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Rare lung diseases

Chad Dammling, Peter Waite, Tim Beukelman
Sarcoidosis is a multisystem granulomatous disease of unknown etiology that commonly affects the lungs, lymph nodes, and skin. The disease often presents in patients between the third and sixth decade and its pathology is defined by the presence of noncaseating granulomas within organs throughout the body. Oral and neurologic involvement of sarcoid is extremely rare and occurs in approximately 1% and 5% of patients with the disease, respectively. A case of sarcoidosis involving the gingiva and submandibular lymph nodes is described in a 14-year-old girl...
January 23, 2019: Journal of Oral and Maxillofacial Surgery
Hirotsugu Hashimoto, Yuta Tsugeno, Keisuke Sugita, Kentaro Inamura
Lung cancers are mainly composed of epithelial tumors such as carcinomas. Since mesenchymal tumors that arise in the lung are very rare, they have garnered little attention. The 2015 World Health Organization (WHO) classification of lung tumors has undergone revision, not only for carcinomas but also for mesenchymal tumors. The current version now includes PEComatous tumors, myoepithelial tumors, and pulmonary myxoid sarcomas with EWSR1-CREB1 translocation as new disease entities. To date, no review article has comprehensively summarized what is known about pulmonary mesenchymal tumors in accordance with the latest WHO classification...
January 2019: Journal of Thoracic Disease
Shu-Jun Ke, Peng Wang, Bing Xu
Background: Clear cell adenocarcinoma of the lung (CCAL) is a rare diagnosis with poorly understood clinicopathological characteristics and disease progression. Methods: A population cohort study was conducted using prospectively extracted data from the Surveillance, Epidemiology and End Results database for patients with histological diagnoses of CCAL. Propensity-matched analysis was performed for survival analysis. Results: A total of 1,203 patients with CCAL were included...
2019: Cancer Management and Research
Andre Monteiro, Guilherme Jose Agnoletto, Manuel F Granja, Ester N Bit-Ivan, Eric Sauvageau
BACKGROUND: Intracranial vascular infections of fungal etiology are extremely rare. The vast majority of cases occur in immunocompromised patients with invasive fungal disease, most commonly originating in the paranasal sinuses or the lungs. An extremely rare type in this part of the world, called Granulomatous Invasive Rhinosinusitis, has been reported to affect immunocompetent patients in most cases, and its causative strain has potential to invade the intracranial arteries. CASE DESCRIPTION: We present a rare case of basilar artery rupture and infarction secondary to granulomatous invasive rhinosinusitis in a 50 year old male who presented with ischemic symptoms and 6 month history of headache, dizziness and falls in Florida, USA...
February 14, 2019: World Neurosurgery
Paolo Borghetti, Marco Lorenzo Bonù, Rachele Giubbolini, Niccolo' Giaj Levra, Rosario Mazzola, Marco Perna, Luca Visani, Fiammetta Meacci, Maria Taraborrelli, Luca Triggiani, Davide Franceschini, Carlo Greco, Alessio Bruni, Stefano Maria Magrini, Vieri Scotti
PURPOSE: To investigate the role of radiotherapy (RT) in the management of EGFR- or ALK-mutated metastatic non-small cell lung cancer (NSCLC) treated with TKI. MATERIALS AND METHODS: Clinical data of 106 patients (pts) from five Institutions treated with RT concomitant to TKI were retrospectively revised. Overall survival (OS) and toxicities were analyzed as endpoints of the study. RESULTS: Median age of pts was 65 years. TKIs were given for EGFR (81%)- or ALK (19%)-mutated metastatic NSCLC...
February 15, 2019: La Radiologia Medica
Yukiko Takakuwa, Hironari Hanaoka, Tomofumi Kiyokawa, Harunobu Iida, Kana Ishimori, Toshimasa Uekusa, Hidehiro Yamada, Kimito Kawahata
OBJECTIVES: Adult-onset Still's disease (AOSD) is an inflammatory disorder characterised by sustained fevers, arthritis, and skin involvement. Interstitial lung disease (ILD) is a rare manifestation, and its clinical characteristics have yet to be determined. METHODS: We sought to examine the clinical characteristics of AOSD-associated ILD. We retrospectively investigated 78 patients diagnosed as AOSD. ILD was diagnosed based on chest high-resolution computed tomography (HRCT)...
February 11, 2019: Clinical and Experimental Rheumatology
Abdulrahman Hakami, Evita Zwartkruis, Teodora Radonic, Johannes M A Daniels
BACKGROUND: Pulmonary carcinoids are included in the group of neuroendocrine tumors (NET) and derive from pulmonary neuroendocrine cells. The incidence of these tumors is increasing, but disease awareness remains low among clinicians. The synchronous presentation of lung cancer and mycobacterial infection is well known but the combination of pulmonary carcinoid and mycobacterial infection is rare. CASE PRESENTATION: We treated a 45-year-old female who presented with recurrent pneumonia...
