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Pediatric pulmonary arterial hypertension

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https://www.readbyqxmd.com/read/28078552/pediatric-development-of-bosentan-facilitated-by-modeling-and-simulation
#1
Jochen Zisowsky, Martine Géhin, Andjela Kusic-Pajic, Andreas Krause, Maurice Beghetti, Jasper Dingemanse
BACKGROUND: Bosentan is approved for use in adult patients with pulmonary arterial hypertension. The primary aim of the pharmacokinetic modeling was the provision of a systematic guidance for study design and enhanced understanding of pharmacokinetics across the entire pediatric age range. METHODS: A physiologically based pharmacokinetic model was developed for the pediatric population; starting from an adult model, the effects of body weight, age, and maturation of relevant metabolizing enzymes were incorporated to extrapolate the pharmacokinetics to children...
January 11, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28072745/safety-and-efficacy-of-chloral-hydrate-for-conscious-sedation-of-infants-in-the-pediatric-cardiovascular-intensive-care-unit
#2
Mei-Lian Chen, Qiang Chen, Fan Xu, Jia-Xin Zhang, Xiao-Ying Su, Xiao-Zhen Tu
This study evaluates the safety and efficacy of chloral hydrate administration for the conscious sedation of infants in the pediatric cardiovascular intensive care unit (PCICU).We conducted a retrospective review of the charts of 165 infants with congenital heart disease who received chloral hydrate in our PCICU between January 2014 and December 2014. Chloral hydrate was administered orally or rectally to infants using doses of 50 mg/kg. We collected and analyzed relevant clinical parameters.The overall length of time to achieve sedation was ranged from 5 to 35 min (10...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28029743/statins-ameliorate-pulmonary-hypertension-secondary-to-left-ventricular-dysfunction-through-the-rho-kinase-pathway-and-nadph-oxidase
#3
I-Chen Chen, Mian-Shin Tan, Bin-Nan Wu, Chee-Yin Chai, Jwu-Lai Yeh, Shah-Hwa Chou, Ing-Jun Chen, Zen-Kong Dai
BACKGROUND: Pulmonary hypertension (PH) is a devastating disorder, for which no therapy is curative. It has been reported that pulmonary vascular remodeling, associated with increasing mean pulmonary arterial pressure and upregulated expression of endothelial nitric oxide synthase (eNOS), endothelin-1 (ET-1), RhoA/RhoH-kinase results in the development of PH. Oxidative stress and the RhoA/Rho-kinase pathway are also thought to be involved in the pathophysiology of PH. Statins are 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors (HMG-CoA reductase inhibitors) with pleiotropic effects and are potential agents for the treatment of PH...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27995825/treatment-initiation-in-paediatric-pulmonary-hypertension-insights-from-a-multinational-registry
#4
Tilman Humpl, Rolf M F Berger, Eric D Austin, Margrit S Fasnacht Boillat, Damien Bonnet, Dunbar D Ivy, Malgorzata Zuk, Maurice Beghetti, Ingram Schulze-Neick
Different treatment options for pulmonary hypertension have emerged in recent years, and evidence-based management strategies have improved quality of life and survival in adults. In children with pulmonary vascular disease, therapeutic algorithms are not so clearly defined; this study determined current treatment initiation in children with pulmonary hypertension in participating centres of a registry. Through the multinational Tracking Outcomes and Practice in Pediatric Pulmonary Hypertension registry, patient demographics, diagnosis, and treatment as judged and executed by the local physician were collected...
December 20, 2016: Cardiology in the Young
https://www.readbyqxmd.com/read/27861431/pediatric-artificial-lung-a-low-resistance-pumpless-artificial-lung-alleviates-an-acute-lamb-model-of-increased-right-ventricle-afterload
#5
Fares Alghanem, Benjamin S Bryner, Emilia M Jahangir, Uditha P Fernando, John M Trahanas, Hayley R Hoffman, Robert H Bartlett, Alvaro Rojas-Peña, Ronald B Hirschl
Lung disease in children often results in pulmonary hypertension and right heart failure. The availability of a pediatric artificial lung (PAL) would open new approaches to the management of these conditions by bridging to recovery in acute disease or transplantation in chronic disease. This study investigates the efficacy of a novel PAL in alleviating an animal model of pulmonary hypertension and increased right ventricle afterload.Five juvenile lambs (20-30 Kg) underwent PAL implantation in a pulmonary artery to left atrium configuration...
