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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
#1
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
#2
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
https://www.readbyqxmd.com/read/27904052/recent-advances-in-the-diagnosis-of-pneumocystis-pneumonia
#3
Yinggai Song, Yi Ren, Xiaowen Wang, Ruoyu Li
Pneumocystis jirovecii is a prototypical opportunistic pathogen, causing an asymptomatic or mild infection in normal hosts and fulminating pneumonia (Pneumocystis pneumonia, PCP) in immunocompromised hosts. PCP is a leading cause of morbidity and mortality in immunocompromised patients such as AIDS patients. Microscopic detection of cysts and trophic forms of P. jirovecii in respiratory secretions is simple and useful but may underestimate the P. jirovecii infection. Conventional polymerase chain reaction (PCR) and nested PCR increase the sensitivity and specificity to identify PCP and provide an approach to discriminate PCP from pulmonary P...
2016: Medical Mycology Journal
https://www.readbyqxmd.com/read/27902675/infected-congenital-epicardial-cyst-presenting-as-acute-abdomen
#4
Timothy Dribin, Matthew D Files, Erin R Rudzinski, Ron Kaplan, Kimberly P Stone
A previously healthy 3-year-old boy presented to the emergency department with abdominal pain, fever, and emesis. Laboratory and radiologic evaluation for causes of acute abdomen were negative; however, review of the abdominal x-ray demonstrated cardiomegaly with the subsequent diagnosis of pericardial cyst by echocardiogram and computed tomography. The patient underwent surgical decompression and attempted removal of the cystic structure revealing that the cyst originated from the epicardium. His abdominal pain and fever resolved postoperatively and he completed a 3-week course of ceftriaxone for treatment of Propionibacterium acnes infected congenital epicardial cyst...
December 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27898174/treatment-of-a-giant-pulmonary-emphysematous-cyst-with-primary-bronchoalveolar-papillary-carcinoma-in-a-shih-tzu-dog
#5
Jiyoung Park, Hae-Beom Lee, Seong Mok Jeong
OBJECTIVE: To report the surgical treatment of a pulmonary emphysematous cyst concurrent with primary pulmonary bronchoalveolar papillary carcinoma in a dog. STUDY DESIGN: Clinical case report. ANIMALS: 12-year-old 6.4 kg spayed female Shih Tzu dog. METHODS: The dog presented for surgical treatment of pulmonary emphysema. Radiography revealed that more than half of the left caudal lung lobe was enlarged and hyperlucent and computed tomography (CT) confirmed the presence of an emphysematous space...
November 28, 2016: Veterinary Surgery: VS
https://www.readbyqxmd.com/read/27876940/echinococcus-granulosus-in-humans-associated-with-disease-incidence-in-domestic-animals-in-kermanshah-west-of-iran
#6
Abdolali Chalechale, Mohammad Hashemnia, Farid Rezaei, Maryam Sayadpour
Hydatidosis is one of the important zoonotic diseases that cause considerable economic losses and public health problems worldwide. This study was conducted to determine the prevalence Echinococcus granulosus in people and slaughtered animals in Kermanshah province, west of Iran. Hospitals data and meat-inspection records in Kermanshah abattoir were used in this study. A total number of 32,130 slaughtered livestock (7000 cattle, 19,950 sheep and 5180 goats) was inspected in the 3-year period and overall 2043 (6...
December 2016: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
https://www.readbyqxmd.com/read/27876271/hiv-infection-is-independently-associated-with-increased-ct-scan-lung-density
#7
M Bradley Drummond, Allison A Lambert, Amira F Hussien, Cheng T Lin, Christian A Merlo, Robert A Wise, Gregory D Kirk, Robert H Brown
RATIONALE AND OBJECTIVES: Noninfectious pulmonary complications are common among HIV-infected individuals and may be detected early by quantitative computed tomography (CT) scanning. The association of HIV disease markers with CT lung density measurement remains poorly understood. MATERIALS AND METHODS: One hundred twenty-five participants free of spirometry-defined lung disease were recruited from a longitudinal cohort study of HIV-infected and HIV-uninfected individuals to undergo standardized CT scan of the chest...
