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https://www.readbyqxmd.com/read/28195247/type-ii-congenital-pulmonary-airway-malformation-associated-with-intralobar-pulmonary-sequestration-report-of-a-case-and-review-of-classification-criteria
#1
M G Mastrogiulio, A Barone, M G Disanto, A Ginori, M R Ambrosio, S F Carbone, D Spina
Pulmonary congenital abnormalities are rare disorders including congenital pulmonary airway malformations (CPAM) and pulmonary sequestration (PS). CPAM is a lesion characterized by the presence of anomalous bronchiolar or acinar structures, variable in size, either cystic or not cystic. PS is generally defined as nonfunctioning lung tissue that is not in normal continuity with the tracheobronchial tree and that derives its blood supply from systemic vessels. We describe a case of a baby girl with a very rare association between CPAM type 2 and intralobar pulmonary sequestration (IPS) focusing on the cystic lesions typical of CPAM and on the lymphatic and blood vessels...
March 2016: Pathologica
https://www.readbyqxmd.com/read/28151982/birt-hogg-dube-syndrome-prospectively-detected-by-review-of-chest-computed-tomography-scans
#2
Hye Jung Park, Chul Hwan Park, Sang Eun Lee, Geun Dong Lee, Min Kwang Byun, Sungsoo Lee, Kyung-A Lee, Tae Hoon Kim, Seong Han Kim, Seo Yeon Yang, Hyung Jung Kim, Chul Min Ahn
PURPOSE: Birt-Hogg-Dube syndrome (BHD) is a rare disorder caused by mutations in the gene that encodes folliculin (FLCN) and is inherited in an autosomal dominant manner. BHD is commonly accompanied by fibrofolliculomas, renal tumors, multiple pulmonary cysts, and spontaneous pneumothorax. The aim of this study was to detect BHD prospectively in patients undergoing chest computed tomography (CT) scans and to evaluate further the characteristics of BHD in Korea. METHODS: We prospectively checked and reviewed the chest CT scans obtained for 10,883 patients at Gangnam Severance Hospital, Seoul, Korea, from June 1, 2015 to May 31, 2016...
2017: PloS One
https://www.readbyqxmd.com/read/28143893/giant-pulmonary-hydatid-cyst
#3
Chenglin Guo, Qiang Pu, Jiandong Mei, Lunxu Liu
No abstract text is available yet for this article.
January 31, 2017: Thorax
https://www.readbyqxmd.com/read/28132530/computed-tomography-lymphangiography-findings-in-27-cases-of-lymphangioleiomyomatosis
#4
Chunyan Zhang, Xiaobai Chen, Tingguo Wen, Qijin Zhang, Meng Huo, Jian Dong, Wen-Bin Shen, Rengui Wang
Background Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Material and Methods Twenty-seven patients with LAM underwent direct lymphangiography (DLG), followed by a post-procedural thoracoabdominal CT examination. Results All 27 patients were diagnosed with LAM, including 25 (93%) with chylothorax, eight (30%) with chyloperitoneum, seven (26%) with chylous fluid in the chest and abdomen, and one (4%) with lower-limb lymphedema...
January 1, 2017: Acta Radiologica
https://www.readbyqxmd.com/read/28127219/invasive-pericardial-hydatid-cyst-excision-of-multiple-huge-cysts
#5
Jignesh Kothari, Ketav Lakhia, Parth Solanki, Sahil Bansal, Hiren Boraniya, Himani Pandya, Himanshu Acharya
Cardiac hydatid cyst is rare even in endemic countries, and poses a therapeutic challenge due to varying presentation and unpredictable pre-, peri-, and postoperative complications. We herein present a case of multiple, multifocal, huge pericardial hydatid cyst, with invasion into the left ventricle and main pulmonary artery in a young male patient, presented with atypical chest pain.
January 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28123731/bone-langerhans-cell-histiocytosis-with-pulmonary-involvement-in-an-adult-non-smoker-a-case-report-and-brief-review-of-the-literature
#6
Jie Shen, Shicheng Feng
Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause, which encompasses a set of disorders of multiple organs with various clinical presentations that share the common characteristic of sizeable Langerhans cell infiltration. The clinical spectrum of LCH ranges from solitary bone lesions to involvement of other organs. lung involvement in LCH may be part of a multisystem disease, which almost exclusively occurs in adult smokers, while it is overlooked or misdiagnosed in the majority of non-smokers...
