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Pulmonary hypertension diagnosis and treatment

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https://www.readbyqxmd.com/read/28642988/development-of-pulmonary-hypertension-during-treatment-with-diazoxide-a-case-series-and-literature-review
#1
Matthew R Timlin, Alexander B Black, Heather M Delaney, Renée I Matos, Candace S Percival
Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in infancy. The mainstay of medical management for CHI is diazoxide. Diazoxide inhibits insulin release from the pancreas, but also causes smooth muscle relaxation and fluid retention so it is typically given with chlorothiazide. In July 2015, the FDA issued a drug safety communication warning that pulmonary hypertension (PH) had been reported in 11 infants being treated with diazoxide and that the PH resolved with withdrawal of diazoxide...
June 22, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28641568/an-expanding-role-of-biomarkers-in-pulmonary-arterial-hypertension
#2
Mustafa Yildiz, Alparslan Sahin, Michael Behnes, İbrahim Akin
BACKGROUND: Pulmonary arterial hypertension (PAH), is a chronic disease which may cause or result from mulitple cardiopulmonary disorders. The disease has complex pathophysiological mechanisms and involves many systematic, cellular and molecular changes. Therefore, it is crutial to find out underlying mechanisms and detect biomarkers to achieve early and proper diagnosis, evaluate of disease severity, for follow-up and monitor response to treatment. Many biomarker for PAH have been investigated but yet no such biomarker has been found specific and easily accessible to use for the patients...
June 14, 2017: Current Pharmaceutical Biotechnology
https://www.readbyqxmd.com/read/28634363/long-term-health-outcomes-in-patients-with-prader-willi-syndrome-a-nationwide-cohort-study-in-denmark
#3
E Hedgeman, S P Ulrichsen, S Carter, N C Kreher, K P Malobisky, M M Braun, J Fryzek, M S Olsen
BACKGROUND: Prader-Willi syndrome (PWS) is a rare congenital disease that affects growth, sexual development, cognitive function and behavior. Individuals exhibit food preoccupation and hyperphagia, which may lead to obesity with premature morbidity and mortality. The aim of this work was to evaluate the risk of venous thromboembolisms (VTE), myocardial infarction, pulmonary hypertension, sleep apnea, depression, anxiety and all-cause mortality among persons with PWS as compared to an age- and sex-matched general population cohort...
June 21, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28628189/-a-statement-the-polish-cardiac-society-working-group-on-pulmonary-circulation-on-screening-for-cteph-patients-after-acute-pulmonary-embolism
#4
Michał Ciurzyński, Marcin Kurzyna, Grzegorz Kopeć, Piotr Błaszczak, Łukasz Chrzanowski, Karol Kamiński, Katarzyna Mizia-Stec, Tatiana Mularek-Kubzdela, Ewa Mroczek, Andrzej Biederman, Piotr Pruszczyk, Adam Torbicki
Both pharmacological and invasive treatment of chronic thromboembolic pulmonary hypertension (CTEPH) is now available in Poland and the awareness of the disease among physicians is growing. Thus, the Polish Cardiac Society's Working Group on Pulmonary Circulation in cooperation with independent experts in this field, have launched the statement on algorithm to guide a CTEPH diagnosis in patients with previous acute pulmonary embolism (APE). In Poland, every year this disease affects about 250 patients. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months period of effective anticoagulation, particularly when specified risk factors are present...
