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Paediatric Pulmonary Hypertension

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https://www.readbyqxmd.com/read/28748509/clinical-characteristics-and-thrombosis-outcomes-of-paediatric-antiphospholipid-syndrome-analysis-of-58-patients
#1
Jingran Ma, Hongmei Song, Min Wei, Yanyan He
The study aims to analyse the clinical and immunological manifestations of paediatric antiphospholipid syndrome (APS) in patients, based on the 2006 revised classification criteria of definite APS. Fifty-eight paediatric patients with APS were enrolled and analysed retrospectively. A total of 37 female and 21 male patients with a mean age of 14 ± 3 years at disease onset were included. Fourteen (24%) cases were primary APS, and 40 (69%) cases were secondary to systemic lupus erythaematosus (SLE). Anti-nuclear antibody (ANA) positivity and hypocomplementemia were more common in secondary APS than in primary APS...
July 26, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28737730/sildenafil-in-infants-and-children
#2
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/28679172/potts-shunt-in-children-with-pulmonary-arterial-hypertension-institutional-experience
#3
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/28639216/biocomparison-study-of-adult-and-paediatric-dose-strengths-of-the-prostacyclin-receptor-agonist-selexipag
#4
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/28619123/chd-and-respiratory-syncytial-virus-global-expert-exchange-recommendations
#5
Robert M R Tulloh, Constancio Medrano-Lopez, Paul A Checchia, Claudia Stapper, Naokata Sumitomo, Matthias Gorenflo, Eun Jung Bae, Antonio Juanico, Juan M Gil-Jaurena, Mei-Hwan Wu, Talal Farha, Ali Dodge-Khatami, Rocky Tsang, Gerard Notario, Colleen Wegzyn
BACKGROUND: Palivizumab is the standard immunoprophylaxis against serious disease due to respiratory syncytial virus infection. Current evidence-based prophylaxis guidelines may not address certain children with CHD within specific high-risk groups or clinical/management settings. METHODS: An international steering committee of clinicians with expertise in paediatric heart disease identified key questions concerning palivizumab administration; in collaboration with an additional international expert faculty, evidence-based recommendations were formulated using a quasi-Delphi consensus methodology...
June 16, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28552078/comparison-of-the-therapeutic-effects-and-side-effects-of-tadalafil-and-sildenafil-after-surgery-in-young-infants-with-pulmonary-arterial-hypertension-due-to-systemic-to-pulmonary-shunts
#6
Mohammad Reza Sabri, Hamid Bigdelian, Mohsen Hosseinzadeh, Alireza Ahmadi, Mehdi Ghaderian, Mohammad Shoja
Young children with CHD and large systemic-to-pulmonary shunts eventually develop pulmonary hypertension. At present, phosphodiesterase type-5 inhibitors such as sildenafil have been used to control pulmonary pressure before and after cardiac surgery. Recently, tadalafil has been utilised in older children with similar efficacy, but it has been used to a lesser extent in young infants. From April, 2015 to June, 2016, 42 patients aged 3-24 months with a large septal defect and pulmonary arterial hypertension were randomly divided into two equal groups: one group received oral sildenafil (1-3 mg/kg/day every 8 hours), whereas the other group received oral tadalafil (1 mg/kg once a day) from 7-10 days before surgery to 3-4 weeks after surgery...
May 29, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28523023/surgical-repair-of-partial-atrioventricular-septal-defect
#7
Tariq Waqar, Muhammad Usman Riaz, Muhammad Shuaib
OBJECTIVE: To review the results of surgical correction of partial atrioventricular septal defect and associated cardiac comorbidities. METHODS: Retrospective case analysis of electronic database of department of paediatrics cardiac surgery, CPEIC, Multan was done. Forty consecutive patients operated for partial atrioventricular septal defect repair from September 2011 to October 2016 were included. Mean age was 14.67±7.96 years. 60% (24) patients were male. Regarding echocardiographic findings, pre-operatively 40% (n=16) had mild, 47...