January 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28116411/meningoencephalitis-caused-by-pathogenic-sarcocystis-species-in-a-naturally-infected-sheep-in-turkey
#7
Aylin Alasonyalilar-Demirer, M Mufit Kahraman, Ahmet Akkoç, S Pelin Erturkuner, E Elif Guzel, Oguz Kul, Volkan Ipek
A 3-year-old sheep was examined after an acute onset of hind limb paralysis and ataxia. At necropsy, central nervous system, pulmonary and intestinal hyperaemia and ecchymoses in the aortic arch were observed. Main microscopic lesions were confined to the heart, cerebrum and cerebellum. There were a multifocal mild myocarditis and nonsuppurative meningoencephalitis together with protozoal cysts in the heart and the brain. Protozoal cystic structures were observed within many of the myocardial fibers as well as in the cerebrum and cerebellum...
February 9, 2017: Tierärztliche Praxis. Ausgabe G, Grosstiere/Nutztiere
https://www.readbyqxmd.com/read/28100056/pneumatosis-cystoides-intestinalis
#8
Adriana Fabiola Romano-Munive, Rafael Barreto-Zuñiga
A 54-year-old woman underwent colonoscopy for colon cancer screening. Colonoscopy showed multiple cysts in the sigmoid colon, with the largest being 4 cm in diameter. One of the cysts was biopsied. Cyst walls were observed; during biopsy, the gas was released and the cyst collapsed. Computed tomography of the abdomen confirmed a diagnosis of pneumatosis cystoides intestinalis. Pneumatosis cystoides intestinalis is a rare disease characterized by the presence in the intestinal submucosa or subserosa of multiple cysts filled with gas (nitrogen, oxygen, carbon dioxide and hydrogen)...
January 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28079847/relationship-between-abnormalities-on-high-resolution-computerized-tomography-pulmonary-function-and-bronchoalveolar-lavage-in-progressive-systemic-sclerosis
#9
Gulfidan Cakmak, Tuba Selcuk Can, Sule Gundogdu, Canan Akman, Hande Ikitimur, Benan Musellim, Gul Ongen
:  Introduction and aim: Progressive systemic sclerosis (pSS) is a multisystemic connective tissue disease characterized by fibrosis of the skin and internal organs including lung. The mechanisms that leads to progressive lung fibrosis in scleroderma remain obscure. In this study, we aimed to investigate the correlation between HRCT findings and patients' clinical and functional status and the degree of alveolitis based on the BAL resultsMaterials and methods: 65 patients with pSS were evaluated...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28076684/radiographic-differentiation-of-advanced-fibrocystic-lung-diseases
#10
Masanori Akira
The concept of end-stage lung disease suggests a final common pathway for most diffuse parenchymal lung diseases. In accordance with this concept, end-stage disease is characterized radiographically and pathologically by the presence of extensive honeycombing. However, sequential computed tomographic (CT) scans obtained from patients with chronic diffuse lung disease evolve over time to show various advanced lung disease patterns other than honeycombing. Also, several radiographically distinct honeycomb patterns, including microcystic, macrocystic, mixed, and combined emphysema and honeycombing differentiate one advanced lung disease from another...
January 11, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28076290/tailored-interactive-sequences-for-continuous-mr-image-guided-freehand-biopsies-of-different-organs-in-an-open-system-at-1-0-tesla-t-initial-experience
#11
Florian Streitparth, Christian Althoff, Martin Jonczyk, Felix Guettler, Martin Maurer, Hendrik Rathke, Keno Moritz Sponheuer, Bernd Hamm, Ulf K Teichgräber, Maximilian de Bucourt
OBJECTIVES: To assess the feasibility, image quality, and accuracy of freehand biopsies of liver, bone, muscle, vertebral disc, soft tissue, and other lesions using balanced steady-state free precession (SSFP, balanced fast field echo: bFFE), spoiled and nonspoiled gradient echo (FFE), and turbo spin echo (TSE) sequences for interactive continuous navigation in an open magnetic resonance imaging (MRI) system at 1.0 tesla (T). METHODS: Twenty-six MR-guided biopsies (five liver, five bone, four muscle, four vertebral disc, one lung, one kidney, one suprarenal gland, and five soft or other tissue) were performed in 23 patients in a 1...
