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https://www.readbyqxmd.com/read/29457372/clinical-characteristics-and-factors-associated-with-disability-and-impaired-quality-of-life-in-children-with-juvenile-systemic-sclerosis
#1
Brandi E Stevens, Kathryn S Torok, Suzanne C Li, Nicole Hershey, Megan Curran, Gloria C Higgins, Katharine F Moore, C Egla Rabinovich, Samuel Dodson, Anne M Stevens
OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics...
February 18, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29457208/children-deserve-smoke-free-world
#2
REVIEW
R Remesh Kumar, P R Jayakumar, R Krishna Mohan
Tobacco smoke, active or passive exposure was the major cause of preventable morbidity and mortality in the world during twentieth century and will continue to be the same in the twenty-first century also if the current trends continue. Both active and passive smoking are having significance in relation to child health. Exposure starts antenatally from placenta to the fetus and later phases through passive exposure to experimental and regular smoking and ultimately addiction and habitual smoking. Evidences are in favour of causal relationship with intrauterine growth restriction, sudden infant death syndrome, decreased pulmonary function, increased risk for respiratory tract infection, otitis media, wheeze, asthma, neurobehavioral disorders, cleft palate and triggering pathogenesis of fetal and childhood onset of adult diseases, especially pulmonary and cardio vascular diseases...
February 19, 2018: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/29445936/pulmonary-arterial-hemodynamic-assessment-by-a-novel-index-in-systemic-sclerosis-patients-pulmonary-pulse-transit-time
#3
Mehmet Dogan, Tolga Han Efe, Tolga Cimen, Cem Ozisler, Mehmet Ali Felekoglu, Ahmet Goktug Ertem, Mehmet Erat, Omer Yiginer, Murat Tulmac
OBJECTIVES: Systemic sclerosis (SSc) is a chronic, inflammatory, and autoimmune connective tissue disease that is associated with vascular lesions, and fibrosis of the skin and visceral organs. Cardiac complications may occur as a secondary effect of SSc as a result of pulmonary arterial hypertension and interstitial lung disease. The objective of this study was to assess whether the pulmonary pulse transit time (pPTT) could serve as a diagnostic marker for pulmonary arterial alterations in patients with SSc, prior to development of pulmonary hypertension...
February 14, 2018: Lung
https://www.readbyqxmd.com/read/29444773/lung-function-inflammation-and-endothelin-1-in-congenital-heart-disease-associated-pulmonary-arterial-hypertension
#4
Andrew Low, Sarah George, Luke Howard, Nicholas Bell, Ann Millar, Robert M R Tulloh
BACKGROUND: Breathlessness is the most common symptom in people with pulmonary arterial hypertension and congenital heart disease (CHD-APAH), previously thought to be caused by worsening PAH, but perhaps also by inflammation and abnormalities of lung function. We studied lung function and airway inflammation in patients with CHD-APAH and compared the results with controls. METHODS AND RESULTS: Sixty people were recruited into the study: 20 CHD-APAH, 20 CHD controls, and 20 healthy controls...
February 14, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29436381/subcutaneous-treprostinil-in-congenital-heart-disease-related-pulmonary-arterial-hypertension
#5
Nika Skoro-Sajer, Christian Gerges, Olga Hajnalka Balint, Dora Kohalmi, Monika Kaldararova, Iveta Simkova, Johannes Jakowitsch, Harald Gabriel, Helmut Baumgartner, Mario Gerges, Roela Sadushi-Kolici, David S Celermajer, Irene Marthe Lang
OBJECTIVE: To assess the efficacy and safety of subcutaneous treprostinil in adult patients with congenital heart disease (CHD)-associated pulmonary arterial hypertension (PAH) after 12 months of treatment. METHODS: Consecutive adult patients with CHD-PAH received subcutaneous treprostinil to maximum tolerated doses in an observational study. RESULTS: Advanced CHD-PAH patients with WHO class III or IV disease (n=32, age 40±10 years, 20 females) received treprostinil for suboptimal response to bosentan (n=12), WHO functional class IV disease (FC, n=7) or prior to bosentan approval (n=13)...
February 7, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29435748/surgery-for-benign-esophageal-disorders-in-the-us-risk-factors-for-complications-and-trends-of-morbidity
#6
Francisco Schlottmann, Paula D Strassle, Marco G Patti
BACKGROUND: Gastroesophageal reflux disease (GERD), paraesophageal hernia (PEH), and achalasia are the most frequent benign esophageal disorders that may need surgical treatment. We aimed to identify risk factors for postoperative complications and to characterize trends of morbidity for surgery for benign esophageal disorders in a national cohort. METHODS: A retrospective population-based analysis was performed using the National Inpatient Sample for the period 2000-2013...
