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https://www.readbyqxmd.com/read/28332379/a-novel-de-novo-pathogenic-variant-in-foxf1-in-a-newborn-with-alveolar-capillary-dysplasia-with-misalignment-of-pulmonary-veins
#1
Youngeun Ma, Mi Ae Jang, Hye Soo Yoo, So Yoon Ahn, Se In Sung, Yun Sil Chang, Chang Seok Ki, Won Soon Park
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is an autosomal dominant, fatal developmental disorder of the lungs, with a mortality rate of about 100%. ACD/MPV is caused by mutations in FOXF1. Herein, we describe a newborn boy with ACD/MPV carrying a novel pathogenic variant of FOXF1. The patient developed respiratory distress and severe pulmonary hypertension on the first day of life. Despite aggressive cardiorespiratory management, including veno-venous extracorporeal membrane oxygenation, his condition deteriorated rapidly, and he died within the first month of his life...
May 2017: Yonsei Medical Journal
https://www.readbyqxmd.com/read/28332341/risk-factors-for-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-systematic-review-and-meta-analysis
#2
Meihua Qiu, Yuqing Chen, Qiao Ye
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic andprogressive fibrotic disease limited to the lungs.The course of disease varies widely, with some patients experiencing acute respiratory deterioration, a conditioncalled acute exacerbations of IPF (AE-IPF). The risk factors contributing to AE-IPF are unclear. This systematic review and meta-analysis investigatedthe risk factors for AE-IPF. METHODS: Studies ofrisk factors for AE-IPF were identified in Medline, EMBASE and Cochrane databases...
March 23, 2017: Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28331629/congenital-diaphragmatic-hernia-a-review
#3
REVIEW
Praveen Kumar Chandrasekharan, Munmun Rawat, Rajeshwari Madappa, David H Rothstein, Satyan Lakshminrusimha
Congenital Diaphragmatic hernia (CDH) is a condition characterized by a defect in the diaphragm leading to protrusion of abdominal contents into the thoracic cavity interfering with normal development of the lungs. The defect may range from a small aperture in the posterior muscle rim to complete absence of diaphragm. The pathophysiology of CDH is a combination of lung hypoplasia and immaturity associated with persistent pulmonary hypertension of newborn (PPHN) and cardiac dysfunction. Prenatal assessment of lung to head ratio (LHR) and position of the liver by ultrasound are used to diagnose and predict outcomes...
2017: Maternal Health, Neonatology and Perinatology
https://www.readbyqxmd.com/read/28331450/pulmonary-thromboendarterectomy-for-pulmonary-hypertension-before-considering-transplant
#4
Hannah Kooperkamp, Inder Mehta, David Fary, Michael Bates
BACKGROUND: In cases of chronic thromboembolic pulmonary hypertension (CTEPH), referral for possible surgical intervention is important because surgery can be curative. Surgery necessitates cardiopulmonary bypass and deep circulatory arrest with pulmonary thrombectomy and bilateral endarterectomy (PTE). If surgery fails, lung transplant is the next best surgical option. Medical treatment is also an important adjunct. CASE REPORT: A 35-year-old female presented 3 months after a pulmonary embolus was found to be completely occluding her left pulmonary artery...
2017: Ochsner Journal
https://www.readbyqxmd.com/read/28330842/monocrotaline-induces-endothelial-injury-and-pulmonary-hypertension-by-targeting-the-extracellular-calcium-sensing-receptor
#5
Rui Xiao, Yuan Su, Tian Feng, Mengxiang Sun, Bingxun Liu, Jiwei Zhang, Yankai Lu, Jiansha Li, Tao Wang, Liping Zhu, Qinghua Hu
BACKGROUND: Monocrotaline has been widely used to establish an animal model of pulmonary hypertension. The molecular target underlying monocrotaline-induced pulmonary artery endothelial injury and pulmonary hypertension remains unknown. The extracellular calcium-sensing receptor (CaSR) and particularly its extracellular domain hold the potential structural basis for monocrotaline to bind. This study aimed to reveal whether monocrotaline induces pulmonary hypertension by targeting the CaSR...
