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Pediatric pulmonary arterial hypertension

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https://www.readbyqxmd.com/read/28590293/pediatric-pulmonary-arterial-hypertension-on-the-eve-of-growing-up
#1
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/28548215/protein-z-and-endothelin-1-genetic-polymorphisms-in-pediatric-egyptian-sickle-cell-disease-patients
#2
Mervat M Khorshied, Nohair S Mohamed, Rania S Hamza, Rasha M Ali, Mona K El-Ghamrawy
BACKGROUND: Sickle cell disease (SCD) is a monogenic disease associated with multisystem morbidity. Vasculopathy caused by delicate imbalance between coagulation and endothelial systems plays a pivotal role in disease course. As Protein Z and Endothelin-1 genetic polymorphisms may increase the thrombotic risk, the aim of the current work was to verify the possible impact of Protein Z (PROZ G79A) and Endothelin-1 (EDN1 G5665T) polymorphisms on the clinic-laboratory features of the SCD in a cohort of Egyptian pediatric patients...
May 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28506578/-congenital-heart-disease-in-adolescents-and-adults-management-in-a-general-cardiology-department-in-senegal
#3
A Mbaye, M Bodian, A A Ngaïdé, H Abdourafiq, M C B O Leye, S Savodogo, F Aw, M Ndiaye, I Kouamé, K Babaka, M Dioum, N D Gaye, S A Sarr, M B Ndiaye, A D Kane, A Kane
BACKGROUND: Congenital heart diseases in adults include malformations treated in childhood that decompensate secondarily and those asymptomatic at birth, appear later. This study aims to identify congenital heart diseases in adults in general cardiology department of Senegal and to assess clinical presentations, treatment and outcomes. METHODS: We conducted a cross-sectional and descriptive study based on the records of patients aged at least 16 years and followed for congenital heart disease in the cardiology department of the General Hospital of Grand-Yoff in Dakar between May 2003 and March 2015...
May 12, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28475298/a-modified-noninvasive-screening-protocol-for-pulmonary-hypertension-in-children-with-sickle-cell-disease-who-should-be-sent-for-invasive-evaluation
#4
Christian Lilje, Jordan Harry, Kelly K Gajewski, Renee V Gardner
BACKGROUND: Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. OBJECTIVES: We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies...
May 5, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28425226/a-review-of-pediatric-pulmonary-hypertension-with-new-guidelines
#5
Serdar Kula, Ayhan Pektaş
This study aims to review pediatric pulmonary hypertension (PH) by comparing the guidelines of the European Society of Cardiology (ESC)/European Respiratory Society (ERS), the American Heart Association (AHA)/American Thoracic Society (ATS), and the European Pediatric Pulmonary Vascular Disease Network (EPPVDN). All three sets of guidelines define PH as having a mean pulmonary artery pressure of ≥25 mmHg and accept the validity of the World Health Organization (WHO) classification system. Every child with a high index of suspicion for PH should undergo an initial work-up of chest X-rays, electrocardiography, and echocardiography...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28375639/prognostic-significance-of-reduced-blood-pressure-response-to-exercise-in-pediatric-pulmonary-arterial-hypertension
#6
Hong-Da Zhang, Zi-Chao Lv, Li-Ting Wang, Alexander Rothman, Tian-Yu Lian, Yang-Yang He, Yan Wu, Allan Lawrie, Maurice Beghetti, Zhi-Cheng Jing
No abstract text is available yet for this article.
April 4, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28296782/the-safety-and-efficacy-of-dexmedetomidine-remifentanil-in-children-undergoing-flexible-bronchoscopy-a-retrospective-dose-finding-trial
#7
Xia Li, Xue Wang, Shuguang Jin, Dongsheng Zhang, Yanuo Li
Flexible bronchoscopy is more and more used for diagnosis and management of various pulmonary diseases in pediatrics. As poor coordination of children, the procedure is usually performed under general anesthesia with spontaneous or controlled ventilation to increase children and bronchoscopists' safety and comfort. Previous studies have reported that dexmedetomidine (DEX) could be safely and effectively used for flexible bronchoscopy in both adulate and children. However, there is no trial to evaluate the dose-finding of safety and efficacy of dexmedetomidine-remifentanil (DEX-RF) in children undergoing flexible bronchoscopy...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28193613/apparent-aortic-stiffness-in-children-with-pulmonary-arterial-hypertension-existence-of-vascular-interdependency
#8
Michal Schäfer, D Dunbar Ivy, Steven H Abman, Alex J Barker, Lorna P Browne, Brian Fonseca, Vitaly Kheyfets, Kendall S Hunter, Uyen Truong
BACKGROUND: Left ventricular dysfunction, mediated by ventricular interdependence, has been associated with negative outcomes in children with pulmonary arterial hypertension (PAH). Considering the dilation of the pulmonary arteries as a paramount sign of PAH, we hypothesized that the ascending aorta will present signs of apparent stiffness in children with PAH and that this effect may be because of mechanical interaction with the dilated main pulmonary artery (MPA). METHODS AND RESULTS: Forty-two children with PAH and 26 age- and size-matched controls underwent comprehensive cardiac magnetic resonance evaluation...