February 15, 2019: BMC Pulmonary Medicine
G Paolino, R Pampena, S Grassi, S R Mercuri, M Cardone, P Corsetti, E Moliterni, M Muscianese, A Rossi, P Frascione, C Longo
Alopecia neoplastica (AN) from visceral tumors is a rare form of cutaneous metastasis in which internal malignancies spread to the scalp. The diagnosis of AN may be very challenging, especially when its onset precedes the diagnosis of the primary tumor. We aimed to improve the knowledge on AN, highlighting that in case of scarring localized alopecia, a differential diagnosis with metastasis should always be considered. We performed a systematic review to describe the main demographic and clinical features associated with AN from visceral malignancies; a survival analysis was also performed...
February 14, 2019: Journal of the European Academy of Dermatology and Venereology: JEADV
Pengcheng Zhou, Wei Yu, Kelin Chen, Xuelian Li, Qianming Xia
RATIONALE: Dieulafoy's lesions are characterized by the presence of a dysplastic artery in the submucosa, most frequently associated with gastrointestinal hemorrhage. They are rarely identified in the bronchial submucosa and can cause massive or fatal hemoptysis PATIENT CONCERNS:: The patient was a 62-year-old male farmer with intermittent hemoptysis of approximately 2 years duration and a definite diagnosis could not be established. DIAGNOSIS: A thorax-computed tomography at our hospital revealed that the bronchus of left lower lobe was narrowed with associated local atelectasis, and lung cancer was suspected...
February 2019: Medicine (Baltimore)
M K Khan, M N Islam, J Ferdous, M M Alam
Tuberculosis (TB) is a progressive granulomatous infectious disease caused by the gram positive, acid fast bacilli classified under the genus Mycobacterium. Tuberculosis in human is mostly by Mycobacterium tuberculosis and primarily affects lungs causing pulmonary tuberculosis. It can also affect intestine, meninges, bones, joints, lymph nodes, skin and other tissues of the body causing extra-pulmonary tuberculosis. Human TB is transmitted mainly through droplet infection and droplet nuclei. Infection of human with M...
January 2019: Mymensingh Medical Journal: MMJ
Koshi Ota, Yusuke Sasabuchi, Hiroki Matsui, Taisuke Jo, Kiyohide Fushimi, Hideo Yasunaga
BACKGROUND: Acute eosinophilic pneumonia (AEP) is a rare inflammatory lung disease. Previous studies have shown that most patients with AEP are aged 20 to 40 years, whereas several case studies have included older patients with AEP. These studies also suggested that AEP is more prevalent in summer, but they were limited due to their small sample sizes. We therefore investigated the age distribution and seasonality among patients with AEP using a national inpatient database. METHODS: Using the Japanese Diagnosis Procedure Combination database, we identified patients with a recorded diagnosis of AEP from 1 July 2010 to 31 March 2015...
February 12, 2019: BMC Pulmonary Medicine
Ping Xu, Yazhou Cai, Xiaona Ying, Shiqiao Shi, Weidong Song
Pyoderma gangrenosum (PG) is a rare and recurrent ulcerating, non-infectious, inflammatory dermatosis, with occasional concomitant extracutaneous manifestations. The pathogenesis and aetiology of PG are unknown. Moreover, early diagnosis is challenging because there are several visceral manifestations that may occur prior to the skin findings, such that misdiagnosis of PG as an infection is common. Here, we present a case of PG in which pulmonary and spleen lesions preceded the cutaneous manifestations. The correct diagnosis was made 6 months after multiple nodules were detected in the lung and spleen, based on the development of skin wound ulcers...
February 2019: Internal Medicine Journal
Nour Abdullah Aljefri, Fayha Farraj Abothenain, Ahmed Mohamed Hussein, Waleed Saleh, Khaled Alkattan, Shamayel Faheem Mohammed, Mohammed Alhajji
Idiopathic Pleuro-Parenchymal Fibroelsatosis (PPFE) is a rare, progressive and recently recognized subtype of idiopathic interstitial lung disease with no recorded successful treatment other than lung transplant. We report a case of idiopathic pleuroparenchymal fibroelastosis from the Middle East, managed successfully by bilateral lung transplant performed on a 26 year old Saudi male.