March 2017: Pakistan Journal of Medical Sciences Quarterly
https://www.readbyqxmd.com/read/28506331/glucocorticoids-for-treating-paediatric-pulmonary-hypertension-a-novel-use-for-a-common-medication
#8
Manish Aggarwal, Ronald Mark Grady
Laboratory investigations have shown the role of inflammation in the pathogenesis of pulmonary hypertension and improvement after anti-inflammatory drugs. Despite these observations, reports on the use of steroids to treat pulmonary hypertension in humans are absent from the literature. In this article, we report the use of glucocorticoids in the treatment of two children with pulmonary hypertension, demonstrating its potential utility.
September 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28484313/management-of-cardiac-involvement-in-muscular-dystrophies-paediatric-versus-adult-forms
#9
REVIEW
Alberto Palladino, Paola D'Ambrosio, Andrea Antonio Papa, Roberta Petillo, Chiara Orsini, Marianna Scutifero, Gerardo Nigro, Luisa Politano
Muscular dystrophies are a group of genetic disorders characterized by muscle degeneration and consequent substitution by fat and fibrous tissue. Cardiac involvement is an almost constant feature in a great part of these diseases, as both primary myocardial involvement and secondary involvement due to respiratory insufficiency, pulmonary hypertension or reduced mobility. Primary myocardial involvement usually begins more precociously compared to the secondary involvement. In fact the first signs of cardiomyopathy can be observed in the first decade of life in muscular dystrophies with childhood onset and later in adult form of muscular dystrophies as myotonic dystrophy type 1...
December 2016: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/28408467/isolated-mild-sleep-associated-hypoventilation-in-children-with-down-syndrome
#10
Wai Wong, Dennis Rosen
INTRODUCTION: Children with Down syndrome (DS) have a high incidence of obstructive sleep apnea (OSA) that is often associated with hypoventilation. Little is known, however, about the prevalence of sleep-associated hypoventilation independent of OSA in these children. METHODS: Retrospective chart review of all children with DS under 18 years of age undergoing polysomnography at a tertiary care paediatric hospital during a 2-year period. Exclusion criteria were as follow: those requiring oxygen or positive-pressure ventilation; with tracheostomy, baseline hypoxia, unrepaired cyanotic heart disease, pulmonary hypertension, and cerebral palsy; or OSA with >5 obstructions/hour...
September 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28366184/the-role-of-inflammatory-biomarkers-in-chd-associated-pulmonary-hypertension-in-children
#11
Gokcen Oz-Tuncer, Rana Olgunturk, Ayhan Pektas, Erman Cilsal, Serdar Kula, Deniz A Oguz, Sedef F Tunaoglu, Ozge T Pasaoglu, Hatice Pasaoglu
OBJECTIVE: The present study aims to identify the role of inflammatory markers such as C-reactive protein, interleukin-6, and fractalkine in CHD-associated pulmonary hypertension in children. METHODS: This is a prospective review of 37 children with CHD-related pulmonary hypertension, 21 children with congenital heart defects, and 22 healthy children. RESULTS: Serum C-reactive protein and interleukin-6 levels were significantly higher in the children with CHD-related pulmonary hypertension (respectively, p=0...
March 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28213957/a-bosentan-pharmacokinetic-study-to-investigate-dosing-regimens-in-paediatric-patients-with-pulmonary-arterial-hypertension-future-3
#12
Rolf M F Berger, Martine Gehin, Maurice Beghetti, Dunbar Ivy, Andjela Kusic-Pajic, Peter Cornelisse, Simon Grill, Damien Bonnet
AIM: The aim of the present study was to investigate whether increasing the bosentan dosing frequency from 2 mg kg(-1) twice daily (b.i.d.) to 2 mg kg(-1) three times daily (t.i.d.) in children with pulmonary arterial hypertension (PAH) (from ≥3 months to <12 years of age) would increase exposure. METHODS: An open-label, prospective, randomized, multicentre, multiple-dose, phase III study was conducted. Patients (n = 64) were randomized 1:1 to receive oral doses of bosentan of 2 mg kg(-1) b...