March 22, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28330686/imaging-of-acute-and-chronic-thromboembolic-disease-state-of-the-art
#6
REVIEW
A Ruggiero, N J Screaton
Acute pulmonary embolism (PE) is a life-threatening condition that requires prompt diagnosis and treatment. Recent advances in imaging allow acute and rapid recognition even by the non-specialist radiologist. Most acute emboli resolve on anticoagulation without sequelae; however, some emboli fail to fully resolve becoming endothelialised with the development of chronic thromboembolic disease (CTED). Increased pulmonary vascular resistance arising from CTED may lead to chronic thromboembolic pulmonary hypertension (CTEPH) a debilitating disease affecting up to 5% of survivors of acute PE...
March 19, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28330499/a-novel-multi-network-approach-reveals-tissue-specific-cellular-modulators-of-fibrosis-in-systemic-sclerosis
#7
Jaclyn N Taroni, Casey S Greene, Viktor Martyanov, Tammara A Wood, Romy B Christmann, Harrison W Farber, Robert A Lafyatis, Christopher P Denton, Monique E Hinchcliff, Patricia A Pioli, J Matthew Mahoney, Michael L Whitfield
BACKGROUND: Systemic sclerosis (SSc) is a multi-organ autoimmune disease characterized by skin fibrosis. Internal organ involvement is heterogeneous. It is unknown whether disease mechanisms are common across all involved affected tissues or if each manifestation has a distinct underlying pathology. METHODS: We used consensus clustering to compare gene expression profiles of biopsies from four SSc-affected tissues (skin, lung, esophagus, and peripheral blood) from patients with SSc, and the related conditions pulmonary fibrosis (PF) and pulmonary arterial hypertension, and derived a consensus disease-associate signature across all tissues...
March 23, 2017: Genome Medicine
https://www.readbyqxmd.com/read/28329267/right-ventricular-recovery-after-bilateral-lung-transplantation-for-pulmonary-arterial-hypertension%C3%A2
#8
Thomas M Gorter, Erik A M Verschuuren, Dirk J van Veldhuisen, Elke S Hoendermis, Michiel E Erasmus, Harm J Bogaard, Anton Vonk Noordegraaf, Rolf M F Berger, Joost P van Melle, Tineke P Willems
OBJECTIVES: Pulmonary arterial hypertension (PAH) is a progressive and often fatal disease characterized by increased pulmonary vascular resistance (PVR) and right ventricular (RV) failure. End-stage PAH is often an indication for a lung transplant (LTX). Our goal was to study ventricular recovery using cardiac magnetic resonance imaging late after LTX. METHODS: We studied 10 patients with PAH who underwent isolated bilateral LTX. RV and left ventricular (LV) volumes, function and mass were measured...
March 3, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329232/mid-term-results-of-bilateral-lung-transplant-with-postoperatively-extended-intraoperative-extracorporeal-membrane-oxygenation-for-severe-pulmonary-hypertension%C3%A2
#9
Jawad Salman, Fabio Ius, Wiebke Sommer, Thierry Siemeni, Christian Kuehn, Murat Avsar, Dietmar Boethig, Ulrich Molitoris, Christoph Bara, Jens Gottlieb, Tobias Welte, Axel Haverich, Marius M Hoeper, Gregor Warnecke, Igor Tudorache
OBJECTIVES: In severe pulmonary hypertension, diastolic dysfunction of the left ventricle causes significant morbidity and mortality after lung transplantation, which may be successfully reversed using a protocol based on perioperative veno-arterial extracorporeal membrane oxygenation (ECMO) and early extubation. Here, we present echocardiographic data and mid-term outcomes. METHODS: The records of lung transplanted patients at our institution between May 2010 and January 2016 were retrospectively reviewed...
March 2, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28327934/pazopanib-or-placebo-in-completely-resected-stage-i-nsclc-patients-results-of-the-phase-ii-ifct-0703-trial
#10
B Besse, J Mazières, L Ribassin-Majed, F Barlesi, J Bennouna, R Gervais, L Moreau, H Berard, D Debieuvre, O Molinier, D Moro-Sibilot, P J Souquet, S Jacquot, L Petit, H Lena, J P Pignon, B Lacas, F Morin, B Milleron, G Zalcman, J C Soria
Background: Adjuvant treatment in resected stage I non-small cell lung cancer (NSCLC) is generally not recommended. Pazopanib is an oral tyrosine kinase inhibitor of VEGFR-1/2/3 and PDGFR-α/β. We explored the feasibility and efficacy of adjuvant pazopanib in this population. Patients and methods: In this double-blind phase II/III trial, patients with resected stage I NSCLC were randomized to placebo or pazopanib 800 mg/d (P800) for 6 months with a two-step Fleming design...