November 18, 2016: Academic Radiology
https://www.readbyqxmd.com/read/27871249/benign-clear-cell-sugar-tumor-of-the-lung-in-a-patient-with-birt-hogg-dub%C3%A3-syndrome-a-case-report
#8
Yoko Gunji-Niitsu, Toshio Kumasaka, Shigehiro Kitamura, Yoshito Hoshika, Takuo Hayashi, Hitoshi Tokuda, Riichiro Morita, Etsuko Kobayashi, Keiko Mitani, Mika Kikkawa, Kazuhisa Takahashi, Kuniaki Seyama
BACKGROUND: Birt-Hogg-Dubé (BHD) syndrome is a rare inherited autosomal genodermatosis and caused by germline mutation of the folliculin (FLCN) gene, a tumor suppressor gene of which protein product is involved in mechanistic target of rapamycin (mTOR) signaling pathway regulating cell growth and metabolism. Clinical manifestations in BHD syndrome is characterized by fibrofolliculomas of the skin, pulmonary cysts with or without spontaneous pneumothorax, and renal neoplasms. There has been no pulmonary neoplasm reported in BHD syndrome, although the condition is due to deleterious sequence variants in a tumor suppressor gene...
November 21, 2016: BMC Medical Genetics
https://www.readbyqxmd.com/read/27867584/birt-hogg-dube-syndrome-accompanied-by-pulmonary-arteriovenous-malformation
#9
Noriyuki Matsutani, Hitoshi Dejima, Yusuke Takahashi, Hirofumi Uehara, Hisae Iinuma, Fumihiko Tanaka, Masafumi Kawamura
A 25-year-old male with Birt-Hogg-Dube (BHD) syndrome who developed hemothorax caused by ruptured of pulmonary arteriovenous malformation was reported. The patient was admitted to the hospital due to chest pain. A chest X-ray showed pleural fluid in his left lung, and a chest CT showed the presence of a tumor with enhanced contrast in the lower left lobe of approximately 5 cm in a diameter. Pleural fluid was collected by tap and indicated the presence of blood; therefore, hemothorax was suspected. Thoracoscopic surgery was performed to remove the mass for homostasis...
October 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/27829068/clinical-course-and-changes-in-high-resolution-computed-tomography-findings-in-patients-with-idiopathic-pulmonary-fibrosis-without-honeycombing
#10
Hiroyoshi Yamauchi, Masashi Bando, Tomohisa Baba, Kensuke Kataoka, Yoshihito Yamada, Hiroshi Yamamoto, Atsushi Miyamoto, Soichiro Ikushima, Takeshi Johkoh, Fumikazu Sakai, Yasuhiro Terasaki, Akira Hebisawa, Yoshinori Kawabata, Yukihiko Sugiyama, Takashi Ogura
Some patients with idiopathic pulmonary fibrosis (IPF) do not have honeycombing on high-resolution computed tomography (HRCT) at their initial evaluation. The clinical course and sequential changes in HRCT findings in these patients are not fully understood. We reviewed the cases of 43 patients with IPF without honeycombing on initial HRCT from institutions throughout Japan. All patients were diagnosed with IPF based on a surgical lung biopsy. Multidisciplinary discussions were held five times between 2011 and 2014, to exclude alternative etiologies...
2016: PloS One
https://www.readbyqxmd.com/read/27798316/facial-drooping-aphasia-and-an-incidental-lung-mass-in-a-nonsmoker
#11
Justin K Lui, Lacey J McIntosh, Jorge Escobar Valle, Thomas Stockl, Stephen Summers
BACKGROUND: Atrial fibrillation and atrial flutter are atrial tachycardias associated with embolic strokes. To date, there have only been a few reports highlighting the incidence of these atrial tachycardias due to mechanical compression of myocardial structures and the pulmonary vasculature in certain mediastinal masses and cysts. CASE: We present a case of a 75-year-old gentleman who is a nonsmoker with a history of hypertension who presents with an acute embolic stroke due to atrial flutter likely from mechanical compression from an underlying squamous cell carcinoma of the lung...