February 12, 2018: Surgical Endoscopy
https://www.readbyqxmd.com/read/29435739/multiparametric-magnetic-resonance-imaging-in-the-assessment-of-pulmonary-hypertension-initial-experience-of-a-one-stop-study
#7
Gisela M B Meyer, Fernanda B Spilimbergo, Stephan Atmayer, Gabriel S Pacini, Matheus Zanon, Guilherme Watte, Edson Marchiori, Bruno Hochhegger
INTRODUCTION: Our goal was to assess the diagnostic performance of magnetic resonance imaging (MRI) as a single method to diagnose pulmonary hypertension (PH) compared to right heart catheterization (RHC), computed tomography (CT), and ventilation/perfusion (V/Q) scintigraphy. METHODS: We identified 35 patients diagnosed with PH by RHC in our institution who have also undergone a CT, a scintigraphy, and an MRI within a month. All cases were discussed in multidisciplinary meetings...
February 12, 2018: Lung
https://www.readbyqxmd.com/read/29432661/is-pulmonary-vascular-disease-reversible-with-ppar-%C3%A9-agonists
#8
Peter Afdal, Antoine F AbdelMassih
Severe angio-obliterative pulmonary vascular disease (PAH) remains a disease characterized by great morbidity and shortened survival. Unfortunately, the only currently available treatments for angio-obliterative changes are palliative in the form of pulmonary vasodilators evolving from the phosphodiesterase inhibitor sildenafil to endothelin receptor antagonist: Bosentan; while the only definitive treatment is lung transplantation which remains dependent on the availability of donors and the transplant policies which vary widely from a country to another...
February 12, 2018: Microcirculation: the Official Journal of the Microcirculatory Society, Inc
https://www.readbyqxmd.com/read/29431689/dysregulated-th1-immune-and-vascular-responses-in-scrub-typhus-pathogenesis
#9
REVIEW
Lynn Soong
Scrub typhus is an emerging, insect-transmitted disease caused by Orientia tsutsugamushi, a Gram- and LPS-negative bacterium that replicates freely within professional phagocytes and endothelial cells. Scrub typhus is prevalent with high mortality rates, but information regarding its molecular pathogenesis, microbial virulence determinants, and key immune responses is limited. Improved animal models have recently been developed that respectively resemble the pathological features of self-limiting or severe scrub typhus in humans...
February 15, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29431643/epigenetic-dysregulation-of-the-drp1-binding-partners-mid49-and-mid51-increases-mitotic-mitochondrial-fission-and-promotes-pulmonary-arterial-hypertension-mechanistic-and-therapeutic-implications
#10
Kuang-Hueih Chen, Asish Dasgupta, Jianhui Lin, Francois Potus, Sebastien Bonnet, James Iremonger, Jennifer Fu, Jeffrey Mewburn, Danchen Wu, Kimberly Dunham-Snary, Anne L Theilmann, Zhi-Cheng Jing, Charles Hindmarch, Mark L Ormiston, Allan Lawrie, Stephen L Archer
Background -Mitotic fission is increased in pulmonary arterial hypertension (PAH), a hyperproliferative, apoptosis-resistant disease. The fission mediator, dynamin related protein 1 (Drp1) must complex with adaptor proteins to cause fission. Drp1-induced fission has been therapeutically targeted in experimental PAH. Here we examine the role of two recently discovered, poorly understood, Drp1 adapter proteins, mitochondrial dynamics protein of 49 and 51 kDa (MiD49 and MiD51) in normal vascular cells and explore their dysregulation in PAH...
February 5, 2018: Circulation
https://www.readbyqxmd.com/read/29431238/basigin-expression-as-a-prognostic-indicator-in-stage-i-pulmonary-adenocarcinoma
#11
Toshihide Matsumoto, Ryo Nagashio, Shinichiro Ryuge, Satoshi Igawa, Makoto Kobayashi, Eriko Fukuda, Naoki Goshima, Masaaki Ichinoe, Shi-Xu Jiang, Yukitoshi Satoh, Noriyuki Masuda, Yoshiki Murakumo, Makoto Saegusa, Yuichi Sato
We established the KU-Lu-8 monoclonal antibody (MoAb) using a lung cancer cell line as an antigen and a random immunization method. The KU-Lu-8 MoAb recognizes basigin (BSG), which is a transmembrane-type glycoprotein that is strongly expressed on the cell membranes of lung cancer cells. This study aimed to clarify the relationships between BSG expression and clinicopathological parameters and determine the prognostic significance of BSG expression in pulmonary adenocarcinoma (AC) patients. To evaluate the significance of BSG expression in lung cancer, we immunohistochemically analyzed 113 surgically resected pulmonary adenocarcinomas, and the associations between BSG expression and various clinicopathological parameters were evaluated...
