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Pulmonary hypertension in children

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https://www.readbyqxmd.com/read/28791823/echocardiographic-characteristics-of-pulmonary-arterial-hypertension-in-children-with-horizontally-transmitted-hiv
#1
Anca Meda Georgescu, Cosmin Moldovan, Janos Szederjesi, Dan Georgescu, Leonard Azamfirei
BACKGROUND: The prevalence of primitive pulmonary arterial hypertension (PAH) in patients with human immunodeficiency virus infection (HIV) is estimated at approximately 0.5%, significantly higher than in the general population. OBJECTIVES: This study aimed to assess the echocardiographic modifications in HIV-associated pulmonary arterial hypertension (PAH). MATERIAL AND METHODS: A group of 117 patients, aged under 16, with horizontally transmitted HIV staged according to the U...
May 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28791664/pharmacokinetics-of-sildenafil-in-children-with-pulmonary-arterial-hypertension
#2
Hugo Juárez Olguín, Hector Osnaya Martínez, Carmen Flores Pérez, Blanca Ramírez Mendiola, Liliana Rivera Espinosa, Juan Luis Chávez Pacheco, Janett Flores Pérez, Ignacio Mora Magaña
BACKGROUND: Recently, sildenafil was introduced to treat pulmonary arterial hypertension (PAH); however, there are currently few studies on the pharmacokinetics of sildenalfil in children. Therefore, we aimed to carry out a pharmacokinetic study of sildenafil in children with PAH using a single dose. METHODS: Twelve children diagnosed with PAH, consisting of with ten males and two females, were recruited for the study after obtaining written consent from their parents or guardians...
August 8, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28756079/-exercise-testing-new-guidelines
#3
Dany Michel Marcadet
The GERS (Exercise Rehabilitation and Sports Group of the French Society of Cardiology) has decided to update current guidelines regarding the practice of EKG stress tests. Since the last update dates from 1997, the GERS judged it necessary to integrate data from new works and advancements made in the last 20 years. Good clinical practices and safety conditions are better defined regarding the structure, location, material, staff competency, as well as convention with hospital structures. The diagnosis of coronary artery disease remains the principal indication for a stress test...
July 26, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28755186/liver-transplantation-for-hereditary-tyrosinaemia-type-1-in-the-united-kingdom
#4
Patrick McKiernan
Fourteen children have undergone liver transplantation for hereditary tyrosinaemia type 1 (HT1) at Birmingham Children's hospital (BCH) since 1989; six were treated prior to the availability of Nitisinone in 1993 and eight in the post Nitisinone era. Prior to 1993 essentially all children with HT1 were referred for transplantation. In the Nitisinone era only those with unresponsive liver failure or suspected malignancy were considered for transplantation. Those who were treated pre-emptively following newborn screening have no evidence of liver disease and none have required transplantation...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28745124/dysfunction-of-the-foetal-arterial-duct-results-in-a-wide-spectrum-of-cardiovascular-pathology
#5
Marc Gewillig, Stephen C Brown, Mieke Roggen, Benedicte Eyskens, Ruth Heying, Patrice Givron, Bjorn Cools, Luc de Catte
OBJECTIVE: Foetal ductal problems may have various cardiopulmonary consequences. This study aimed to identify the spectrum of ductus arteriosus (DA) dysfunction (closure, constriction, kinking, aneurysm and thrombosis) and the resultant clinical and echocardiographic presentation in foetuses and neonates. METHODS AND RESULTS: This is a retrospective analysis of serial pre- and post-natal data of 27 cases of foetal ductal dysfunction diagnosed at a median gestational age of 33 weeks (range 20-39)...
July 26, 2017: Acta Cardiologica
https://www.readbyqxmd.com/read/28737730/sildenafil-in-infants-and-children
#6
REVIEW
Larisa Simonca, Robert Tulloh
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. This might apply to persistent pulmonary hypertension of the newborn (PPHN) or bronchopulmonary dysplasia, as well as to more complex diseases, such as idiopathic pulmonary hypertension...
July 24, 2017: Children
https://www.readbyqxmd.com/read/28714608/selective-serotonin-reuptake-inhibitors-during-pregnancy-do-we-have-now-more-definite-answers-related-to-prenatal-exposure
#7
REVIEW
Asher Ornoy, Gideon Koren
Despite extensive studies, there still seems to be uncertainty as to the possible reproductive risk of selective serotonin reuptake inhibitors (SSRIs) and selective serotonin norepinephrine reuptake inhibitors (SNRIs) in pregnancy. We, therefore, assess the current data on the risk/benefit of SSRI use in pregnancy. As the neurodevelopmental effects of SSRIs are discussed in another paper in this issue, we will not address the possible neurodevelopmental effects. Special emphasis is given to the newer, large population-based studies...