2017: Kardiologia Polska
https://www.readbyqxmd.com/read/28626631/chronic-eosinophilic-pneumonia-adjunctive-therapy-with-inhaled-steroids
#5
Christopher Chan, David DeLapp, Perry Nystrom
Idiopathic chronic eosinophilic pneumonia (ICEP) is a rare form of diffuse parenchymal lung disease first identified by Carrington et al. in 1969. It is characterized by the presence of constitutional and respiratory symptoms with associated peripheral opacities on imaging and elevated serum and/or bronchoalveolar eosinophilia. Although data is limited regarding etiology or prevalence, it is known that ICEP has a 2:1 female: male predominance and typically affects non-smokers. Diagnosis rests on the clinical constellation of respiratory symptoms of at least 2-4 weeks duration, the presence of diffuse pulmonary alveolar consolidation, classically described as the "photographic negative of pulmonary edema", the presence of eosinophils ≥40% on bronchoalveolar lavage or ≥1000/mm(3) eosinophils on peripheral blood and the exclusion of other known causes of eosinophilic lung diseases such as drugs, toxins, fungi, parasites, and collagen-vascular disorders...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28606232/-a-clinical-analysis-of-15-children-with-systemic-lupus-erythematosus-accompanied-by-pulmonary-hypertension
#6
Ji Li, Jing-Ran Ma, Zhi-Xing Sun, Jing-Jing Jiang, Yan-Qing Dong, Qian Wang, Hong-Mei Song
OBJECTIVE: To evaluate the clinical features, laboratory findings, diagnosis and treatment, and prognosis of children with systemic lupus erythematosus (SLE) accompanied by pulmonary hypertension (PH). METHODS: The clinical symptoms, laboratory findings, echocardiographic features, SLE disease activity index, and treatment outcome of 15 hospitalized children with SLE accompanied by PH were retrospectively analyzed. RESULTS: Among the 15 patients, the median interval from diagnosis of SLE to diagnosis of PH was 0...
June 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28597780/novel-approach-to-classifying-patients-with-pulmonary-arterial-hypertension-using-cluster-analysis
#7
Kishan S Parikh, Youlan Rao, Tariq Ahmad, Kai Shen, G Michael Felker, Sudarshan Rajagopal
Pulmonary arterial hypertension (PAH) patients have distinct disease courses and responses to treatment, but current diagnostic and treatment schemes provide limited insight. We aimed to see if cluster analysis could distinguish clinical phenotypes in PAH. An unbiased cluster analysis was performed on 17 baseline clinical variables of PAH patients from the FREEDOM-M, FREEDOM-C, and FREEDOM-C2 randomized trials of oral treprostinil versus placebo. Participants were either treatment-naïve (FREEDOM-M) or on background therapy (FREEDOM-C, FREEDOM-C2)...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28597760/late-onset-pulmonary-arterial-hypertension-in-repaired-d-transposition-of-great-arteries-an-uncommon-complication
#8
Janani Baskaran, Kimara S March, Thenappan Thenappan
Late-onset pulmonary arterial hypertension (PAH) is a rare but fatal complication in patients with childhood surgical repair of dextro-transposition of great arteries (D-TGA), especially with the Mustard and Senning procedures. The pathogenic mechanisms of PAH in patients with repaired D-TGA are not well understood and treatment is not standardized. In this manuscript, we present a case of late-onset PAH in an adult D-TGA patient after Mustard repair and discuss the pathogenic mechanisms, diagnosis, and treatment of pulmonary hypertension in repaired D-TGA...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28594706/diagnosis-of-tuberous-sclerosis-complex-in-a-patient-referred-for-uncontrolled-hypertension-and-renal-dysfunction-a-case-highlighting-the-importance-of-proper-diagnostic-work-up-of-hypertensive-patients
#9
Pantelis A Sarafidis, Athanasios Bikos, Charalampos Loutradis, Stergios Tzikas, Efstratios Vakirlis, Eugenia Avdelidou, Dimitrios Zafeiriou, Aikaterini Papagianni, Vasileios Vassilikos
: We report a case of a 39-year-old woman with resistant hypertension and renal dysfunction. The patient was hospitalized 3 months earlier for dyspnea at the Department of Cardiology, where she was diagnosed with heart failure (left ventricle injection fraction: 25-30%), pulmonary hypertension, chronic kidney disease (serum creatinine: 1.58 mg/dl), and resistant hypertension and discharged with optimal heart failure treatment. At presentation to our clinic, apart from uncontrolled hypertension for more than 10 years and history of pre-eclampsia and fetal loss, the patient had obesity (BMI: 38 kg/m) and facial fibromas...