January 11, 2017: Biomedizinische Technik. Biomedical Engineering
https://www.readbyqxmd.com/read/28069055/novel-germline-mutations-in-flcn-gene-identified-in-two-chinese-patients-with-birt-hogg-dub%C3%A3-syndrome
#12
Teng Li, Xianghui Ning, Qun He, Kan Gong
Birt-Hogg-Dubé (BHD) syndrome, a hereditary renal cancer syndrome caused by mutations in the folliculin (FLCN) gene, is characterized by the presence of fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax, and renal cell carcinoma (RCC). Few BHD syndrome cases have been reported in Asian countries, and cutaneous presentations are relatively rare in Asian patients. Asian BHD patients may be misdiagnosed due to their atypical manifestations. Here, we report two Chinese BHD patients with novel FLCN mutations (c...
January 9, 2017: Chinese Journal of Cancer
https://www.readbyqxmd.com/read/28060131/nonfunctioning-adrenocortical-carcinoma-in-pediatric-acute-lymphoblastic-leukemia-a-case-report-of-a-rare-multiple-primaries-combination
#13
Wafaa M Rashed, Wael Zekri, Madiha Awad, Hala Taha, Badr Abdalla, Ahmad S Alfaar
Childhood adrenocortical carcinoma (ACC) is a rare tumor and its association with acute lymphoblastic leukemia (ALL) is even rarer. One such case is discussed in this case report. A 3-year-old patient was concomitantly diagnosed with ALL and an initially nonmetastatic ACC. Management started by following the Total XV protocol without a window phase. Left adrenalectomy was conducted after the consolidation phase. Recurrence of a mass at the tumor bed was discovered at week 33 of the continuation phase. Reexcision was conducted, followed by the administration of an ACC protocol including cisplatin, etoposide, and doxirubicin...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28050776/enlarged-mediastinal-air-cyst-in-a-patient-with-bronchial-diverticula-localized-in-the-left-main-bronchus-a-case-report-with-surgical-and-bronchoscopic-findings
#14
Hideo Ichimura, Yuichiro Ozawa, Masanari Shiigai, Seiji Shiotani, Kazunori Kikuchi, Yukio Sato
BACKGROUND: A mediastinal air cyst is a rare computed tomography (CT) finding. Once the lesion is identified, it is difficult to diagnose and treat. Meanwhile, bronchial diverticula have been reported as a CT finding observed in certain pulmonary pathologic conditions. We encountered the case of an enlarged mediastinal air cyst accompanied with bronchial diverticula and upper lobe-dominant fibrous changes of the lung. CASE PRESENTATION: A 69-year-old man with a chronic cough who had regularly visited a chest physician for upper lobe-dominant pulmonary fibrosis was referred to our hospital for the examination of an enlarged mediastinal air cyst...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28012778/b-cell-and-antibody-responses-in-mice-induced-by-a-putative-cell-surface-peptidase-of-pneumocystis-murina-protect-against-experimental-infection
#15
Sanbao Ruan, Yang Cai, Alistair J Ramsay, David A Welsh, Karen Norris, Judd E Shellito
RATIONALE: Pneumocystis pneumonia is a major cause of morbidity and mortality in HIV-infected subjects, cancer patients undergoing chemotherapy and solid organ transplant recipients. No vaccine is currently available. By chemical labeling coupled with proteomic approach, we have identified a putative surface protein (SPD1, Broad Institute gene accession number PNEG_01848) derived from single suspended P. murina cysts. SPD1 was expressed in an insect cell line and tested for vaccine development...
