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idiopathic pulmonary

Aiko Ogawa, Yukari Takahashi, Hiromi Matsubara
BACKGROUND: Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension. Although diagnosis is based on pathological findings, an early diagnosis is crucial because of poor prognosis compared to other types of pulmonary hypertension. Furthermore, vasodilators may cause fatal pulmonary edema in patients with PVOD/PCH. This study aimed to identify specific characteristics for patients with PVOD/PCH to clinically diagnose PVOD/PCH...
March 13, 2018: Journal of Cardiology
Shinichiro Ohshimo, Ulrich Costabel, Nobuaki Shime
No abstract text is available yet for this article.
March 2018: Respiratory Investigation
Yoshinori Tanino
No abstract text is available yet for this article.
March 2018: Respiratory Investigation
Tetsuro Sawata, Masashi Bando, Masayuki Nakayama, Naoko Mato, Hideaki Yamasawa, Masaharu Takahashi, Hiroaki Okamoto, Yukihiko Sugiyama
BACKGROUND: Several studies have reported that viral infections are related to lung cancer. We previously reported the involvement of Torque teno virus (TTV) in patients with lung cancer and idiopathic pulmonary fibrosis. However, the role of TTV in lung cancer growth, and its influence on changes in TTV DNA titers due to idiopathic pulmonary fibrosis (IPF) in lung cancer patients are poorly understood. METHODS: Serum TTV DNA titers were measured in serum samples obtained from patients with lung cancer...
March 2018: Respiratory Investigation
Susumu Sakamoto, Hiroshige Shimizu, Takuma Isshiki, Keishi Sugino, Atsuko Kurosaki, Sakae Homma
BACKGROUND AND OBJECTIVES: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often-fatal condition associated with endothelial damage and abnormalities of the coagulation system. Recombinant human soluble thrombomodulin (rhTM) has anti-inflammatory effects and regulates the coagulation pathway. This study evaluated the effectiveness of rhTM for the treatment of AE-IPF. METHODS: This historically controlled study included 80 patients with AE-IPF admitted to our center during the period from 2006 through 2016...
March 2018: Respiratory Investigation
Kenji Yorita, Kazuya Tsuji, Yoko Takano, Naoto Kuroda, Kei Sakamoto, Kaoru Arii, Yukio Yoshimoto, Kimiko Nakatani, Satoshi Ito
BACKGROUND: Acantholytic squamous cell carcinoma (ASQCC), histologically characterized by intercellular bridge loosening, is recognized as a rare variant of squamous cell carcinoma (SQCC). ASQCC may demonstrate a worse prognosis than conventional SQCC. Pulmonary ASQCC is particularly rare; its biological behavior and prognostic data have not been reported. CASE PRESENTATION: We report the clinical and autopsy findings of a 71-year-old Japanese man with pulmonary ASQCC...
March 16, 2018: BMC Cancer
Jonathan H Chung, Justin M Oldham, Steven M Montner, Rekha Vij, Ayodeji Adegunsoye, Aliya N Husain, Imre Noth, David A Lynch, Mary E Strek
OBJECTIVE: The purpose of this study was to assess the diagnostic significance of CT patterns that cannot be classified according to current idiopathic pulmonary fibrosis (IPF) guidelines and of specific findings of the inconsistent with usual interstitial pneumonitis (UIP) pattern. MATERIALS AND METHODS: Subjects with a multidisciplinary diagnosis of interstitial lung disease who had undergone surgical lung biopsy and chest CT within 1 year of each other were included in the study...
March 16, 2018: AJR. American Journal of Roentgenology
Elizabeth F Redente, Martin A Aguilar, Bart P Black, Benjamin Edelman, Ali Bahadur, Stephen M Humphries, David A Lynch, Lutz Wollin, David W H Riches
Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) develops in approximately 20% of patients with RA. SKG mice, which are genetically prone to developing autoimmune arthritis, develop a pulmonary interstitial pneumonia that resembles human cellular and fibrotic non-specific interstitial pneumonia. Nintedanib, a tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis, has been shown to reduce lung function decline. Therefore, we investigated the effect of nintedanib on the development of pulmonary fibrosis and joint disease in female SKG mice with arthritis induced by intraperitoneal injection of zymosan (5 mg)...
