a possible treatment option of pseudomonas infections in this syndrome.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1709
[Cu] Class update date: 170911
[Lr] Last revision date:170911
[St] Status:Publisher
[do] DOI:10.1080/17843286.2017.1373498

  4 / 261 MEDLINE  
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[PMID]: 28845373
[Au] Autor:Ayub F; Saif MW
[Ad] Address:Internal Medicine, Rawalpindi Medical College, Rawalpindi, Pakistan.
[Ti] Title:First Case Report of Metastatic Squamous Cell Carcinoma of Lung Associated with Mounier-Kuhn Syndrome and Review of Literature.
[So] Source:Cureus;9(6):e1394, 2017 Jun 26.
[Is] ISSN:2168-8184
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Mounier-Kuhn syndrome is a relatively rare condition, mostly involving the trachea and main stem bronchi. It is caused either by the atrophy of elastic fibers or faulty fetal development of cartilage and smooth muscles, hence leading to an overall increase in the diameter of lower respiratory tract. No certain etiology was found in the majority of cases reported previously, however, several other connective tissue diseases have also been implicated with the congenital tracheobronchomegaly. One anecdotal case report mentioned the development of lung malignancy in a patient who had previously received external beam radiotherapy. Herein, we report the first case of Mounier-Kuhn syndrome in a 62-year-old male with a recent diagnosis of metastatic squamous cell carcinoma (SCC) of the lung.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 170831
[Lr] Last revision date:170831
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.7759/cureus.1394

  5 / 261 MEDLINE  
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[PMID]: 28757767
[Au] Autor:Lee CC; Lin BS; Chen JY; Chuang CC
[Ad] Address:Department of Anesthesiology, Chi Mei Medical Center, Tainan, Taiwan.
[Ti] Title:Anesthesia for a patient with unexpected giant tracheobronchomegaly.
[So] Source:Ci Ji Yi Xue Za Zhi;29(1):59-61, 2017 Jan-Mar.
[Is] ISSN:1016-3190
[Cp] Country of publication:China (Republic : 1949- )
[La] Language:eng
[Ab] Abstract:Tracheobronchomegaly (also called Mounier-Kuhn syndrome) is a rare disease characterized by flaccid and markedly dilated trachea and main bronchi on inspiration with narrowing or collapse on expiration or cough. It is associated with recurrent lower respiratory tract infection. A 75-year-old man with unexpected giant tracheomegaly had a significant peritubal air leak which impeded an operation. Lumbar epidural anesthesia was performed for a subsequent operation without any sequela. Careful evaluation with chest radiography is basic to exclude a large airway. Chest computed tomography and fiber-optic bronchoscopy provided the diagnosis of a large airway. If a large airway is suspected, these examinations help to evaluate and manage the airway.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1708
[Cu] Class update date: 170803
[Lr] Last revision date:170803
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.4103/tcmj.tcmj_1_17

  6 / 261 MEDLINE  
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[PMID]: 28734804
[Au] Autor:Geng Y; Zhou J; Liu Z
[Ad] Address:Intensive Care Unit, Affiliated Hospital of Nanjing University of Traditional Chinese Medicine, Nanjing, China.
[Ti] Title:Tracheobronchomegaly in Intubated Ventilation of ARDS.
[So] Source:Arch Bronconeumol;, 2017 Jul 19.
[Is] ISSN:1579-2129
[Cp] Country of publication:Spain
[La] Language:eng; spa
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1707
[Cu] Class update date: 170723
[Lr] Last revision date:170723
[St] Status:Publisher

  7 / 261 MEDLINE  
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[PMID]: 28596443
[Au] Autor:Simkins A; Maiti A; Cherian SV; Trujillo DO; Estrada-Y-Martin RM
[Ad] Address:Department of Internal Medicine, The University of Texas Health Science Center at Houston, McGovern School of Medicine, Houston, Texas, USA.
[Ti] Title:Mounier-Kuhn syndrome.
[So] Source:Postgrad Med J;93(1104):642, 2017 Oct.
[Is] ISSN:1469-0756
[Cp] Country of publication:England
[La] Language:eng
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1706
[Cu] Class update date: 170930
[Lr] Last revision date:170930
[St] Status:In-Data-Review
[do] DOI:10.1136/postgradmedj-2017-134958