July 17, 2017: Birth defects research
https://www.readbyqxmd.com/read/28704835/high-prevalence-of-pulmonary-hypertension-complicates-the-care-of-infants-with-omphalocele
#8
Shandee Hutson, Joanne Baerg, Douglas Deming, Shawn D St Peter, Andrew Hopper, Donna A Goff
BACKGROUND: Omphalocele is one of the most common abdominal wall defects. Many newborn infants born with omphalocele present with significant respiratory distress at birth, requiring mechanical ventilatory support, and have clinical evidence of pulmonary hypertension. Little information exists on the prevalence of and risk factors associated with pulmonary hypertension in this cohort of infants. OBJECTIVES: To describe the prevalence of and risk factors associated with pulmonary hypertension among infants with omphalocele...
July 14, 2017: Neonatology
https://www.readbyqxmd.com/read/28697724/early-characteristics-of-infants-with-pulmonary-hypertension-in-a-referral-neonatal-intensive-care-unit
#9
Shilpa Vyas-Read, Usama Kanaan, Prabhu Shankar, Jane Stremming, Curtis Travers, David P Carlton, Anne Fitzpatrick
BACKGROUND: Approximately 8-23% of premature infants develop pulmonary hypertension (PH), and this diagnosis confers a higher possibility of mortality. As a result, professional societies recommend PH screening in premature infants. However, the risk factors for and the outcomes of PH may differ depending on the timing of its diagnosis, and little evidence is available to determine at-risk infants in the referral neonatal population. The objective of this study was to define clinical and echocardiographic characteristics of infants with pulmonary hypertension during the neonatal hospital course and at or near-term...
July 11, 2017: BMC Pediatrics
https://www.readbyqxmd.com/read/28681131/hemodynamic-evaluation-of-children-with-persistent-or-recurrent-pulmonary-arterial-hypertension-following-complete-repair-of-congenital-heart-disease
#10
Heiner Latus, Inken Wagner, Stefan Ostermayer, Gunter Kerst, Joachim Kreuder, Dietmar Schranz, Christian Apitz
Persistent or recurrent pulmonary arterial hypertension (PAH) following complete surgical repair of congenital heart disease (CHD) represents one of the largest group of PAH associated with CHD (PAH-CHD) in recent registry studies and seems to have a particularly poor prognosis. However, little is known about this fourth clinical subclass of PAH-CHD, especially in children. The purpose of this study was to assess specific characteristics of invasive hemodynamics of this disease in children, including acute vasodilator testing (AVT) and pulmonary endothelial function (PEF) and to compare to patients with idiopathic PAH (IPAH), who usually present with a similar fatal clinical course...
July 5, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28680572/pulmonary-arterial-hypertension-in-the-usa-an-epidemiological-study-in-a-large-insured-pediatric-population
#11
Lin Li, Susan Jick, Stefanie Breitenstein, Gemzel Hernandez, Alexander Michel, David Vizcaya
Pulmonary arterial hypertension (PAH) is rare in children and few data are available in a pediatric general population. This study aims to calculate the annual incidence and prevalence of PAH and to describe these children in a large US population of patients aged under 18 years. Using the US MarketScan claims database we identified 695 children with PAH in 2010-2013. We calculated annual incidence rates and prevalence overall, by age and PAH type (idiopathic and non-idiopathic) using Byar's method. We also described characteristics, co-morbidities, treatment patterns, and diagnostic procedures for these children...
March 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28679172/potts-shunt-in-children-with-pulmonary-arterial-hypertension-institutional-experience
#12
Sergey V Gorbachevsky, Anton A Shmalts, Irina Y Barishnikova, Sergey B Zaets
OBJECTIVES: Since 2004, different techniques for the Potts shunt have been used in patients with incurable pulmonary arterial hypertension. However, the experience with these interventions in a single institution is limited. We present our series of Potts shunts in children with pulmonary arterial hypertension. METHODS: Eight patients with suprasystemic pulmonary arterial hypertension (pulmonary artery-to-aorta mean pressure ratio of 1.39 ± 0.30) had a Potts shunt at the median age of 13...
June 29, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28676038/efficacy-and-safety-of-oral-sildenafil-in-children-with-down-syndrome-and-pulmonary-hypertension
#13
Maurice Beghetti, Andrzej Rudzinski, Min Zhang
BACKGROUND: Despite the increased risk for pulmonary hypertension in children with Down syndrome, the response to treatment with targeted therapies for pulmonary hypertension in these patients is not well characterized. The Sildenafil in Treatment-naive children, Aged 1-17 years, with pulmonary arterial hypertension (STARTS-1) trial was a dose-ranging study of the short-term efficacy and safety of oral sildenafil in children with pulmonary arterial hypertension. We assessed the safety and efficacy of oral sildenafil in children with Down syndrome and pulmonary arterial hypertension...
