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https://www.readbyqxmd.com/read/28090043/pheochromocytoma-multisystem-crisis-behaving-like-interstitial-pneumonia-an-autopsy-case
#1
Yohta Nomoto, Kiyoshi Kawano, Naoki Fujisawa, Keiko Yoshida, Tomoko Yamashita, Naoki Makita, Hiroaki Takeshita, Kimio Kamimori, Shiro Yanagi, Minoru Yoshiyama
Pheochromocytoma multisystem crisis is a rare and life-threatening disease that is associated with numerous symptoms and which is also difficult to diagnose. We herein report an autopsy case of a 61-year-old man who died due to pheochromocytoma multisystem crisis. The patient complained of vomiting and breathlessness. Computed tomography showed a shadow-like region with a similar appearance to interstitial pneumonia. The patient was diagnosed with takotsubo cardiomyopathy induced by severe lung disease based on the results of echocardiography and coronary angiography...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28089977/antimelanoma-differentiation-associated-gene-5-antibody-expanding-the-clinical-spectrum-in-north-american-patients-with-dermatomyositis
#2
Siamak Moghadam-Kia, Chester V Oddis, Shinji Sato, Masataka Kuwana, Rohit Aggarwal
OBJECTIVE: To determine the clinical features associated with the antimelanoma differentiation-associated gene 5 antibody (anti-MDA5) in US patients with clinically amyopathic dermatomyositis (CADM) and classic DM. METHODS: Patients with CADM were consecutively selected from the University of Pittsburgh Myositis Database from 1985 to 2013. CADM was defined by a typical DM rash without objective muscle weakness and no or minimal abnormalities of muscle enzymes, electromyography, or muscle biopsy...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28089974/cardiopulmonary-disease-development-in-anti-rna-polymerase-iii-positive-systemic-sclerosis-comparative-analyses-from-an-unselected-prospective-patient-cohort
#3
Anna-Maria Hoffmann-Vold, Øyvind Midtvedt, Anders H Tennøe, Torhild Garen, May Brit Lund, Trond M Aaløkken, Arne K Andreassen, Fadi Elhage, Cathrine Brunborg, Eli Taraldsrud, Øyvind Molberg
OBJECTIVE: Extensive skin disease and renal crisis are hallmarks of anti-RNA polymerase III (RNAP)-positive systemic sclerosis (SSc), while lung and heart involvement data are conflicting. Here, the aims were to perform time-course analyses of interstitial lung disease (ILD) and pulmonary hypertension (PH) in the RNAP subset of a prospective unselected SSc cohort and to use the other autoantibody subsets as comparators. METHODS: The study cohort included 279 patients with SSc from the observational Oslo University Hospital cohort with complete data on (1) SSc-related autoantibodies, (2) paired, serial analyses of lung function and fibrosis by computed tomography, and (3) PH verified by right heart catheterization...
January 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28089118/occupational-lung-diseases-from-old-and-novel-exposures-to-effective-preventive-strategies
#4
REVIEW
Paul Cullinan, Xavier Muñoz, Hille Suojalehto, Raymond Agius, Surinder Jindal, Torben Sigsgaard, Anders Blomberg, Denis Charpin, Isabella Annesi-Maesano, Mridu Gulati, Yangho Kim, Arthur L Frank, Metin Akgün, David Fishwick, Rafael E de la Hoz, Subhabrata Moitra
Occupational exposure is an important, global cause of respiratory disease. Unlike many other non-communicable lung diseases, the proximal causes of many occupational lung diseases are well understood and they should be amenable to control with use of established and effective approaches. Therefore, the risks arising from exposure to silica and asbestos are well known, as are the means of their prevention. Although the incidence of occupational lung disease has decreased in many countries, in parts of the world undergoing rapid economic transition and population growth-often with large informal and unregulated workforces-occupational exposures continue to impose a heavy burden of disease...
January 6, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28088797/automated-interpretation-of-pulmonary-function-tests-in-adults-with-respiratory-complaints
#5
Marko Topalovic, Stefan Laval, Jean-Marie Aerts, Thierry Troosters, Marc Decramer, Wim Janssens
BACKGROUND: The use of pulmonary function tests is primarily based on expert opinion and international guidelines. Current interpretation strategies are using predefined cutoffs for the description of a typical pattern. OBJECTIVES: We aimed to explore the predicted disease outcome based on the American Thoracic Society/European Respiratory Society (ATS/ERS) interpreting strategy. Subsequently, we investigated whether an unbiased machine learning framework integrating lung function with clinical variables may provide alternative decision trees resulting in a more accurate diagnosis...