June 7, 2017: Journal of Hypertension
https://www.readbyqxmd.com/read/28593996/epidemiology-and-treatment-of-pulmonary-arterial-hypertension
#10
REVIEW
Edmund M T Lau, Eleni Giannoulatou, David S Celermajer, Marc Humbert
In the past 2 decades, major changes have occurred in the epidemiological and treatment landscape of pulmonary arterial hypertension (PAH). Previously regarded as a disease of the young and middle-aged, contemporary registries from the Western world have demonstrated an increase in the age of patients with PAH, many of whom are elderly with multiple comorbidities. Another important observation is the improvement in survival of patients with PAH in the modern treatment era compared with historical cohorts, before the availability of advanced therapy...
June 8, 2017: Nature Reviews. Cardiology
https://www.readbyqxmd.com/read/28590293/pediatric-pulmonary-arterial-hypertension-on-the-eve-of-growing-up
#11
Johannes M Douwes, Rolf M F Berger
PURPOSE OF REVIEW: Current recommendations for diagnosis and treatment of pulmonary arterial hypertension (PAH) during childhood are expert opinion based, because of lacking pediatric data. In recent years, however, important pediatric data have emerged on PAH. RECENT FINDINGS: PAH in children shows similarities as well as differences compared to adults. Neonates and children know specific clinical presentations and a hemodynamic profile that differs from adults with PAH...
June 5, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28584899/development-of-aerosol-phospholipid-microparticles-for-the-treatment-of-pulmonary-hypertension
#12
Sarah Brousseau, Zimeng Wang, Sweta K Gupta, Samantha A Meenach
Pulmonary arterial hypertension (PAH) is an incurable cardiovascular disease characterized by high blood pressure in the arteries leading from the heart to the lungs. Over two million people in the USA are diagnosed with PAH annually and the typical survival rate is only 3 years after diagnosis. Current treatments are insufficient because of limited bioavailability, toxicity, and costs associated with approved therapeutics. Aerosol delivery of drugs is an attractive approach to treat respiratory diseases because it increases localized drug concentration while reducing systemic side effects...
June 5, 2017: AAPS PharmSciTech
https://www.readbyqxmd.com/read/28575277/a-comprehensive-risk-stratification-at-early-follow-up-determines-prognosis-in-pulmonary-arterial-hypertension
#13
David Kylhammar, Barbro Kjellström, Clara Hjalmarsson, Kjell Jansson, Magnus Nisell, Stefan Söderberg, Gerhard Wikström, Göran Rådegran
Aims: Guidelines recommend a goal-oriented treatment approach in pulmonary arterial hypertension (PAH). The aim is to reach a low-risk profile, as determined by a risk assessment instrument. This strategy is incompletely validated. We aimed to investigate the bearing of such risk assessment and the benefit of reaching a low-risk profile. Methods and results: Five hundred and thirty PAH patients were included. Follow-up assessments performed after a median of 4 (interquartile range 3-5) months were available for 383 subjects...
June 1, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28546762/cardiovascular-magnetic-resonance-imaging-clinical-implications-in-the-evaluation-of-connective-tissue-diseases
#14
REVIEW
Sophie Mavrogeni, George Markousis-Mavrogenis, Loukia Koutsogeorgopoulou, Genovefa Kolovou
Cardiovascular magnetic resonance imaging is a recently developed noninvasive, nonradiating, operator-independent technique that has been successfully used for the evaluation of congenital heart disease, valvular and pericardial diseases, iron overload, cardiomyopathies, great and coronary vessel diseases, cardiac inflammation, stress-rest myocardial perfusion, and fibrosis. Rheumatoid arthritis and other spondyloarthropathies, systemic lupus erythematosus, inflammatory myopathies, mixed connective tissue diseases (CTDs), systemic sclerosis, vasculitis, and sarcoidosis are among CTDs with serious cardiovascular involvement; this is due to multiple causative factors such as myopericarditis, micro/macrovascular disease, coronary artery disease, myocardial fibrosis, pulmonary hypertension, and finally heart failure...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28545542/recognition-and-treatment-of-sleep-disordered-breathing-an-important-component-of-chronic-disease-management
#15
REVIEW
Peter C Farrell, Glenn Richards
Sleep-disordered breathing (SDB) is a highly prevalent condition, and is associated with many debilitating chronic diseases. The role of untreated obstructive sleep apnea (OSA) in arterial hypertension has been recognized in international guidelines. Treatment with continuous positive airway pressure (CPAP) is associated with clinically-relevant reductions in blood pressure. In heart failure (HF), SDB is associated with worse prognosis and increased mortality. Major HF guidelines recommend that patients should be treated for sleep apnea to improve their HF status...