February 23, 2017: Annals of Oncology: Official Journal of the European Society for Medical Oncology
https://www.readbyqxmd.com/read/28321847/antenatal-corticosteroids-for-accelerating-fetal-lung-maturation-for-women-at-risk-of-preterm-birth
#11
REVIEW
Devender Roberts, Julie Brown, Nancy Medley, Stuart R Dalziel
BACKGROUND: Respiratory morbidity including respiratory distress syndrome (RDS) is a serious complication of preterm birth and the primary cause of early neonatal mortality and disability. While researching the effects of the steroid dexamethasone on premature parturition in fetal sheep in 1969, Liggins found that there was some inflation of the lungs of lambs born at gestations at which the lungs would be expected to be airless. Liggins and Howie published the first randomised controlled trial in humans in 1972 and many others followed...
March 21, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#12
Michaël Levy, Nolwenn Le Sache, Mostafa Mokhtari, Guy Fagherazzi, Gaelle Cuzon, Benjamin Bueno, Virginie Fouquet, Alexandra Benachi, Sergio Eleni Dit Trolli, Pierre Tissieres
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. METHODS: This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28321539/secoisolariciresinol-diglucoside-attenuates-cardiac-hypertrophy-and-oxidative-stress-in-monocrotaline-induced-right-heart-dysfunction
#13
Stephanie Puukila, Rafael Oliveira Fernandes, Patrick Türck, Cristina Campos Carraro, Jéssica Hellen Poletto Bonetto, Bruna Gazzi de Lima-Seolin, Alex Sander da Rosa Araujo, Adriane Belló-Klein, Douglas Boreham, Neelam Khaper
Pulmonary arterial hypertension (PAH) occurs when remodeling of pulmonary vessels leads to increased pulmonary vascular resistance resulting in increased pulmonary arterial pressure. Increased pulmonary arterial pressure results in right ventricle hypertrophy and eventually heart failure. Oxidative stress has been implicated in the pathogenesis of PAH and may play a role in the regulation of cellular signaling involved in cardiac response to pressure overload. Secoisolariciresinol diglucoside (SDG), a component from flaxseed, has been shown to reduce cardiac oxidative stress in various pathophysiological conditions...
March 20, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28320395/vasoproliferative-process-resembling-pulmonary-capillary-hemangiomatosis-in-a-cat
#14
J A Jaffey, K J Williams, I Masseau, M Krueger, C Reinero
BACKGROUND: Pulmonary capillary hemangiomatosis is a rare, vascular obstructive disorder that uniformly causes pulmonary arterial hypertension. Clinically, pulmonary capillary hemangiomatosis is indistinguishable from primary pulmonary arterial hypertension and histology is required for definitive diagnosis. The distinctive histologic feature of pulmonary capillary hemangiomatosis is non-malignant extensive proliferation of capillaries in the alveolar septae. Vasodilator treatment of humans with primary arterial hypertension due to pulmonary capillary hemangiomatosis can result in fatal acute pulmonary edema...
March 20, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/28316463/the-role-of-serotonin-transporter-in-human-lung-development-and-in-neonatal-lung-disorders
#15
E C C Castro, P Sen, W T Parks, C Langston, C Galambos
Introduction. Failure of the vascular pulmonary remodeling at birth often manifests as pulmonary hypertension (PHT) and is associated with a variety of neonatal lung disorders including a uniformly fatal developmental disorder known as alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV). Serum serotonin regulation has been linked to pulmonary vascular function and disease, and serotonin transporter (SERT) is thought to be one of the key regulators in these processes. We sought to find evidence of a role that SERT plays in the neonatal respiratory adaptation process and in the pathomechanism of ACD/MPV...