November 15, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27860161/closure-of-very-large-pda-with-pulmonary-hypertension-initial-clinical-case-series-with-the-new-occlutech-%C3%A2-pda-occluder
#6
Anja Lehner, Sarah Ulrich, Christoph M Happel, Marcus Fischer, Marinos Kantzis, Ingram Schulze-Neick, Nikolaus A Haas
OBJECTIVE: We report our recent experience with the new Occlutech(®) PDA Occluder, which seems suitable especially for closure of large patent ductus arteriosus (PDA) sizes with elevated pulmonary artery pressure (PAH). BACKGROUND: Although considered a standard procedure in pediatric cath labs, closure of very large PDA (PDA/Aorta ratio > 0.5) and PAH remains a challenge. METHODS/RESULTS: The Occlutech(®) PDA occluder was used in eight children and adolescents (age 4-16, median 10...
November 10, 2016: Catheterization and Cardiovascular Interventions
https://www.readbyqxmd.com/read/27832003/development-of-a-model-of-pediatric-lung-failure-pathophysiology
#7
John M Trahanas, Fares Alghanem, Catalina Ceballos-Muriel, Hayley R Hoffman, Alice Xu, Kristopher B Deatrick, Marie Cornell, Alvaro Rojas-Pena, Robert H Bartlett, Ronald B Hirschl
A pediatric artificial lung (PAL) is under development as a bridge to transplantation or lung remodeling for children with end-stage lung failure (ESLF). In order to evaluate the efficiency of a PAL, a disease model mimicking the physiologic derangements of pediatric ESLF is needed. Our previous right pulmonary artery ligation (rPA-LM) ovine model achieved that goal, but caused immediate mortality in nearly half of the animals. In this study, we evaluated a new technique of gradual postoperative right pulmonary artery (rPA) occlusion using a Rummel tourniquet (rPA-RT) in seven (25-40 Kg) sheep...
November 9, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27826712/subclinical-pulmonary-hypertension-in-childhood-systemic-lupus-erythematosus-associated-with-minor-disease-manifestations
#8
Pedro Anuardo, Monica Verdier, Natali W S Gormezano, Gabriela R V Ferreira, Gabriela N Leal, Alessandro Lianza, Juliana C O A Ferreira, Rosa M R Pereira, Nadia E Aikawa, Maria Teresa Terreri, Claudia S Magalhães, Simone Appenzeller, Maria Carolina Dos Santos, Silvana B Sachetti, Claudio A Len, Gecilmara S Pilleggi, Simone Lotufo, Eloisa Bonfá, Clovis A Silva
The aim of this study was to evaluate pulmonary hypertension (PH) in 852 childhood-onset systemic lupus erythematosus (cSLE) patients. This was a large multicenter study conducted in 10 Pediatric Rheumatology Services of São Paulo state, Brazil. PH was defined as systolic pulmonary artery pressure >35 mmHg and/or measurement of the mean pulmonary artery pressure >25 mmHg and/or diastolic pressure >15 mmHg by transthoracic echocardiogram. Demographic data, clinical manifestations, disease activity score (SLEDAI-2K), disease damage score (SLICC/ACR-DI) and treatments were also evaluated...