March 15, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
Johannes Krämer, Felix Kreuzer, Michael Kaestner, Peter Bride, Fabian von Scheidt, Jannos Siaplaouras, Heiner Latus, Dietmar Schranz, Christian Apitz
Right ventricular (RV) hypertrophy is regarded as the adaptation on chronic RV pressure load in pulmonary hypertension. As the RV Sokolow-Lyon index (RVSLI) is an electrocardiographic marker of RV hypertrophy, we hypothesized that RVSLI might be able to reflect RV pressure load. Therefore, the purpose of this study was to characterize the diagnostic impact of the RVSLI in children with idiopathic pulmonary arterial hypertension (IPAH) in order to assess disease severity and to evaluate its value for the prediction of worse outcome...
March 14, 2018: Pediatric Cardiology
Yuichi Mukai, Toshihiko Agatsuma, Gen Ideura
Idiopathic pulmonary haemosiderosis (IPH) is a diagnosis of exclusion, which is characterized by persistent or recurrent episodes of alveolar haemorrhage. Early diagnosis of IPH, especially in the case of first-time manifestation, is challenging because previous episodes of alveolar haemorrhage are often difficult to prove. Repeated episodes of alveolar haemorrhage can result in chronic iron-deficient anaemia and irreversible interstitial fibrosis; thus, early recognition and intervention are desirable in terms of clinical outcome...
April 2018: Respirology Case Reports
Yufeng Du, Xiaoyan Hao, Xuejun Liu
The present study aimed to investigate the expression of long non-coding RNA (lncRNA) cyclin dependent kinase inhibitor-2B-antisense RNA 1 CDKN2B-AS1 in patients with peripheral blood of idiopathic pulmonary fibrosis (IPF). A total of 24 patients with IPF and 24 healthy controls were included in the study, four patients with IPF and four healthy controls were selected randomly to extract RNA. There were no other diseases such as hypertension and diabetes in the two groups. RNA from peripheral blood was extracted by high-throughput sequencing and bioinformatics analysis was performed...
April 2018: Oncology Letters
Shubhra Jain, Vinod Joshi, Yogendra S Rathore, Narendra Khippal
Several occupational hazards, especially exposure to silica, have been implicated as causal factors for the development of scleroderma-like disorders. Compared to other connective tissue disorders, silica-associated systemic sclerosis (SA-SS) is relatively rare. Silica-induced scleroderma is indistinguishable from idiopathic systemic sclerosis. However, the former expresses a high predisposition of pulmonary involvement and anti-Scl-70 antibody. We report the case of a 42-year-old male, stone cutter by occupation, who was diagnosed as simple chronic silicosis and developed systemic sclerosis...
May 2017: Indian Journal of Occupational and Environmental Medicine
Gülsüm Özkan, Gaye Kübra Emeksiz, Reşit Volkan Atar, Samet Sedef, Pınar Sonat Kara, Meltem Öznur, Burhan Turgut
Purpura fulminans associated with antithrombin 3 (AT 3) deficiency is very rare in adults and neonates. It can be categorized into three principal forms - neonatal, idiopathic and acute infectious. Purpura fulminans has been reported to cause cardiac, pulmonary and renal damage in rare cases. We describe an adult case of purpura fulminans developing in association with AT 3 deficiency without infection following a surgical procedure, and acute kidney injury (AKI) developing secondary to rhabdomyolysis and disseminated intravascular coagulation (DIC)...
2018: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
Hye In Jung, Jae Seok Park, Mi-Young Lee, ByeongJu Park, Hyun Jung Kim, Sun Hyo Park, Won-Il Choi, Choong Won Lee
We aimed to explore lung cancer prevalence in interstitial lung disease (ILD) patients with or without connective tissue disorder (CTD) and idiopathic pulmonary fibrosis (IPF) in comparison with chronic obstructive pulmonary disorder (COPD).We evaluated lung cancer prevalence associated with ILD and IPF using Korean Health Insurance Review and Assessment Service (HIRA) data from January to December 2011. This database (HIRA-NPS-2011-0001) was sampled using random sampling of outpatients; 1,375,842 sample cases were collected, and 670,258 (age ≥ 40 ys) were evaluated...