2017: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
https://www.readbyqxmd.com/read/28315287/elective-primary-or-secondary-delayed-sternal-closure-improves-outcome-in-severely-compromised-patients
#16
Antonio M Calafiore, Azmat A Sheikh, Juan J Alfonso, Tarek Tantawi, Belgeit Maklouf, Ahmed Shawki, Ahmed Allam, Mohammed O Awadi, Ahmed A Osman, Aly M Habib, Michele Di Mauro
Background Delayed sternal closure (DSC) in patients with severely compromised preoperative hemodynamics can be helpful as the chest sometimes cannot be able to contain both lungs and heart. We report our experience to evaluate the midterm results of this strategy in an adult population. Materials and Methods From May 2009 till July 2015, 33 patients had DSC as first treatment of severe hemodynamic deterioration after cardiac surgery. Surgical procedures were valvular (9.27%) or coronary artery bypass grafting + others (24...
March 17, 2017: Thoracic and Cardiovascular Surgeon
https://www.readbyqxmd.com/read/28314671/a-case-of-pulmonary-hypertension-due-to-fistulas-between-multiple-systemic-arteries-and-the-right-pulmonary-artery-in-an-adult-discovered-for-occulted-dyspnoea
#17
Ji-Feng Li, Zhen-Guo Zhai, Tu-Guang Kuang, Min Liu, Zhan-Hong Ma, Yi-Dan Li, Yuan-Hua Yang
BACKGROUND: Pulmonary hypertension (PH) can be caused by a fistula between the systemic and pulmonary arteries. Here, we report a case of PH due to multiple fistulas between systemic arteries and the right pulmonary artery where the ventilation/perfusion scan showed no perfusion in the right lung. METHODS: A 32-year-old male patient was hospitalised for community-acquired pneumonia. After treatment with antibiotics, the pneumonia was alleviated but dyspnoea persisted...
February 22, 2017: Heart, Lung & Circulation
https://www.readbyqxmd.com/read/28302503/the-giessen-pulmonary-hypertension-registry-survival-in-pulmonary-hypertension-subgroups
#18
Henning Gall, Janine F Felix, Franziska K Schneck, Katrin Milger, Natascha Sommer, Robert Voswinckel, Oscar H Franco, Albert Hofman, Ralph T Schermuly, Norbert Weissmann, Friedrich Grimminger, Werner Seeger, Hossein A Ghofrani
BACKGROUND: Pulmonary hypertension (PH) is a severe, progressive disease. Although 5 PH subgroups are recognized, reports on survival have focused mainly on pulmonary arterial hypertension (PAH). METHODS: Long-term transplant-free survival and its determinants were investigated in patients with PH (diagnosed by right heart catheterization) within a prospective registry at a single referral center in Giessen, Germany. RESULTS: In total, 2,067 patients were enrolled (PAH, 685 patients [33...
February 17, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28301582/task-1-potassium-channel-is-not-critically-involved-in-mediating-hypoxic-pulmonary-vasoconstriction-of-murine-intra-pulmonary-arteries
#19
Ghulam Murtaza, Petra Mermer, Anna Goldenberg, Uwe Pfeil, Renate Paddenberg, Nobert Weissmann, Guenter Lochnit, Wolfgang Kummer
The two-pore domain potassium channel KCNK3 (TASK-1) is expressed in rat and human pulmonary artery smooth muscle cells. There, it is associated with hypoxia-induced signalling, and its dysfunction is linked to pathogenesis of human pulmonary hypertension. We here aimed to determine its role in hypoxic pulmonary vasoconstriction (HPV) in the mouse, and hence the suitability of this model for further mechanistic investigations, using appropriate inhibitors and TASK-1 knockout (KO) mice. RT-PCR revealed expression of TASK-1 mRNA in murine lungs and pre-acinar pulmonary arteries...
2017: PloS One
https://www.readbyqxmd.com/read/28298957/the-intersection-of-pulmonary-hypertension-and-solid-organ-transplantation
#20
Adaani E Frost
Pulmonary hypertension (PH) is a complication and marker of disease severity in many parenchymal lung diseases. It also is a frequent complication of portal hypertension and negatively impacts survival with liver transplant. Pulmonary hypertension is frequently diagnosed in patients with end-stage renal disease who are undergoing dialysis, and it has recently been demonstrated to adversely affect posttransplant outcome in this patient population even though the mechanism of PH is substantially different from that associated with liver disease...
October 2016: Methodist DeBakey Cardiovascular Journal
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