February 12, 2018: Pathology International
https://www.readbyqxmd.com/read/29428622/targeted-increase-in-pulmonary-blood-flow-in-a-bidirectional-glenn-circulation
#12
Samuel L Casella, Aditya Kaza, Pedro Del Nido, James E Lock, Audrey Marshall
Objective In patients with a Glenn shunt and unilateral pulmonary vascular abnormalities, supplemental pulmonary blood flow may improve antegrade flow in the target lung and may permit eventual Fontan completion, while mitigating the risks of uncontrolled additional flow to the contralateral lung. We attempted this by inserting a small aortopulmonary shunt into the affected pulmonary artery and banding the central pulmonary artery. .Methods We performed a retrospective review of all patients who underwent this procedure from 2000-2015...
February 8, 2018: Seminars in Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29428236/liver-fibrosis-marker-7s-domain-of-collagen-type-iv-in-patients-with-pre-capillary-pulmonary-hypertension
#13
Akiomi Yoshihisa, Yusuke Kimishima, Takatoyo Kiko, Yu Sato, Shunsuke Watanabe, Yuki Kanno, Satoshi Abe, Makiko Miyata-Tatsumi, Takamasa Sato, Satoshi Suzuki, Masayoshi Oikawa, Atsushi Kobayashi, Takayoshi Yamaki, Koichi Sugimoto, Hiroyuki Kunii, Kazuhiko Nakazato, Hitoshi Suzuki, Takafumi Ishida, Yasuchika Takeishi
BACKGROUND: Pulmonary hypertension (PH) causes right ventricular dysfunction and central venous congestion, and may lead to congestive hepatopathy. The serum 7S domain of collagen type IV (P4NP 7S) is an established marker of liver fibrosis in chronic liver disease. We aimed to determine whether P4NP 7S is related to hemodynamic parameters, and assessed the potential values of P4NP 7S to predict mortality. METHODS: Consecutive 76 pre-capillary PH patients were divided into tertiles based on their serum P4NP 7S levels...
February 7, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29426959/acute-effect-of-inhaled-iloprost-in-children-with-pulmonary-arterial-hypertension-associated-with-simple-congenital-heart-defects
#14
Qiangqiang Li, Konstantinos Dimopoulos, Chen Zhang, Yan Zhu, Qian Liu, Hong Gu
Inhaled prostacyclin analogue iloprost is currently utilized in adult patients with pulmonary arterial hypertension (PAH), but little information is available on its use in the pediatric population. This study evaluated the safety and acute haemodynamic effects of inhaled iloprost in children with PAH associated with congenital heart disease (CHD). Children with PAH-CHD who underwent cardiac catheterization and iloprost administration in our catheter laboratory between June 2007 and October 2015 were included...
February 9, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29422852/recurrent-stroke-in-a-young-woman-with-a-single-pulmonary-arteriovenous-fistula-an-unusual-association
#15
Sandra Sousa, Nuno Vasco Costa, Cátia Carmona, Élia Coimbra, Fernando Pita
Introduction: Cryptogenic stroke is present in about 40% of ischemic stroke patients. Extracardiac shunt related to pulmonary arteriovenous fistula (PAVF) could be a rare potential risk factor for embolic stroke. Most PAVFs are multiple, congenital, and associated with hereditary conditions. On the other hand, isolated PAVFs are rare conditions and an uncommon cause of cryptogenic stoke. Case Report: We describe a case of a young woman without history of respiratory diseases or vascular risk factors, who presented with acute onset of transitory aphasia and right hemiplegia...