March 2018: Medicine (Baltimore)
Marjolein Drent, Petal Wijnen, Aalt Bast
PURPOSE OF REVIEW: Fibrosing interstitial pneumonias are associated with various stages of fibrosis. The cause of this group of syndromes remains largely unknown. For most of these diseases, a genetic basis, environmental factors and certain triggers have been suggested as possible risk factors. Various studies have found an association between genetic polymorphisms, or the presence of certain variant alleles, and the occurrence and/or progression of interstitial pneumonias of unknown origin...
March 13, 2018: Current Opinion in Pulmonary Medicine
Azam Peyvandipour, Nafiseh Saberian, Adib Shafi, Michele Donato, Sorin Draghici
Motivation: Identification of novel therapeutic effects for existing U.S. Food and Drug Administration (FDA)-approved drugs, drug repurposing, is an approach aimed to dramatically shorten the drug discovery process, which is costly, slow and risky. Several computational approaches use transcriptional data to find potential repurposing candidates. The main hypothesis of such approaches is that if gene expression signature of a particular drug is opposite to the gene expression signature of a disease, that drug may have a potential therapeutic effect on the disease...
March 9, 2018: Bioinformatics
Gali Epstein Shochet, Lutz Wollin, David Shitrit
BACKGROUND AND OBJECTIVE: Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with poor prognosis. Activated fibroblasts are the key effector cells in fibrosis, producing excessive amounts of collagen and extracellular matrix (ECM) proteins. Whether the ECM conditioned by IPF fibroblasts determines the phenotype of naïve fibroblasts is difficult to explore. METHODS: IPF-derived primary fibroblasts were cultured on Matrigel and then cleared using ammonium hydroxide, creating an IPF-conditioned matrix (CM)...
March 12, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
Yangyang Gu, Bo Huang, Yanfei Yang, Mengdie Qi, Guohua Lu, Dajing Xia, Hequan Li
Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible lung disease with high mortality rate. The etiology is unknown and treatment choices are limited. Thus, there is great interest to investigate novel agents for IPF therapy. Ibrutinib, BTK, and ITK irreversible inhibitor is a FDA-approved small molecule for the clinical therapy of B cell lymphoma. Its role in pulmonary fibrosis remains unknown. In this study, we investigated the anti-fibrotic activity of ibrutinib. Strikingly, ibrutinib did not inhibit but exacerbated bleomycin-induced pulmonary fibrosis by increased epithelial cell apoptosis, and inflammation in the lung...
March 13, 2018: Inflammation
Laurens J De Sadeleer, Ellen De Langhe, Nicolas Bodart, Alain Vigneron, Xavier Bossuyt, Wim A Wuyts
Although included in the serological domain of the 'interstitial pneumonia with auto-immune features' (IPAF) research statement, the search for myositis-specific antibodies (MSA) is not incorporated in routine clinical practice. The objective of the study was to evaluate MSA prevalence in an idiopathic interstitial pneumonia (IIP) cohort (n = 68) with suggestive morphological interstitial lung disease patterns. Twelve of 68 patients (17.6%) carried MSA, whereof only two were anti-nuclear antibody-positive...
March 12, 2018: Lung
Charlotte H Maden, David Fairman, Michelle Chalker, Maria J Costa, William A Fahy, Nadia Garman, Pauline T Lukey, Tim Mant, Simon Parry, Juliet K Simpson, Robert J Slack, Stuart Kendrick, Richard P Marshall
PURPOSE: Inhaled drug delivery is an attractive route by which to deliver drugs to lungs of patients with idiopathic pulmonary fibrosis (IPF). GSK3008348 is a potent and selective small molecule being developed as the first inhaled inhibitor of the αvβ6 integrin for the treatment of IPF. The phase 1 first-time-in-human clinical trial (NCT02612051) presented here was designed to investigate the safety, tolerability and pharmacokinetic (PK) profile of single doses of GSK3008348 in healthy participants...
March 12, 2018: European Journal of Clinical Pharmacology
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