May 25, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28531365/a-rare-case-of-primary-ovarian-carcinoid
#16
Karan Saraf, Efterpi Tingi, Adrian Brodison, Sanjay Sinha
We present a rare case in an elderly Caucasian lady of primary ovarian carcinoid tumor, causing symptoms of the carcinoid syndrome and severe carcinoid heart disease. Carcinoid tumors are the commonest neuroendocrine tumors, the majority of which are found in the gastrointestinal tract and lungs. Ovarian carcinoids represent only 0.1% of all ovarian neoplasms and 1% of all carcinoid tumors. Carcinoid syndrome can present with flushing, diffuse watery diarrhea, wheezing and breathlessness and in advanced cases lead to carcinoid heart disease...
May 20, 2017: Gynecological Endocrinology
https://www.readbyqxmd.com/read/28528258/epidemiology-and-management-of-chronic-thromboembolic-pulmonary-hypertension-experience-from-two-expert-centers
#17
Maria Anna Bazmpani, Alexandra Arvanitaki, Maria Toumpourleka, Georgia Pitsiou, Evaggelia Panagiotidou, Sophia Anastasia Mouratoglou, Georgios Sianos, Stavros Hadjimiltiades, Antonios Pitsis, Eckhard Mayer, Ioannis Stanopoulos, Haralambos Karvounis, George Giannakoulas
BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, distinct pulmonary vascular disease, and, therefore, there is lack of data regarding its clinical presentation, diagnosis and management at a national basis. Our objective was to describe the demographics and management of CTEPH patients in Northern Greece. METHODS: We conducted a retrospective, observational study with a joint collaboration from two pulmonary hypertension expert centers in Greece and included patients diagnosed with CTEPH...
May 17, 2017: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
https://www.readbyqxmd.com/read/28527960/mortality-and-respiratory-failure-after-thoracoscopic-lung-biopsy-for-interstitial-lung-disease
#18
Michael T Durheim, Sunghee Kim, Brian C Gulack, William R Burfeind, Henning A Gaissert, Andrzej S Kosinski, Matthew G Hartwig
BACKGROUND: Surgical lung biopsy contributes to establishing a specific diagnosis among many patients with interstitial lung disease (ILD). The risks of death and respiratory failure associated with elective thoracoscopic surgical lung biopsy, and patient characteristics associated with these outcomes, are not well understood. METHODS: This is a retrospective cohort study of patients who underwent elective thoracoscopic lung biopsy for ILD between 2008 and 2014, according to The Society of Thoracic Surgeons database...
May 17, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28524624/pulmonary-arterial-hypertension-progress-in-understanding-the-disease-and-prioritizing-strategies-for-drug-development
#19
REVIEW
P Ghataorhe, C J Rhodes, L Harbaum, M Attard, J Wharton, M R Wilkins
Pulmonary arterial hypertension (PAH), at one time a largely overlooked disease, is now the subject of intense study in many academic and biotech groups. The availability of new treatments has increased awareness of the condition. This in turn has driven a change in the demographics of PAH, with an increase in the mean age at diagnosis. The diagnosis of PAH in more elderly patients has highlighted the need for careful phenotyping of patients and for further studies to understand how best to manage pulmonary hypertension associated with, for example, left heart disease...
May 19, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28521893/pulmonary-hypertension-in-infants-children-and-young-adults
#20
REVIEW
Georg Hansmann
Pulmonary hypertension (PH) in neonates, infants, children, adolescents, and young adults is a complex condition that can be associated with several cardiac, pulmonary, and systemic diseases contributing to morbidity and mortality. The underlying pulmonary hypertensive vascular disease (PHVD) is characterized by inflammation, pulmonary vascular remodeling, and angio-obliteration leading to elevated pulmonary arterial pressure and resistance, right ventricular dysfunction, left ventricular compression, and subsequent heart failure...
May 23, 2017: Journal of the American College of Cardiology
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