November 8, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27801982/risk-factors-for-pulmonary-arterial-hypertension-in-children-and-young-adults
#9
Estelle Naumburg, Lars Söderström, Daniel Huber, Inge Axelsson
OBJECTIVES: Pulmonary hypertension (PH) has been linked to preterm birth explained by congenital heart defects and pulmonary diseases. WORKING HYPOTHESIS: Other factors may influence the risk of PH among adolescences and children born premature. STUDY DESIGN: This national registry-based study assess risk of PH following premature birth adjusted for known risk factors. PATIENT-SUBJECT SELECTION AND METHODOLOGY: All cases born 1993-2010, identified by diagnostic codes applicable to PH and retrieved from the Swedish Registry of Congenital Heart Disease (N = 67)...
November 1, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27800456/successful-lung-transplant-after-prolonged-extracorporeal-membrane-oxygenation-ecmo-in-a-child-with-pulmonary-hypertension-a-case-report
#10
Cecile Tissot, Walid Habre, Paola Soccal, Maja Isabel Hug, Dominique Bettex, Michel Pellegrini, Yacine Aggoun, Anne Mornand, Afksendyios Kalangos, Peter Rimensberger, Maurice Beghetti
INTRODUCTION: The use of extracorporeal membrane oxygenation (ECMO) is considered a risk factor for, or even a potential contraindication to, lung transplantation. However, only a few pediatric cases have been described thus far. CASE PRESENTATION: A 9-year-old boy with idiopathic pulmonary arterial hypertension developed cardiac arrest after the insertion of a central catheter. ECMO was used as a bridge to lung transplantation. However, after prolonged resuscitation, he developed medullary ischemia and medullary syndrome...
August 2016: Research in Cardiovascular Medicine
https://www.readbyqxmd.com/read/27770446/the-role-of-genetics-in-pulmonary-arterial-hypertension
#11
REVIEW
Lijiang Ma, Wendy K Chung
Group 1 pulmonary hypertension or pulmonary arterial hypertension (PAH) is a rare disease characterized by proliferation and occlusion of small pulmonary arterioles, leading to progressive elevation of pulmonary artery pressure and pulmonary vascular resistance, and right ventricular failure. Historically, it has been associated with a high mortality rate, although, over the last decade, treatment has improved survival. PAH includes idiopathic PAH (IPAH), heritable PAH (HPAH), and PAH associated with certain medical conditions...
January 2017: Journal of Pathology
https://www.readbyqxmd.com/read/27760452/-chronic-thromboembolic-pulmonary-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#12
H Wilkens, S Konstantinides, I Lang, A C Bunck, M Gerges, F Gerhardt, A Grgic, C Grohé, S Guth, M Held, J Hinrichs, M M Hoeper, W Klepetko, T Kramm, U Krüger, M Lankeit, B C Meyer, K M Olsson, H-J Schäfers, M Schmidt, H J Seyfarth, S Ulrich, C B Wiedenroth, E Mayer
The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH), but also other significant subgroups of pulmonary hypertension (PH). In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany to discuss open and controversial issues surrounding the practical implementation of the European Guidelines...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760451/-pulmonary-hypertension-due-to-chronic-lung-disease-recommendations-of-the-cologne-consensus-conference-2016
#13
H Olschewski, J Behr, H Bremer, M Claussen, P Douschan, M Halank, M Held, M M Hoeper, S Holt, H Klose, S Krüger, T J Lange, F Reichenberger, D Skowasch, S Ulrich, H Wilkens, W Seeger
The 2015 European Guidelines on Pulmonary Hypertension did not cover only pulmonary arterial hypertension (PAH) but also some aspects of pulmonary hypertension (PH) associated with chronic lung disease. The European Guidelines point out that the drugs currently used to treat patients with PAH (prostanoids, endothelin receptor antagonists, phosphodiesterase-5 inhibitors, sGC stimulators) have not been sufficiently investigated in other forms of PH. Therefore, the European Guidelines do not recommend the use of these drugs in patients with chronic lung disease and PH...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760450/-pulmonary-hypertension-associated-with-left-heart-disease-recommendations-of-the-cologne-consensus-conference-2016
#14