October 26, 2016: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/27797454/thoracic-mri-evaluation-of-sarcoidosis-in-children
#12
Sureyya Burcu Gorkem, Seçil Köse, Edward Y Lee, Selim Doğanay, Ayse Seda Coskun, Mehmet Köse
BACKGROUND: Childhood sarcoidosis is a very rare granulomatous disorder with an unknown etiology. Stage 1 disease is the most common whereas stages 2, 3, and 0 are rare in children. OBJECTIVE: To evaluate thoracic findings of pediatric pulmonary sarcoidosis on MRI and to compare them with CT findings. METHODS: Between August 2010 and May 2015, seven consecutive pediatric patients (four male, three female; age range: 8-18 years, mean age: 13...
October 31, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27780965/a-case-of-birt-hogg-dub%C3%A3-bhd-syndrome-harboring-a-novel-folliculin-flcn-gene-mutation
#13
Takuro Yukawa, Takuya Fukazawa, Masakazu Yoshida, Ichiro Morita, Katsuya Kato, Yasumasa Monobe, Mitsuko Furuya, Yoshio Naomoto
BACKGROUND Birt-Hogg-Dubé (BHD) syndrome is an autosomal dominant disorder clinically characterized by pulmonary cysts, spontaneous pneumothorax, renal cell cancer, and skin fibrofolliculomas. The disorder is caused by germline mutations in the FLCN gene. CASE REPORT A 56-year-old female was admitted to our hospital with a diagnosis of bilateral spontaneous pneumothorax. A computed tomography (CT) scan of the chest revealed bilateral multiple bullae predominantly located in the subpleural and mediastinal areas in the bilateral upper and lower lobes...
October 26, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27760423/endosonography-of-a-pulmonary-artery-obstruction-in-echinococcosis
#14
Mark J Schuuring, Peter I Bonta, Michele van Vugt, Frank Smithuis, Otto M van Delden, Jouke T Annema, Kees Stijnis
A 44-year-old woman with a history of pulmonary embolism and abdominal echinococcosis complained of sudden thoracic pain and shortness of breath. A D-dimer of 77.5 mg/l (reference ≤0.5 mg/l) was found. Chest CT scan revealed obstruction of the right lower and middle lobe pulmonary artery (PA). Anticoagulation therapy was initiated for the presumed diagnosis of recurrent pulmonary embolism. However, due to persistent symptoms of dyspnea, follow-up CT angiography of the chest was performed 3 months later. A persistent PA obstruction was found and the presumed diagnosis of embolism was questioned...
2016: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/27756520/prevalence-and-clinical-significance-of-extracardiac-findings-in-cardiovascular-magnetic-resonance
#15
Agne Ulyte, Nomeda Valeviciene, Darius Palionis, Simona Kundrotaite, Algirdas Tamosiunas
OBJECTIVE: In cardiac magnetic resonance imaging (CMR), incidental pathological findings are frequently found outside the investigated cardiovascular system. Some of these findings might have clinical implications. The aim of this study was to determine the prevalence of incidental extracardiac findings (ECF) in CMR and their clinical significance. METHODS: A total of 4165 CMR reports from 2009-2012 were retrospectively reviewed for ECF. Two hundred-twenty reports with ECF were found...
September 22, 2016: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/27734028/nk-cell-activating-receptor-ligand-expression-in-lymphangioleiomyomatosis-is-associated-with-lung-function-decline
#16
Andrew R Osterburg, Rebecca L Nelson, Benyamin Z Yaniv, Rachel Foot, Walter R F Donica, Madison A Nashu, Huan Liu, Kathryn A Wikenheiser-Brokamp, Joel Moss, Nishant Gupta, Francis X McCormack, Michael T Borchers
Lymphangioleiomyomatosis (LAM) is a rare lung disease of women that leads to progressive cyst formation and accelerated loss of pulmonary function. Neoplastic smooth muscle cells from an unknown source metastasize to the lung and drive destructive remodeling. Given the role of NK cells in immune surveillance, we postulated that NK cell activating receptors and their cognate ligands are involved in LAM pathogenesis. We found that ligands for the NKG2D activating receptor UL-16 binding protein 2 (ULBP2) and ULBP3 are localized in cystic LAM lesions and pulmonary nodules...