February 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28185814/obstructive-sleep-apnea-and-pulmonary-hypertension-in-children
#9
REVIEW
David G Ingram, Alvin V Singh, Zarmina Ehsan, Brian F Birnbaum
Obstructive sleep apnea (OSA) is a common pediatric breathing disorder, affecting 1-5% of all children. Pulmonary hypertension (PH), a severe complication of OSA, is associated with significant morbidity and mortality. Despite this important relationship between OSA and PH, there is sparse literature addressing this subject in children. This review will examine the putative relationship between OSA and PH, synthesize the available literature in children, and suggest a reasonable approach, despite limited data, for clinicians...
January 11, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28185800/inhaled-sildenafil-as-an-alternative-to-oral-sildenafil-in-the-treatment-of-pulmonary-arterial-hypertension-pah
#10
Jahidur Rashid, Brijeshkumar Patel, Eva Nozik-Grayck, Ivan F McMurtry, Kurt R Stenmark, Fakhrul Ahsan
The practice of treating PAH patients with oral or intravenous sildenafil suffers from the limitations of short dosing intervals, peripheral vasodilation, unwanted side effects, and restricted use in pediatric patients. In this study, we sought to test the hypothesis that inhalable poly(lactic-co-glycolic acid) (PLGA) particles of sildenafil prolong the release of the drug, produce pulmonary specific vasodilation, reduce the systemic exposure of the drug, and may be used as an alternative to oral sildenafil in the treatment of PAH...
March 28, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28178426/physical-activity-in-pediatric-pulmonary-arterial-hypertension-measured-by-accelerometry-a-candidate-clinical-endpoint
#11
Willemijn M H Zijlstra, Mark-Jan Ploegstra, Theresia Vissia-Kazemier, Marcus T R Roofthooft, Gideon du Marchie Sarvaas, Beatrijs Bartelds, Annette Rackowitz, Freek van den Heuvel, Hans L Hillege, Guy Plasqui, Rolf M F Berger
Rationale- The development of evidence-based treatment guidelines for pediatric pulmonary arterial hypertension (PAH) is hampered by lack of pediatric clinical trials. Trial-design is hampered by lack of a feasible clinical endpoint in this population. Objectives- To evaluate the use of accelerometry for measuring physical activity (PA) in pediatric PAH and to investigate its correlation with clinical disease severity markers. Methods- We included children from the Dutch National Network for Pediatric Pulmonary Hypertension...
February 8, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28145758/serially-measured-uric-acid-levels-predict-disease-severity-and-outcome-in-pediatric-pulmonary-arterial-hypertension
#12
Lynn J Leberkühne, Mark-Jan Ploegstra, Johannes M Douwes, Beatrijs Bartelds, Marcus T R Roofthooft, Hans L Hillege, Rolf M F Berger
No abstract text is available yet for this article.
February 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#13
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
https://www.readbyqxmd.com/read/28101803/lung-retransplantation-in-an-adult-13%C3%A2-years-after-single-lobar-transplant-in-childhood
#14
Seiichiro Sugimoto, Shinji Otani, Takashi Ohki, Takeshi Kurosaki, Kentaroh Miyoshi, Masaomi Yamane, Shinichiro Miyoshi, Takahiro Oto
Single living-donor lobar lung transplantation provides acceptable results for critically ill children; however, an additional lung transplantation may be required in the future as the recipient grows. We describe a case of successful lung retransplantation in a grown-up patient after single lobar lung transplantation in childhood. A 23-year-old man underwent bilateral cadaveric lung retransplantation for chronic lung allograft dysfunction 13 years after right single living-donor lobar transplantation for idiopathic pulmonary arterial hypertension performed at the age of 10 years...
January 18, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#15
Julius Koifman, John Granton, John Thenganatt
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. Presentation is most common among the pediatric population, and cases in adults are rare. The number of nonhematologic presentations described in relation to HLH has been growing. We present a case involving a woman who developed HLH after autologous stem cell transplantation for mantle cell lymphoma. Months later, she received a diagnosis of pulmonary arterial hypertension (PAH) while undergoing treatment for her HLH...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28078552/pediatric-development-of-bosentan-facilitated-by-modeling-and-simulation
#16
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...
April 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
#17
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
#18
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
#19
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
#20
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...
March 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
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