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Pulmonary Circulation

G Ruffenach, S Bonnet, S Rousseaux, S Khochbin, S Provencher, F Perros
Pulmonary arterial hypertension (PAH) shares many hallmarks with cancer. Cancer cells acquire their hallmarks by a pathological Darwinian evolution process built on the so-called cancer cell "identity crisis." Here we demonstrate that PAH shares the most striking features of the cancer identity crisis: the ectopic expression of normally silent tissue-specific genes.
January 2018: Pulmonary Circulation
James C Coons, Cheryl Bunner, David C Ishizawar, Michael G Risbano, Belinda Rivera-Lebron, Michael A Mathier, Stephen Y Chan, Marc A Simon
Oral treprostinil (TRE) is a prostacylin that is approved for the treatment of patients with pulmonary arterial hypertension (PAH). Dosing is approved for two or three times daily (t.i.d.); however, adverse effects, including gastrointestinal-related symptoms, may limit the ability to reach optimal doses. We report our experience with a four times daily (q.i.d.) regimen of oral TRE for goal-directed therapy of PAH. We describe three patients that were transitioned from infusion or inhaled TRE to oral TRE with initial t...
January 2018: Pulmonary Circulation
Kaori Oshima, Sarah M Haeger, Joseph A Hippensteel, Paco S Herson, Eric P Schmidt
Advances in tissue fixation and imaging techniques have yielded increasing appreciation for the glycosaminoglycan-rich endothelial glycocalyx and its in vivo manifestation, the endothelial surface layer (ESL). Pathological loss of the ESL during critical illness promotes local endothelial dysfunction and, consequently, organ injury. Glycosaminoglycan fragments, such as heparan sulfate, are released into the plasma of animals and humans after ESL degradation and have thus served as a biomarker of endothelial injury...
January 2018: Pulmonary Circulation
Mary Beth Brown, Attie Kempf, Catherine M Collins, Gary M Long, Matthew Owens, Shikha Gupta, Yaron Hellman, Vincent Wong, Mark Farber, Tim Lahm
Current evidence suggests that exercise training is beneficial in pulmonary arterial hypertension (PAH). Unfortunately, the standard supervised, hospital-based programs limit patient accessibility to this important intervention. Our proof-of-concept study aimed to provide insight into the usefulness of a prescribed walking regimen along with arginine supplementation to improve outcomes for patients with PAH. Twelve PAH patients (all women) in New York Heart Association (NYHA) functional class (FC) II (n = 7) or III (n = 5) and in stable condition for ≥ 3 months were enrolled...
January 2018: Pulmonary Circulation
Grant H Lowther, Jason Chertoff, Jessica Cope, Hassan Alnuaimat, Ali Ataya
Adult-onset Still's disease (AOSD) is an inflammatory disorder characterized by recurrent fevers, arthralgia, leukocytosis, and a salmon-colored rash. Diagnosis is made based on the Yamaguchi criteria. Various cardiac and pulmonary manifestations have been described in association with AOSD, including acute respiratory distress syndrome (ARDS) and pulmonary arterial hypertension (PAH). We describe the first case of both PAH and ARDS in a patient with AOSD who, despite aggressive therapy, declined rapidly and ultimately died...
October 2017: Pulmonary Circulation
Mohamad Taha, Virgilio J Cadete, Duncan J Stewart
No abstract text is available yet for this article.
October 2017: Pulmonary Circulation
(no author information available yet)
No abstract text is available yet for this article.
October 2017: Pulmonary Circulation
Karan Seegobin, Amit Babbar, Jason Ferreira, Brittany Lyons, James Cury, Vandana Seeram
A 52-year-old man with a past medical history of chronic myeloid leukemia (CML) in remission developed progressive shortness of breath over a two-month period. He was initially treated with dasatinib for four years, until developing pulmonary arterial hypertension (PAH) with pleural effusions. His symptoms improved after stopping dasatinib. He was then switched to bosutinib for approximately one year, which was then stopped before admission due to worsening shortness of breath. His initial workup showed bilateral pleural effusions with severe PAH and cor pulmonale...
October 2017: Pulmonary Circulation
Nadine Al-Naamani, Jessica K Paulus, Kari E Roberts, Michael W Pauciulo, Katie Lutz, William C Nichols, Steven M Kawut
This study explores the racial and ethnic differences in presentation, severity, and treatment of patients with pulmonary arterial hypertension (PAH) in a large multicenter registry. African American and Hispanic patients are more likely to present with associated PAH compared to non-Hispanic whites. Hispanic patients with PAH were less likely to be treated with PAH-specific medications compared to non-Hispanic whites.
October 2017: Pulmonary Circulation
Ji Young Lee, Sarah A McMurtry, Troy Stevens
Pulmonary artery, capillary, and vein endothelial cells possess distinctive structures and functions, which represent a form of vascular segment specific macroheterogeneity. However, within each of these segmental populations, individual cell functional variability represents a poorly characterized microheterogeneity. Here, we hypothesized that single cell clonogenic assays would reveal microheterogeneity among the parent cell population and enable isolation of highly representative cells with committed parental characteristics...