October 6, 2016: JCI Insight
https://www.readbyqxmd.com/read/27722904/genetically-diagnosed-birt-hogg-dub%C3%A3-syndrome-and-familial-cerebral-cavernous-malformations-in-the-same-individual-a-case-report
#17
James Whitworth, Brian Stausbøl-Grøn, Anne-Bine Skytte
When faced with an unusual clinical feature in a patient with a Mendelian disorder, the clinician may entertain the possibilities of either the feature representing a novel manifestation of that disorder or the co-existence of a different inherited condition. Here we describe an individual with a submandibular oncocytoma, pulmonary bullae and renal cysts as well as multiple cerebral cavernous malformations and haemangiomas. Genetic investigations revealed constitutional mutations in FLCN, associated with Birt-Hogg-Dubé syndrome (BHD) and CCM2, associated with familial cerebral cavernous malformation...
October 8, 2016: Familial Cancer
https://www.readbyqxmd.com/read/27722781/lung-function-in-asphalt-pavers-a-longitudinal-study
#18
Bente Ulvestad, Britt Grethe Randem, Øivind Skare, Trond Mogens Aaløkken, Georg Karl Myranek, Karine Elihn, May Brit Lund
PURPOSE: To study longitudinal changes in lung function in asphalt pavers and a reference group of road maintenance workers, and to detect possible signs of lung disease by high-resolution computed tomography (HRCT) scans. METHODS: Seventy-five asphalt pavers and 71 road maintenance workers were followed up with questionnaires and measurements of lung function. Not every worker was tested every year, but most of them had four or more measurement points. The 75 asphalt pavers were also invited to have HRCT scans of the lungs at the end of the follow-up period...
October 8, 2016: International Archives of Occupational and Environmental Health
https://www.readbyqxmd.com/read/27716413/pulmonary-tumor-diagnosed-as-an-undifferentiated-sarcoma-with-epithelioid-features-a-case-report
#19
Mohamed Réda El Ochi, Mohammed Massine El Hammoumi, Abdelhamid Biyi, Mohamed Allaoui, El Hassane Kabiri, Abderrahman Albouzidi, Mohamed Oukabli
BACKGROUND: Pulmonary sarcomas are uncommon accounting for 0.5 % of all primary lung cancers. Undifferentiated sarcomas account for up to 20 % of soft tissue sarcomas. A lung tumor revealed to be an undifferentiated sarcoma with epithelioid features has never been reported in the literature. CASE PRESENTATION: A 61-year-old white Moroccan man presented with 2 months' history of hemoptysis and dyspnea. Chest computed tomography showed a cystic mass involving the lower field of his right lung evoking first a hydatid cyst...
October 3, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27715655/thin-section-ct-features-of-idiopathic-pulmonary-fibrosis-correlated-with-micro-ct-and-histologic-analysis
#20
Cindy Mai, Stijn E Verleden, John E McDonough, Stijn Willems, Walter De Wever, Johan Coolen, Adriana Dubbeldam, Dirk E Van Raemdonck, Eric K Verbeken, Geert M Verleden, James C Hogg, Bart M Vanaudenaerde, Wim A Wuyts, Johny A Verschakelen
Purpose To elucidate the underlying lung changes responsible for the computed tomographic (CT) features of idiopathic pulmonary fibrosis (IPF) and to gain insight into the way IPF proceeds through the lungs and progresses over time. Materials and Methods Micro-CT studies of tissue cores obtained from explant lungs were examined and were correlated 1:1 with a CT study obtained immediately before transplantation. Samples for histologic analysis were obtained from selected cores. Results In areas with no or minimal abnormalities on CT images, small areas of increased attenuation located in or near the interlobular septa can be seen on micro-CT studies...
October 6, 2016: Radiology
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