January 12, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28088772/thrombospondin-immune-regulation-and-the-kidney
#6
Claudio Ponticelli, Hans-Joachim Anders
Most therapeutic attempts to prevent the progression of kidney diseases have been based on interventions to inhibit the production of transforming growth factor-β (TGF-β). Thrombospondins (TSPs) play an important role in activating TGF-β. In the healthy kidney, two TSPs are expressed, TSP1 and TSP2, which exert contrasting effects. While TSP1 is a major activator of TGF-β in renal cells and exerts pro-inflammatory effects both in vitro and in vivo, TSP2 lacks the ability for TGF-β activation but regulates matrix remodeling and inflammation in experimental kidney disease...
January 14, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28088638/the-dlno-dlco-ratio-physiological-significance-and-clinical-implications
#7
J M B Hughes, A T Dinh-Xuan
DLNO/DLCO directly measures the ratio of the diffusing capacities of the lung for nitric oxide (NO) and carbon monoxide (CO). In terms of the Roughton and Forster, 1957; equation, 1/DL=1/Dm+1/θVc, where Dm is the membrane (Dm) and θVc is the red cell component of the overall diffusing conductance (DL), DLNO mostly reflects the Dm component and DLCO the θVc red cell component. The DLNO/DLCO ratio is positively related to the DmCO/Vc ratio and the CO red cell resistance (1/θCOVc) as a percentage of the total resistance (1/DLCO), independent of the absolute values of DLNO or DLCO...
January 11, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28088339/evidence-based-management-of-systemic-sclerosis-navigating-recommendations-and-guidelines
#8
REVIEW
Russell Edward Pellar, Janet Elizabeth Pope
OBJECTIVES: Systemic sclerosis (SSc) is a rare heterogeneous connective tissue disease. Recommendations addressing the major issues in the management of SSc including screening and treatment of organ complications are needed. METHODS: The updated European League Against Rheumatism/European Scleroderma Trial and Research (EULAR/EUSTAR) and the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR) guidelines were compared and contrasted...
December 9, 2016: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28087540/international-evidence-based-consensus-diagnostic-criteria-for-hhv-8-negative-idiopathic-multicentric-castleman-disease
#9
David C Fajgenbaum, Thomas S Uldrick, Adam Bagg, Dale Frank, David Wu, Gordan Srkalovic, David Simpson, Amy Y Liu, David Menke, Shanmuganathan Chandrakasan, Mary Jo Lechowicz, Raymond S M Wong, Sheila Pierson, Michele Paessler, Jean-François Rossi, Makoto Ide, Jason Ruth, Michael Croglio, Alexander Suarez, Vera Krymskaya, Amy Chadburn, Gisele Colleoni, Sunita Nasta, Raj Jayanthan, Christopher S Nabel, Corey Casper, Angela Dispenzieri, Alexander Fosså, Dermot Kelleher, Razelle Kurzrock, Peter Voorhees, Ahmet Dogan, Kazuyuki Yoshizaki, Frits van Rhee, Eric Oksenhendler, Elaine S Jaffe, Kojo S J Elenitoba-Johnson, Megan S Lim
Human Herpesvirus-8(HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction due to a cytokine storm often including interleukin-6. iMCD accounts for one-third to one-half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, as no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders...
January 13, 2017: Blood
https://www.readbyqxmd.com/read/28087439/cerebrovascular-heterogeneity-and-neuronal-excitability
#10
REVIEW
Laura Librizzi, Marco de Curtis, Damir Janigro, Leonie Runtz, Frederic deBock, Emmanuel L Barbier, Nicola Marchi
The cerebral vasculature is a complex tridimensional network of arterial and venous vessels which are anatomically in proximity of and functionally coupled to neurons. Depending on the cellular composition of the vascular wall and its anatomical arrangement, cerebral vessels control regional blood flow, define interstitial homeostasis or cerebrospinal fluid circulation and influence immune cell patrolling. Pathological deviations from these functions promote or are a consequence of brain diseases, directly impacting neuronal firing...