September 2017: Case Reports in Neurology
https://www.readbyqxmd.com/read/29422818/circulating-angiostatin-serum-level-in-patients-with-systemic-sclerosis
#16
Zofia Gerlicz-Kowalczuk, Elzbieta Dziankowska-Zaborszczyk, Bożena Dziankowska-Bartkowiak
Introduction: Systemic sclerosis (SSc) is achronic connective tissue disease characterized by microangiopathy with inadequate angiogenesis. Angiostatin (AS) is a potent antiangiogenic factor specifically inhibiting proliferation and inducing apoptosis of vascular endothelial cells. Aim: To evaluate the level of angiostatin in the serum of patients with SSc. Material and methods: Serum levels of AS were measured in 20 SSc patients and 12 healthy controls...
December 2017: Postȩpy Dermatologii i Alergologii
https://www.readbyqxmd.com/read/29422044/endothelial-cdc42-deficiency-impairs-endothelial-regeneration-and-vascular-repair-after-inflammatory-vascular-injury
#17
Jiawen Lv, Junchao Zeng, Fukun Guo, Yiran Li, Mengying Xu, Yuanxiong Cheng, Lin Zhang, Shaoxi Cai, Yinghua Chen, Yi Zheng, Guodong Hu
BACKGROUND: Endothelial cell (EC) regeneration is essential for inflammation resolution and vascular integrity recovery after inflammatory vascular injury. Cdc42 is a central regulator of cell survival and vessel formation in EC development. However, it is unknown that whether Cdc42 could be a regulating role of EC repair following the inflammatory injury in the lung. The study sought to test the hypothesis that Cdc42 is required for endothelial regeneration and vascular integrity recovery after LPS-induced inflammatory injury...
February 8, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29421689/diabetes-mellitus-associates-with-increased-right-ventricular-afterload-and-remodeling-in-pulmonary-arterial-hypertension
#18
Morgan E Whitaker, Vineet Nair, Shripad Sinari, Parinita Dherange, Balaji Natarajan, Lindsey Trutter, Evan L Brittain, Anna R Hemnes, Eric Austin, Kumar Patel, Stephen M Black, Joe G N Garcia, Jason X Yuan, Rebecca Vanderpool, Franz Rischard, Ayako Makino, Edward J Bedrick, Ankit A Desai
BACKGROUND: Diabetes mellitus is associated with left ventricular hypertrophy and dysfunction. Parallel studies have also reported associations between diabetes mellitus and right ventricle dysfunction and reduced survival in patients with pulmonary arterial hypertension. However, the impact of diabetes mellitus on the pulmonary vasculature has not been well-characterized. We hypothesized that diabetes mellitus and hyperglycemia could specifically influence right ventricular afterload and remodeling in patients with Group I pulmonary arterial hypertension, providing a link to their known susceptibility to right ventricular dysfunction...
February 5, 2018: American Journal of Medicine
https://www.readbyqxmd.com/read/29417467/pulmonary-vascular-disease-hemodynamic-assessment-and-treatment-selection-focus-on-group-ii-pulmonary-hypertension
#19
REVIEW
Bhavadharini Ramu, Brian A Houston, Ryan J Tedford
PURPOSE OF REVIEW: Pulmonary hypertension due to left heart disease (PH-LHD) is the most common cause of pulmonary hypertension worldwide, yet therapies used to treat pulmonary arterial hypertension have failed to show efficacy in this population. Proper hemodynamic assessment and differentiation of pulmonary hypertension phenotypes is therefore critical for both current clinical practice and future research and therapeutic efforts. RECENT FINDINGS: Substantial recent efforts have sought to improve the hemodynamic characterization of pulmonary hypertension for both diagnostic and prognostic purposes...
February 7, 2018: Current Heart Failure Reports
https://www.readbyqxmd.com/read/29416329/sex-specific-cardiopulmonary-exercise-testing-indices-to-estimate-the-severity-of-inoperable-chronic-thromboembolic-pulmonary-hypertension
#20
Tian-Xiang Chen, Bigyan Pudasaini, Jian Guo, Su-Gang Gong, Rong Jiang, Lan Wang, Qin-Hua Zhao, Wen-Hui Wu, Ping Yuan, Jin-Ming Liu
Background: Sex differences in chronic thromboembolic pulmonary hypertension (CTEPH) have been revealed in few studies. Although right heart catheterization (RHC) is the gold standard for clinical diagnosis and assessment of prognosis in pulmonary hypertension (PH), cardiopulmonary exercise testing (CPET) has been a more widely used assessment of functional capacity, disease severity, prognosis, and treatment response in PH. We hypothesized that the "sex-specific" CPET indices could estimate the severity of inoperable CTEPH...
2018: International Journal of Chronic Obstructive Pulmonary Disease
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