January 2019: Annals of Thoracic Medicine
Seitaro Shimada, Takayuki Ando, Tomotaka Yokota, Miho Sakumura, Hiroki Yoshita, Sohachi Nanjo, Hiroshi Mihara, Shinya Kajiura, Haruka Fujinami, Ichiro Yasuda
Although nivolumab was previously reported to cause immune-related interstitial lung diseases (ILD), the detailed characteristics of ILD in gastric cancer are not fully understood. We herein present a rare case of a 66-year-old male with advanced gastric cancer who experienced acute-onset high-grade fever and dyspnea and diagnosed with early-onset ILD during the first cycle of nivolumab. Computed tomography revealed patchy infiltrative shadows and ground-glass opacities. No pathological bacteria were detected in the sputum or the bronchoalveolar lavage, and serous antigens for virus and beta-D-glucan were below the detection limit...
2019: Nihon Shokakibyo Gakkai Zasshi, the Japanese Journal of Gastro-enterology
Lijian Xie, Yun Li, Xunwei Jiang, Jian Zhao, Tingting Xiao
BACKGROUND: Abernethy malformation is an extremely rare congenital malformation characterised by an extrahepatic portosystemic shunt. Children with Abernathy malformation can develop hepatopulmonary syndrome (HPS) with pulmonary arteriovenous fistulas (PAVF) or pulmonary hypertension. PAVF manifests as central cyanosis with effort intolerance. We report a case of PAVF in a Ten-year-old Boy. Persistent symptoms identified Abernathy malformation as the cause of progressive symptoms and current understanding of this rare malformation is reviewed...
February 11, 2019: BMC Pediatrics
Katherine B Hisert, W Conrad Liles, Anne M Manicone
Research to understand the contribution of macrophages to non-resolving airway inflammation in cystic fibrosis (CF) and other chronic suppurative airways diseases has been hindered by a lack of methods for isolating and studying theses cells. With the development of technologies that can characterize small numbers of cells or individual cells, there is an even greater need for methodologies to isolate rare cells in heterogeneous specimens. Here, we describe a method that overcomes the technical obstacles imposed by sputum debris and apoptotic cells, and allows isolation of pure populations of macrophages from CF sputum...
February 11, 2019: American Journal of Respiratory Cell and Molecular Biology
Noor Sameh Darwich, Robert L Tyrrell
Bilateral pneumothoraces following unilateral subclavian vein cannulation is a rare complication. It is usually associated with an intrapleural communication which can be congenital or formed following cardiothoracic and mediastinal surgeries, or trauma. The term Buffalo chest was used to define a single chest cavity with no anatomic separation of the two hemithoraces. It is called Buffalo chest because this unique chest anatomy of the American buffalo, or bison, helped hunters to kill them with a single arrow to the chest because the resultant pneumothorax could collapse both lungs...
2019: Respiratory Medicine Case Reports
Amira Al-Aamri, Kamal Taha, Yousof Al-Hammadi, Maher Maalouf, Dirar Homouz
BACKGROUND: Understanding the genetic networks and their role in chronic diseases (e.g., cancer) is one of the important objectives of biological researchers. In this work, we present a text mining system that constructs a gene-gene-interaction network for the entire human genome and then performs network analysis to identify disease-related genes. We recognize the interacting genes based on their co-occurrence frequency within the biomedical literature and by employing linear and non-linear rare-event classification models...
February 8, 2019: BMC Bioinformatics
Hirotoshi Kawashima, Atsunari Utsugi, Asuka Shibamiya, Kazuma Iida, Norihiro Mimura, Hirohumi Ohashi, Ryota Hase, Makio Kawakami, Takao Yanagisawa, Masaki Hiraguri
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and immunoglobulin G4-related diseases (IgG4-RD) are regarded as entirely different disease types with different etiological mechanisms. However, we experienced two cases that had clinical features of both AAV and IgG4-RD. The first case is an 81-year-old woman who showed periaortitis and retroperitoneal fibrosis and periarteritis with elevation of myeloperoxidase-anti-neutrophil cytoplasmic antibody and IgG4 levels. The second case is a 63-year-old woman who had dura mater, ear, nose, lung, and kidney involvement with serum negative for ANCA and elevated IgG4...
February 8, 2019: Immunologic Research
Panpan Qian, Chun Hong Peng, Xianwei Ye
Introduction: Recently, interstitial lung disease significantly increases and it is difficult to treat. Cyclophosphamide(CP) is one drug administrated in interstitial lung disease, which can also cause pulmonary fibrosis and lung function lesion. This article present a case which exacerbated interstitial pneumonia after treatment by CP, aiming to enhance the understanding of the side effects of CP and standardize usage of the CP. Case presentation: A patient of nephrotic syndrome administrated with CP suffered respiratory insufficiency requiring mechanical ventilation...
2019: Respiratory Medicine Case Reports
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