  8 / 261 MEDLINE  
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[PMID]: 28480154
[Au] Autor:Fletcher A; Stowell J; Jamoulis S
[Ad] Address:Internal Medicine, Truman Medical Center, University of Missouri School of Medicine, Kansas City, MO, USA.
[Ti] Title:Congenital Tracheobronchomegaly (Mounier-Kuhn Syndrome) in a Woman with Human Immunodeficiency Virus: A Case Report.
[So] Source:Cureus;9(4):e1136, 2017 Apr 04.
[Is] ISSN:2168-8184
[Cp] Country of publication:United States
[La] Language:eng
[Ab] Abstract:Congenital tracheobronchomegaly (Mounier-Kuhn Syndrome, MKS) is a rare idiopathic disorder characterized by dilation of the central airways, including the trachea and first through fourth order bronchi. MKS disproportionately affects men and results in chronic respiratory tract infections. The diagnosis is made through the synthesis of clinical and radiological data. Here we report a unique case of MKS in a patient with human immunodeficiency virus (HIV) infection. A 45-year-old African American woman with a past medical history of HIV, tobacco and recreational drug abuse, chronic obstructive pulmonary disease, sleep apnea, and a 15-year history of recurrent respiratory infections presented with dyspnea, wheezing, a productive cough, increased yellow-green sputum production, and subjective fevers. Computerized tomography (CT) of the chest revealed striking dilation of the trachea and central bronchi. Fiberoptic bronchoscopy demonstrated a dilated trachea and bronchial tree with complete collapse of the trachea and bilateral mainstem bronchi during expiration. Serial imaging over 14 years allowed the radiologist to confidently diagnose her underlying disorder and recommend appropriate clinical management, which included mucolytics, chest physiotherapy, prophylactic vaccinations, and antibiotics during infectious exacerbations. To the best of our knowledge, there is only one reported case of MKS in the setting of HIV in the English literature. We report the second such case and outline the clinical presentation, diagnostic criteria, and management of MKS with the hope that increased awareness will prevent delayed or misdiagnosis for patients with MKS. This case highlights the common diagnostic delay for MKS and the need to include MKS in the differential diagnosis of recurrent respiratory tract infections.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1705
[Cu] Class update date: 170816
[Lr] Last revision date:170816
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.7759/cureus.1136