January 10, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28087229/disease-associated-mutations-identify-a-novel-region-in-human-sting-necessary-for-the-control-of-type-i-interferon-signaling
#11
Isabelle Melki, Yoann Rose, Carolina Uggenti, Lien Van Eyck, Marie-Louise Frémond, Naoki Kitabayashi, Gillian I Rice, Emma M Jenkinson, Anaïs Boulai, Nadia Jeremiah, Marco Gattorno, Sefano Volpi, Olivero Sacco, Suzanne W J Terheggen-Lagro, Harm A W M Tiddens, Isabelle Meyts, Marie-Anne Morren, Petra De Haes, Carine Wouters, Eric Legius, Anniek Corveleyn, Frederic Rieux-Laucat, Christine Bodemer, Isabelle Callebaut, Mathieu P Rodero, Yanick J Crow
BACKGROUND: Gain-of-function mutations in transmembrane protein 173 (TMEM173) encoding stimulator of interferon genes (STING) underlie a recently described type I interferonopathy called STING-associated vasculopathy with onset in infancy (SAVI). OBJECTIVES: We sought to define the molecular and cellular pathology relating to 3 individuals variably exhibiting the core features of the SAVI phenotype including systemic inflammation, destructive skin lesions, and interstitial lung disease...
January 3, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28087137/nos-interacting-protein-nosip-is-increased-in-the-colon-of-patients-with-hirschsprung-s-disease
#12
Anne-Marie O'Donnell, David Coyle, Prem Puri
PURPOSE: Voltage-dependent K(+) channels (Kv channels) participate in electrical rhythmicity and smooth muscle responses and are regulated by excitatory and inhibitory neurotransmitters. Kv channels also participate in the interstitial cell of Cajal (ICC) and smooth muscle cell (SMC) responses to neural inputs. The Kv family consists of 12 subfamilies, Kv1-Kv12, with five members of the Kv7 family identified to date: Kv7.1-Kv7.5. A recent study identified the potassium channel Kv7.5 as having a role in the excitability of ICC-IM in the mouse colon...
January 3, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28087004/gimme-a-complex-resident-mononuclear-phagocytes-in-the-kidney-as-monitors-of-circulating-antigens-and-immune-complexes
#13
Wulf Tonnus, Christian Hugo, Andreas Linkermann
Over the past decade, researchers have made substantial progress in characterizing a network of mononuclear phagocytes in the kidney, which variously have been referred to as resident macrophages or dendritic cells. Two recent studies, published in Cell and Kidney International, have identified these resident macrophages/dendritic cells as local immune monitors of peritubular capillaries for circulating antigens and immune complexes. These cells appear to represent an early line of defense against circulating infectious particles and immune complexes, but the resulting inflammatory response may also contribute to interstitial inflammation and kidney disease progression...
February 2017: Kidney International
https://www.readbyqxmd.com/read/28086951/sodium-glucose-transporter-2-sglt2-inhibition-with-empagliflozin-improves-cardiac-diastolic-function-in-a-female-rodent-model-of-diabetes
#14
Javad Habibi, Annayya R Aroor, James R Sowers, Guanghong Jia, Melvin R Hayden, Mona Garro, Brady Barron, Eric Mayoux, R Scott Rector, Adam Whaley-Connell, Vincent G DeMarco
Obese and diabetic individuals are at increased risk for impairments in diastolic relaxation and heart failure with preserved ejection fraction. The impairments in diastolic relaxation are especially pronounced in obese and diabetic women and predict future cardiovascular disease (CVD) events in this population. Recent clinical data suggest sodium glucose transporter-2 (SGLT2) inhibition reduces CVD events in diabetic individuals, but the mechanisms of this CVD protection are unknown. To determine whether targeting SGLT2 improves diastolic relaxation, we utilized empagliflozin (EMPA) in female db/db mice...
January 13, 2017: Cardiovascular Diabetology
https://www.readbyqxmd.com/read/28086844/are-risk-predicting-models-useful-for-estimating-survival-of-patients-with-rheumatoid-arthritis-associated-interstitial-lung-disease
#15
Hanna M Nurmi, Minna K Purokivi, Miia S Kärkkäinen, Hannu-Pekka Kettunen, Tuomas A Selander, Riitta L Kaarteenaho
BACKGROUND: Risk predicting models have been applied in idiopathic pulmonary fibrosis (IPF), but still not validated in patients with rheumatoid arthritis-associated interstitial lung disease (RA-ILD). The purpose of this study was to test the suitability of three prediction models as well as individual lung function and demographic factors for evaluating the prognosis of RA-ILD patients. METHODS: Clinical and radiological data of 59 RA-ILD patients was re-assessed...