October 2017: Pulmonary Circulation
Josephine Pickworth, Alexander Rothman, James Iremonger, Helen Casbolt, Kay Hopkinson, Peter M Hickey, Santhi Gladson, Sheila Shay, Nicholas W Morrell, Sheila E Francis, James D West, Allan Lawrie
Bone morphogenetic protein receptor type 2 (BMPR2) mutations are present in patients with heritable and idiopathic pulmonary arterial hypertension (PAH). Circulating levels of interleukin-1 (IL-1) are raised in patients and animal models. Whether interplay between BMP and IL-1 signaling can explain the local manifestation of PAH in the lung remains unclear. Cell culture, siRNA, and mRNA microarray analysis of RNA isolated from human pulmonary artery (PASMC) and aortic (AoSMC) smooth muscle cells were used. R899X(+/-) BMPR2 transgenic mice fed a Western diet for six weeks were given daily injections of IL-1ß prior to assessment for PAH and tissue collection...
October 2017: Pulmonary Circulation
Rohit Godbole, Rajan Saggar, Alexander Zider, Jamie Betancourt, William D Wallace, Robert D Suh, Nader Kamangar
Pulmonary tumor thrombotic microangiopathy (PTTM) is a disease process wherein tumor cells are thought to embolize to the pulmonary circulation causing pulmonary hypertension (PH) and death from right heart failure. Presented herein are clinical, laboratory, radiographic, and histologic features across seven cases of PTTM. Highlighted in this publication are also involvement of pulmonary venules and clinical features distinguishing PTTM from clinical mimics. We conducted a retrospective chart review of seven cases of PTTM from hospitals in the greater Los Angeles metropolitan area...
October 2017: Pulmonary Circulation
Matthew Jankowich, Ryan Hebel, Jennifer Jantz, Siddique Abbasi, Gaurav Choudhary
Pulmonary hypertension (PH) is often associated with cardiopulmonary co-morbidities, especially in older adults. A multispecialty approach to suspected PH is recommended, but there are few data on adherence to guidelines or outcomes in such patients. This was a single-center retrospective study of consecutively evaluated Veteran patients with suspected PH evaluated in a multispecialty PH clinic at a Veterans Affairs Medical Center, evaluating clinical characteristics, workup outcomes, and prognosis. The referral population (n = 125) was older (mean ± SD age = 73...
October 2017: Pulmonary Circulation
Arun Jose, Hind Rafei, Jalil Ahari
Dasatinib is a small-molecule tyrosine kinase inhibitor used in the treatment of hematological malignancies. Pulmonary arterial hypertension (PAH) is a rare but known complication. The mainstay of treatment is cessation of Dasatinib, and while clinical improvement is rapid, complete hemodynamic resolution of pulmonary hypertension (PH) still remains exceedingly uncommon. We present a case of Dasatinib-induced PAH in a woman with chronic myeloid leukemia, who demonstrated rapid and complete clinical and hemodynamic resolution following treatment with combination pulmonary vasodilator therapy using an endothelin receptor antagonist and a phosphodiesterase-5 inhibitor...
October 2017: Pulmonary Circulation
Rudolf K F Oliveira, Mariana Faria-Urbina, Bradley A Maron, Mario Santos, Aaron B Waxman, David M Systrom
Borderline resting mean pulmonary arterial pressure (mPAP) is associated with adverse outcomes and affects the exercise pulmonary vascular response. However, the pathophysiological mechanisms underlying exertional intolerance in borderline mPAP remain incompletely characterized. In the current study, we sought to evaluate the prevalence and functional impact of exercise pulmonary hypertension (ePH) across a spectrum of resting mPAP's in consecutive patients with contemporary resting right heart catheterization (RHC) and invasive cardiopulmonary exercise testing...
July 2017: Pulmonary Circulation
Haiyang Tang, Rebecca R Vanderpool, Jian Wang, Jason X-J Yuan
No abstract text is available yet for this article.
July 2017: Pulmonary Circulation
Thenappan Thenappan
No abstract text is available yet for this article.
July 2017: Pulmonary Circulation
Horst Olschewski
No abstract text is available yet for this article.
July 2017: Pulmonary Circulation
Rebecca Johnson Kameny, Elizabeth Colglazier, Hythem Nawaytou, Phillip Moore, V Mohan Reddy, David Teitel, Jeffrey R Fineman
Pulmonary arterial hypertension (PAH) is a frequent complication of congenital heart disease as a consequence of altered pulmonary hemodynamics with increased pulmonary blood flow and pressure. The development of pulmonary vascular disease (PVD) in this patient population is an important concern in determining operative strategy. Early, definitive surgical repair, when possible, is the best therapy to prevent and treat PVD. However, this is not possible in some patients because they either presented late, after the development of PVD, or they have complex lesions not amenable to one-step surgical correction, including patients with single ventricle physiology, who have a continuing risk of developing PVD...
July 2017: Pulmonary Circulation
Anna D T Muntingh, Sylvia Gerritsen, Neeltje M Batelaan, Harm Jan Bogaard
Pulmonary arterial hypertension (PAH) affects the physical and emotional wellbeing of patients. More information is needed regarding mental problems and preferences for support. Qualitative interviews were used to explore mental problems and preference for support of PAH patients. Additionally, a survey was used to assess the presence of mental problems (Problem List), distress (Distress Thermometer) and the need for mental support. In 24 semi-structured interviews, the following problem themes were identified: energy balance; loss of abilities; feeling misunderstood; and handling of worries and emotions...
July 2017: Pulmonary Circulation
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