July 4, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28661905/genetics-of-pulmonary-hypertension-in-the-clinic
#14
Barbara Girerd, Edmund Lau, David Montani, Marc Humbert
PURPOSE OF REVIEW: Heritable pulmonary arterial hypertension (PAH) is an autosomal dominant disease with incomplete penetrance because of mutations in bone morphogenetic protein receptor-II (BMPR2), activin A receptor type II-like kinase 1, endoglin, caveolin-1, potassium channel subfamily K, member 3, and T-box gene 4 genes. Heritable pulmonary veno-occlusive disease and/or pulmonary capillary hemangiomatosis (PVOD/PCH) is an autosomal recessive disease because of biallelic mutations in the eukaryotic translation initiation factor 2 alpha kinase 4 gene...
September 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28661513/mid-childhood-outcomes-after-pre-viable-preterm-premature-rupture-of-membranes
#15
M H Bentsen, E Satrell, H Reigstad, S L Johnsen, M Vollsæter, O D Røksund, G Greve, A Berg, T Markestad, T Halvorsen
OBJECTIVES: Investigate cardiorespiratory outcomes in children surviving previable preterm premature rupture of membranes (PV-PPROM) before 22 weeks' gestational age (GA) with minimum 2 weeks latency. STUDY DESIGN: Single institution, follow-up of retrospectively identified children who were born after PV-PPROM during 2000-2004, and individually matched preterm-born controls. RESULTS: Eleven PV-PPROM and matched control children were included at mean age of 10...
June 29, 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/28645441/evaluation-and-management-of-pulmonary-hypertension-in-children-with-bronchopulmonary-dysplasia
#16
Usha Krishnan, Jeffrey A Feinstein, Ian Adatia, Eric D Austin, Mary P Mullen, Rachel K Hopper, Brian Hanna, Lew Romer, Roberta L Keller, Jeffrey Fineman, Robin Steinhorn, John P Kinsella, D Dunbar Ivy, Erika Berman Rosenzweig, Usha Raj, Tilman Humpl, Steven H Abman
No abstract text is available yet for this article.
June 20, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28639216/biocomparison-study-of-adult-and-paediatric-dose-strengths-of-the-prostacyclin-receptor-agonist-selexipag
#17
Margaux Boehler, Shirin Bruderer, Ivan Ulč, Jasper Dingemanse
BACKGROUND AND OBJECTIVES: Selexipag is an oral, non-prostanoid, selective prostacyclin receptor agonist recently marketed for the treatment of pulmonary arterial hypertension (PAH) in adults. Selexipag may also be an effective treatment in children with PAH. The aim of this study was to compare the pharmacokinetics of selexipag and its active metabolite ACT-333679 following single oral administration of one tablet of 200 µg selexipag (Treatment A) vs. 4 paediatric tablets of 50 µg (Treatment B) in healthy adult male subjects...
June 21, 2017: European Journal of Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/28633267/perventricular-implantation-of-melody-valve-in-child-with-pulmonary-hypertension-after-a-potts-shunt
#18
Tomasz Mroczek, Marcin Demkow, Tomasz Moszura, Aleksandra Morka, Janusz Skalski
The Potts operation is a surgical option for the treatment of children with suprasystemic pulmonary arterial hypertension (PAH). Its positive hemodynamic effect may be obliterated by pulmonary valvar insufficiency. We describe a case in which the Potts shunt was followed by perventricular Melody valve implantation in the pulmonary position in a 12-kg child with a previously repaired truncus arteriosus communis (TAC) in whom PAH developed. The combined approach using both procedures significantly improved functional capacity and led to spectacular reverse remodeling of the right ventricle seen at 2-year follow-up...
July 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28633041/study-of-platelet-activation-hypercoagulable-state-and-the-association-with-pulmonary-hypertension-in-children-with-%C3%AE-thalassemia
#19
REVIEW
Mahmoud Alhosiny Fayed, Hesham El-Sayed Abdel-Hady, Mona Mohammed Hafez, Osama Saad Salama, Youssef Abdelhalim Al-Tonbary
BACKGROUND: The increased survival rate of thalassemic patients has led to unmasking of management related complications which were infrequently encountered. OBJECTIVE: Study the increased coagulation and platelet activation in children with β-thalassemia, to analyze the factors that lead to such hypercoagulable state and to study pulmonary hypertension (PH) in conjunction with platelet activation and hypercoagulable state in children with β-thalassemia. METHODS: 36 Egyptian children with β-thalassemia with a mean age of 9...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28632754/permissive-hypercapnia-for-severe-acute-respiratory-distress-syndrome-in-immunocompromised-children-a-single-center-experience
#20
Hans Fuchs, Nicola Rossmann, Manuel B Schmid, Manfred Hoenig, Ulrich Thome, Benjamin Mayer, Daniel Klotz, Helmut D Hummler
BACKGROUND: Controlled hypoventilation while accepting hypercapnia has been advocated to reduce ventilator-induced lung injury. The aim of the study was to analyze outcomes of a cohort of immunocompromised children with acute respiratory distress syndrome (ARDS) ventilated with a strategy of stepwise increasing PCO2 targets up to 140 mm Hg. METHODS: Retrospective analysis of outcomes of a cohort of children with oncologic disease or after stem cell transplantation and severe respiratory failure in comparison with a historical control cohort...
2017: PloS One
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