January 13, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28081341/association-of-neutrophil-lymphocyte-ratio-with-the-degree-ofinterstitial-lung-disease-in-systemic-sclerosis
#16
Nurhan Atilla, Gözde Yıldırım Çetin, Ayşe Balkarlı
BACKGROUND/AIM: Determining the severity of systemic sclerosis related interstitial lung disease (SSc-ILD) is based on clinical and radiological findings, inflammation marker levels, and carbon monoxide diffusing capacity of the lung (DLCO). Recently studies are ongoing for objective and easy markers. Neutrophil/lymphocyte ratio (NLR) is shown to be a good marker for inflammation in recent clinical trials. In this study, we aimed to identify the possible relationship between NLR and carbon monoxide transfer coefficient (KCO) of SSc-ILD patients...
December 20, 2016: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28079846/patient-confidence-and-quality-of-life-in-idiopathic-pulmonary-fibrosis-and-sarcoidosis
#17
Jalpa Kotecha, Christopher Atkins, Andrew Wilson
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) and sarcoidosis impact significantly on health-related quality of life (HRQOL). There are few studies on the impact of patient confidence on HRQOL in these conditions. OBJECTIVES: 1. To investigate whether patient confidence is associated with HRQOL, anxiety, depression, dyspnoea or fatigue. 2. To assess if patient confidence is associated with inpatient admissions, access to community healthcare and, for IPF patients, mortality and disease severity...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079842/increased-erythrocyte-aggregation-and-oxidative-stress-in-patients-with-idiopathic-interstitial-pneumonia
#18
Erhan Ugurlu, Emine Kilic-Toprak, Goksel Altinisik, Ozgen Kilic-Erkek, Betul Cengiz, Vural Kucukatay, Hande Senol, Ismail Hakki Akbudak, Yusuf Ekbic, Melek Bor-Kucukatay
BACKGROUND: Hemorheological properties are important determinants of tissue oxygenation. Although hemorheological alterations in various lung diseases have been well-defined, no information is available about the effects of idiopathic interstitial pneumonia (IIP) on hemorheological parameters. OBJECTIVES: The aim of this study was to investigate hemorheological parameters (erythrocyte deformability, aggregation, and plasma viscosity -PV) and associated oxidative stress indices in patients with IIP...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079811/routine-application-of-lung-ultrasonography-in-the-neonatal-intensive-care-unit
#19
Shui-Wen Chen, Wei Fu, Jing Liu, Yan Wang
The aim of this study was to study the features of lung ultrasonography (LUS) in lung disease and to evaluate the usefulness of LUS in the neonatal intensive care unit (NICU).All of 3405 neonates included in this study underwent an LUS examination. Diagnoses were based on medical history, clinical manifestation, laboratory examination, and signs on chest radiography (CR) and/or computed tomography (CT). A single expert physician performed all LUS examinations.There were 2658 cases (78.9%) with lung disease and 747 cases (21...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28079145/galectin-3-as-a-marker-of-interstitial-atrial-remodelling-involved-in-atrial-fibrillation
#20
Diana Hernández-Romero, Juan Antonio Vílchez, Álvaro Lahoz, Ana I Romero-Aniorte, Eva Jover, Arcadio García-Alberola, Rubén Jara-Rubio, Carlos M Martínez, Mariano Valdés, Francisco Marín
Remodelling in the atria could appear as a result of hypertension, diabetes or ischaemic heart disease. Galectin-3 (Gal-3) is a mediator of profibrotic pathways and a potential biomarker of cardiac remodelling. We prospectively recruited consecutive patients undergoing elective cardiac surgery. Preoperative Gal-3 levels were determined from serum samples, and the presence of fibrosis was assessed from atrial appendage tissue samples obtained during cardiac surgery. We included 100 patients with aortic valve or ischaemic heart diseases and 15 controls with permanent AF...
January 12, 2017: Scientific Reports
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