S Rosenkranz, I M Lang, R Blindt, D Bonderman, L Bruch, G P Diller, R Felgendreher, C Gerges, W Hohenforst-Schmidt, S Holt, C Jung, I Kindermann, T Kramer, W M Kübler, V Mitrovic, A Riedel, A Rieth, A Schmeisser, R Wachter, J Weil, C Opitz
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. While the guidelines contain detailed recommendations regarding pulmonary arterial hypertension (PAH), they contain only a relatively short paragraph on other, much more common forms of PH such as PH due to left heart disease. Despite the lack of data, targeted PAH treatments are increasingly being used for PH associated with left heart disease. This development is of concern because of limited ressources and the need to base treatments on scientific evidence...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760449/-decompensated-right-heart-failure-intensive-care-and-perioperative-management-in-patients-with-pulmonary-hypertension
#15
K M Olsson, M Halank, B Egenlauf, D Fistera, H Gall, C Kaehler, K Kortmann, T Kramm, M Lichtblau, A Marra, C Nagel, A Sablotzki, H-J Seyfarth, D Schranz, S Ulrich, M M Hoeper, T J Lange
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760448/-targeted-therapy-of-pulmonary-arterial-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#16
M M Hoeper, C Apitz, E Grünig, M Halank, R Ewert, H Kaemmerer, H-J Kabitz, C Kähler, H Klose, H Leuchte, S Ulrich, K M Olsson, O Distler, S Rosenkranz, H A Ghofrani
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2016, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760447/-general-and-supportive-therapy-of-pulmonary-arterial-hypertension
#17
E Grünig, N Benjamin, U Krüger, H Kaemmerer, S Harutyunova, K M Olsson, S Ulrich, F Gerhardt, C Neurohr, A Sablotzki, M Halank, H-J Kabitz, G Thimm, K-G Fliegel, H Klose
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed recommendations for the targeted and supportive treatment of pulmonary arterial hypertension (PAH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760446/-risk-stratification-and-follow-up-assessment-of-patients-with-pulmonary-arterial-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#18
H H Leuchte, H Ten Freyhaus, H Gall, M Halank, M M Hoeper, H Kaemmerer, C Kähler, G Riemekasten, S Ulrich, M Schwaiblmair, R Ewert
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension are also valid for Germany. The guidelines contain detailed information about the diagnosis of pulmonary hypertension, and furthermore provide novel recommendations for risk stratification and follow-up assessments. However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27760444/-pathobiology-pathology-and-genetics-of-pulmonary-hypertension-recommendations-of-the-cologne-consensus-conference-2016
#19
A Olschewski, E M Berghausen, C A Eichstaedt, B K Fleischmann, E Grünig, G Grünig, G Hansmann, L Harbaum, J K Hennigs, D Jonigk, W M Kübler, G Kwapiszewska, S S Pullamsetti, E Stacher, N Weissmann, D Wenzel, R T Schermuly
The 2015 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension (PH) are also valid for Germany. While the guidelines contain detailed recommendations regarding clinical aspects of pulmonary arterial hypertension (PAH) and other forms of PH, they contain only a relatively short paragraph on novel findings on the pathobiology, pathology, and genetics. However, these are of great importance for our understanding of this complex disease both from a clinical and scientific point of view, and they are essential for the development of novel treatment strategies...
October 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27729606/-pediatric-iatrogenic-vertebral-arteriovenous-fistula-successfully-treated-with-endovascular-treatment-a-case-report
#20
Rintaro Yokoyama, Satoshi Iihoshi, Kei Miyata, Kentaro Toyama, Katsuya Komatsu, Masahiko Wanibuchi, Nobuhiro Mikuni
OBJECT: We report a relatively rare case of pediatric iatrogenic extradural vertebral arteriovenous fistula. CASE: A five-year-old boy with a history of multiple surgeries for Total Anomalous Pulmonary Venous Return(TAPVR)exhibited vascular engorgement of his right neck from about 4 years old. We thought that this was caused by his past operations. He demonstrated swollen blood vessels in the right neck and pulmonary hypertension with increased right heart load...
October 2016: No Shinkei Geka. Neurological Surgery
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