  9 / 261 MEDLINE  
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[PMID]: 28217888
[Au] Autor:Camilo GB; Carvalho ARS; Guimarães ARM; Kasuki L; Gadelha MR; Mogami R; de Melo PL; Lopes AJ
[Ad] Address:Postgraduate Programme in Medical Sciences, School of Medical Sciences, State University of Rio de Janeiro, Rio de Janeiro, Brazil.
[Ti] Title:Computed tomography airway lumen volumetry in patients with acromegaly: Association with growth hormone levels and lung function.
[So] Source:J Med Imaging Radiat Oncol;61(5):591-599, 2017 Oct.
[Is] ISSN:1754-9485
[Cp] Country of publication:Australia
[La] Language:eng
[Ab] Abstract:INTRODUCTION: The segmentation and skeletonisation of images via computed tomography (CT) airway lumen volumetry provide a new perspective regarding the incorporation of this technique in medical practice. Our aim was to quantify morphological changes in the large airways of patients with acromegaly through CT and, secondarily, to correlate these findings with hormone levels and pulmonary function testing (PFT) parameters. METHODS: This was a cross-sectional study in which 28 non-smoker patients with acromegaly and 15 control subjects underwent CT analysis of airway lumen volumetry with subsequent image segmentation and skeletonisation. Moreover, all participants were subjected to PFT. RESULTS: Compared with the controls, patients with acromegaly presented higher diameters in the trachea, right main bronchus and left main bronchus. The patients with acromegaly also showed a higher tracheal sinuosity index (the deviation of a line from the shortest path, calculated by dividing total length by shortest possible path) than the controls [1.06 (1.02-1.09) vs. 1.03 (1.02-1.04), P = 0.04], and tracheal stenosis was observed in 25% of these individuals. The tracheal area was correlated with the levels of growth hormone (r  = 0.45, P = 0.02) and insulin-like growth factor type I (r  = 0.38, P = 0.04). The ratio between the forced expiratory flow and forced inspiratory flow at 50% of the forced vital capacity was correlated with the tracheal area (r  = 0.36, P = 0.02) and Δ tracheal diameters (r  = 0.58, P < 0.0001). CONCLUSION: Patients with acromegaly exhibit tracheobronchomegaly and tracheal sinuosity/stenosis. Moreover, there are associations between the results of CT airway lumen volumetry, hormone levels and functional parameters of large airway obstruction.
[Pt] Publication type:JOURNAL ARTICLE
[Em] Entry month:1702
[Cu] Class update date: 171009
[Lr] Last revision date:171009
[St] Status:In-Process
[do] DOI:10.1111/1754-9485.12598

  10 / 261 MEDLINE  
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[PMID]: 28197883
[Au] Autor:Barnes D; Gutiérrez Chacoff J; Benegas M; Perea RJ; de Caralt TM; Ramirez J; Vollmer I; Sanchez M
[Ad] Address:Radiology Department, Hospital Clinic de Barcelona, Villarroel 170, 08036, Barcelona, Spain. dbarnes@clinic.cat.
[Ti] Title:Central airway pathology: clinic features, CT findings with pathologic and virtual endoscopy correlation.
[So] Source:Insights Imaging;8(2):255-270, 2017 Apr.
[Is] ISSN:1869-4101
[Cp] Country of publication:Germany
[La] Language:eng
[Ab] Abstract:OBJECTIVES: To describe the imaging features of the central airway pathology, correlating the findings with those in pathology and virtual endoscopy. To propose a schematic and practical approach to reach diagnoses, placing strong emphasis on multidetector computed tomography (MDCT) findings. METHODS: We reviewed our thoracic pathology database and the central airway pathology-related literature. Best cases were selected to illustrate the main features of each disease. MDCT was performed in all cases. Multiplanar and volume-rendering reconstructions were obtained when necessary. Virtual endoscopy was obtained from the CT with dedicated software. RESULTS: Pathological conditions affecting the central airways are a heterogeneous group of diseases. Focal alterations include benign neoplasms, malignant neoplasms, and non-neoplastic conditions. Diffuse abnormalities are divided into those that produce dilation and those that produce stenosis and tracheobronchomalacia. Direct bronchoscopy (DB) visualises the mucosal layer and is an important diagnostic and therapeutic weapon. However, assessing the deep layers or the adjacent tissue is not possible. MDCT and post-processing techniques such as virtual bronchoscopy (VB) provide an excellent evaluation of the airway wall. CONCLUSION: This review presents the complete spectrum of the central airway pathology with its clinical, pathological and radiological features. TEACHING POINTS: • Dividing diseases into diffuse and focal lesions helps narrow the differential diagnosis. • Focal lesions with nodularity are more likely to correspond to tumours. • Focal lesions with stenosis are more likely to correspond to inflammatory disease. • Posterior wall involvement is the main feature in diffuse lesions with stenosis.
[Pt] Publication type:JOURNAL ARTICLE; REVIEW
[Em] Entry month:1702
[Cu] Class update date: 170816
[Lr] Last revision date:170816
[St] Status:PubMed-not-MEDLINE
[do] DOI:10.1007/s13244-017-0545-6


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