January 23, 2017: Vaccine
https://www.readbyqxmd.com/read/28009417/multiple-chromophobe-and-clear-cell-renal-cancer-in-a-patient-affected-by-birt-hogg-dub%C3%A3-syndrome-a-case-report
#16
Roberto Castellucci, Michele Marchioni, Sergio Valenti, Giusepe Sortino, Giulio Borgonovo, Nicola Pesenti, Alberto C A Vismara, Maria C Circo, Barbara Sessa, Emanuele Micheli, Antonino Lembo
OBJECTIVES: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant characterized by the presence of fibrofolliculomas and/or trichodiscomas, pulmonary cysts, spontaneous pneumothorax, and renal tumors. The syndrome is linked to mutations in the FLCN gene, which is preferentially expressed in the skin, kidney, and lung. The aim of our paper is to describe a case of multiple bilateral renal cancer in a patient affected by BHDS. CASE PRESENTATION: Patient subjected to enucleoresection seven kidney tumors discovered right after ultrasound performed for other reasons...
December 16, 2016: Urologia
https://www.readbyqxmd.com/read/27990306/primary-pericardial-sarcoma-with-right-atrial-invasion-and-multiple-bilateral-pulmonary-metastases-in-a-patient-with-hereditary-nonpolyposis-colorectal-cancer
#17
Eugene Wong, Lawrence J Oh, Kazi Nahar, Adrian Lee, Stephen Clarke
Primary tumours originating from the pericardium are extremely rare. Previous studies have reported that these tumours account for only 6.7-12.8% of all mediastinal tumours with an overall prevalence of 0.001% to 0.007%. The majority of these tumours are benign lipomas or pericardial cysts. The most common pericardial malignancy is mesothelioma. Sarcomas are soft-tissue mesenchymal malignancies originating from various parts of the body but are extremely rare in this area. We report a case of a 52-year-old female who was diagnosed with a primary sarcoma with rhabdoid differentiation originating from the pericardium...
2016: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/27938546/-soft-tissue-angiosarcoma-with-pulmonary-metastases-case-report-and-literature-review
#18
X L Mu, Z X Wu, H J Liang, Z Wang, X Fang, D H Yang, Z L Cao, Z C Gao
Objective: To investigate the differential diagnosis between pulmonary metastases from soft-tissue angiosarcoma and primary pulmonary angiosarcoma. Methods: A case of soft-tissue angiosarcoma with pulmonary metastases was reported and related literatures were reviewed. Results: A 39 year-old man complaining of hemoptysis, cough, and sputum for 10 months was admitted to our hospital in September 2013. He was initially diagnosed as having primary pulmonary angiosarcoma after wedge-resection biopsy of the lung...
December 12, 2016: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/27905298/haploinsufficiency-of-the-folliculin-gene-leads-to-impaired-functions-of-lung-fibroblasts-in-patients-with-birt-hogg-dub%C3%A3-syndrome
#19
Yoshito Hoshika, Fumiyuki Takahashi, Shinsaku Togo, Muneaki Hashimoto, Takeshi Nara, Toshiyuki Kobayashi, Fariz Nurwidya, Hideyuki Kataoka, Masatoshi Kurihara, Etsuko Kobayashi, Hiroki Ebana, Mika Kikkawa, Katsutoshi Ando, Koichi Nishino, Okio Hino, Kazuhisa Takahashi, Kuniaki Seyama
Birt-Hogg-Dubé syndrome (BHDS) is an autosomal dominant inherited disorder caused by germline mutations in the FLCN gene, and characterized by skin fibrofolliculomas, multiple lung cysts, spontaneous pneumothorax, and renal neoplasms. Pulmonary manifestations frequently develop earlier than other organ involvements, prompting a diagnosis of BHDS However, the mechanism of lung cyst formation and pathogenesis of pneumothorax have not yet been clarified. Fibroblasts were isolated from lung tissues obtained from patients with BHDS (n = 12) and lung cancer (n = 10) as controls...
November 2016: Physiological Reports
https://www.readbyqxmd.com/read/27904546/cured-transudative-pleural-effusion-a-case-report
#20
Mohsen Shafipoor, Arda Kiani, Kambiz Sheikhy, Atefeh Abedini, Majid Golestani Eraghi
Echinococcosis or hydatid disease is a helminthic infection caused by larvae of tapeworm Echinococcus granulosus. While the cysts can involve all organs, liver is the most common site of infection and the lungs are the second most commonly involved organ in young adults. In addition to endemic areas its incidence is growing all around the world due to the ease of transcontinental travel. Disease presentation varies and usually is due to mass effect or dysfunction of the involved organ and surgical resection is the recommended treatment...
2016: Tanaffos
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