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Pediatric pulmonary hypertension

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https://www.readbyqxmd.com/read/28338487/the-evolution-of-disease-chronic-lung-disease-of-infancy-and-pulmonary-hypertension
#1
Michael C Tracy, David N Cornfield
PURPOSE OF REVIEW: Bronchopulmonary dysplasia (BPD) or chronic lung disease of infancy BPD was originally described 50 years ago, in 1967 by Northway et al. This article possesses two fundamental objectives to provide: a brief historical perspective on BPD; and an update relative to current notions of epidemiology, pathophysiology, evaluation, and clinical management of BPD complicated by vascular disease. The review highlights areas of consensus and ongoing uncertainty. RECENT FINDINGS: The clinical cause and presentation of infants with BPD has evolved over the past several decades...
March 23, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#2
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/28301910/soluble-fms-like-tyrosine-kinase-1-as-a-link-between-angiogenesis-and-endothelial-dysfunction-in-pediatric-patients-with-%C3%AE-thalassemia-intermedia
#3
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Omneya Ibrahim Youssef, Mohamed ElSayed Ali
Endothelial damage has been implicated in the pathogenesis of vascular complications in β-thalassemia intermedia (β-TI). Soluble fms-like tyrosine kinase 1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. Soluble fms-like tyrosine kinase 1 is an antiangiogenic protein that induces endothelial dysfunction by adhering to and inhibiting VEGF and placenta growth factor. The aim of this study was to assess the level of sFLT-1 in 35 children and adolescents with β-TI, correlating it with markers of hemolysis and iron overload as well as cardiopulmonary complications...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28296782/the-safety-and-efficacy-of-dexmedetomidine-remifentanil-in-children-undergoing-flexible-bronchoscopy-a-retrospective-dose-finding-trial
#4
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/28271596/team-based-approach-to-identify-cardiac-toxicity-in-critically-ill-hematopoietic-stem-cell-transplant-recipients
#5
Christopher E Dandoy, Sonata Jodele, Zachary Paff, Russel Hirsch, Thomas D Ryan, John L Jefferies, Michelle Cash, Seth Rotz, Abigail Pate, Michael D Taylor, Javier El-Bietar, Kasiani C Myers, Gregory Wallace, Adam Nelson, Michael Grimley, Thomas Pfeiffer, Adam Lane, Stella M Davies, Ranjit S Chima
INTRODUCTION: We observed pulmonary hypertension (PH), pericardial effusions, and left ventricular systolic dysfunction (LVSD) in multiple critically ill hematopoietic stem cell transplant (HSCT) recipients. We implemented routine structured echocardiography screening for HSCT recipients admitted to the pediatric intensive care unit (PICU) using a standardized multidisciplinary process. METHODS: HSCT recipients admitted to the PICU with respiratory distress, hypoxia, shock, and complications related to transplant-associated thrombotic microangiopathy were screened on admission and every 1-2 weeks thereafter...
March 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28247848/relation-of-cytokine-profile-to-clinical-and-hemodynamic-features-in-young-patients-with-congenital-heart-disease-and-pulmonary-hypertension
#6
Leína Zorzanelli, Nair Maeda, Mariana Clavé, Ana Thomaz, Filomena Galas, Marlene Rabinovitch, Antonio Lopes
In congenital heart disease, severity of pulmonary hypertension and operability is defined by noninvasive parameters (clinical history, physical examination, and echocardiography) and sometimes, cardiac catheterization. We investigated how circulating levels of inflammatory mediators correlate with such parameters in a young pediatric population (age, 2.0 months to 3.1 years) and the effects of preoperative pulmonary vasodilator therapy with sildenafil. Cytokines were analyzed in serum using chemiluminescence signals...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28246340/tsujii-n-shiraishi-i-kokame-k-et-al-severe-hemolysis-and-pulmonary-hypertension-in-a-neonate-with-upshaw-schulman-syndrome-pediatrics-2016-138-6-e20161565
#7
(no author information available yet)
No abstract text is available yet for this article.
March 2017: Pediatrics
https://www.readbyqxmd.com/read/28235796/pulmonary-hypertension-therapy-and-a-systematic-review-of-efficacy-and-safety-of-pde-5-inhibitors
#8
REVIEW
Chinwe Unegbu, Corina Noje, John D Coulson, Jodi B Segal, Lewis Romer
Pulmonary hypertension (PH) is a syndrome that is of growing concern to pediatricians worldwide. Recent data led to concerns about the safety of phosphodiesterase type 5 (PDE5) inhibitors in children and a US Food and Drug Administration safety advisory. Our objective is to provide insight into therapies for PH in children and to systematically review the comparative effectiveness and safety of PDE5 inhibitors in the management of pediatric patients with PH. We searched the following databases through February 2015: Medline, Embase, SCOPUS, and the Cochrane Central Register of Controlled Trials...
March 2017: Pediatrics
https://www.readbyqxmd.com/read/28193613/apparent-aortic-stiffness-in-children-with-pulmonary-arterial-hypertension-existence-of-vascular-interdependency
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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/28105408/predictors-of-prolonged-mechanical-ventilation-in-pediatric-patients-after-cardiac-surgery-for-congenital-heart-disease
#15
Avisa Tabib, Seyed Ehsan Abrishami, Mohammad Mahdavi, Hojjat Mortezaeian, Ziae Totonchi
BACKGROUND: The duration of mechanical ventilation (MV) is one of the most important clinical factors which predict outcomes in pediatric cardiac surgery. The prolonged mechanical ventilation (PMV) following cardiac surgery is a multifactorial phenomenon and there are conflicts regarding its predictors in pediatric population between different centers. OBJECTIVES: The current study aimed to describe PMV predictors in patients undergoing cardiac surgery for congenital heart disease in a tertiary center for pediatric cardiovascular diseases in Iran...
August 2016: Research in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28103851/cystic-fibrosis-physicians-perspectives-on-the-timing-of-referral-for-lung-transplant-evaluation-a-survey-of-physicians-in-the-united-states
#16
Kathleen J Ramos, Ranjani Somayaji, Erika D Lease, Christopher H Goss, Moira L Aitken
BACKGROUND: Prior studies reveal that a significant proportion of patients with cystic fibrosis (CF) and advanced lung disease are not referred for lung transplant (LTx) evaluation. We sought to assess expert CF physician perspectives on the timing of LTx referral and investigate their LTx knowledge. METHODS: We developed an online anonymous survey that was distributed by the Cystic Fibrosis Foundation (CFF) to the medical directors of all CFF-accredited care centers in the United States in 2015...
January 19, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28101803/lung-retransplantation-in-an-adult-13%C3%A2-years-after-single-lobar-transplant-in-childhood
#17
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/28094679/a-retrospective-analysis-of-a-pediatric-tele-echocardiography-service-to-treat-triage-and-reduce-trans-pacific-transport
#18
Christopher A Rouse, Brandon T Woods, C Becket Mahnke
Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28090306/a-case-of-pulmonary-arterial-hypertension-associated-with-adult-hemophagocytic-lymphohistiocytosis
#19
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
#20
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
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