August 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28115472/pulmonary-arterial-hypertension-in-children-after-neonatal-arterial-switch-operation
#13
MULTICENTER STUDY
Willemijn Mh Zijlstra, Ola Elmasry, Shari Pepplinkhuizen, D Dunbar Ivy, Damien Bonnet, Paul Luijendijk, Marilyne Lévy, Jose Luis Gavilan, Alba Torrent-Vernetta, Alberto Mendoza, Maria Jesus Del Cerro, Shahin Moledina, Rolf Mf Berger
OBJECTIVES: Paediatric pulmonary arterial hypertension (PAH) after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA) is a clinically recognised entity with an estimated incidence of 0.6%-1.0%. Nevertheless, a clinical characterisation is lacking. We present an international cohort of children with PAH after neonatal ASO for TGA and describe epidemiology and clinical course. METHODS: Data were collected of children with PAH after neonatal ASO (≤6 weeks after birth) for simple TGA without residual shunt defects, identified in four national paediatric PAH networks in Europe and one US referral centre...
August 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28058755/characteristics-of-bordetella-pertussis-infection-among-infantsand-children-admitted-to-paediatric-intensive-care-units-in-greece-a-multicentre-11-year-study
#14
Maria S Kazantzi, Anargyroula Prezerakou, Serafeia N Kalamitsou, Stavroula Ilia, Panagiotis K Kalabalikis, John Papadatos, Maria M Sdougka, George Briassoulis, Maria N Tsolia
AIM: To describe children with pertussis who required intensive care. METHODS: This is a retrospective analysis of pertussis admissions to all (six) national intensive care units in Greece from 2003 to 2013. RESULTS: A total of 31 children were included, 28 of whom were younger than 12 months old. Cough was the most prominent symptom, being present in 27 of 31 (87%) patients, and on admission, only 7 (22.6%) satisfied the case definition...
January 6, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/27995825/treatment-initiation-in-paediatric-pulmonary-hypertension-insights-from-a-multinational-registry
#15
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/27986977/risk-factors-for-postoperative-mortality-in-congenital-diaphragmatic-hernia-a-single-centre-observational-study
#16
Darya Kadir, Helene Engstrand Lilja
BACKGROUND: The management of congenital diaphragmatic hernia (CDH) is a major challenge. The mortality is dependent on associated malformations, the severity of pulmonary hypoplasia, pulmonary hypertension and iatrogenic lung injury associated with aggressive mechanical ventilation. The aims of the study were to investigate the mortality over time in a single paediatric surgical centre, to compare the results with recent reports and to define the risk factors for mortality. METHODS: The medical records of infants with CDH from two time periods: 1995-2005 and 2006-2016 were reviewed...
March 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/27964948/diagnostics-in-children-and-adolescents-with-suspected-or-confirmed-pulmonary-hypertension
#17
REVIEW
Martin Koestenberger, Georg Hansmann, Christian Apitz, Heiner Latus, Astrid Lammers
We provide a practical approach on the initial assessment and diagnostic work-up of children and adolescents with pulmonary hypertension (PH). Transthoracic echocardiography (TTE) often serves as initial study tool before invasive cardiac catheterization. Misinterpretation of TTE variables may lead to missed or delayed diagnosis with devastating consequences, or unnecessary invasive diagnostics that have inherited risks. In addition to clinical and biochemical markers, serial examination of patients with PH using a standardized TTE approach, determining conventional and novel echocardiographic variables, may allow early diagnosis and treatment in paediatric PH...
October 26, 2016: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/27721218/not-for-adults-only-mitraclip-use-in-a-paediatric-patient
#18
Denise C Joffe, Thomas K Jones, Mark Reisman, Elizabeth Perpetua, Yuk Law, Andreas Schuler, G Burkhard Mackensen
We present the primary report of a paediatric patient who had placement of the MitraClip device for severe functional mitral regurgitation. The patient was a 14-year-old boy with symptomatic end-stage non-compaction type cardiomyopathy secondary to a mitochondrial cytopathy. He had severe mitral regurgitation, tricuspid valve regurgitation, a severely dilated LV with apical non-compaction, severe LV dysfunction and severe pulmonary hypertension. Despite optimal medical therapy he developed progressive symptoms of congestive heart failure and he was not a candidate for an assist device or cardiac transplantation...
October 10, 2016: EuroIntervention
https://www.readbyqxmd.com/read/27628296/feasibility-and-utility-of-portable-ultrasound-during-retrieval-of-sick-term-and-late-preterm-infants
#19
Kathryn Browning Carmo, Tracey Lutz, Andrew Berry, Martin Kluckow, Nick Evans
AIM: To determine the role of clinician performed ultrasound (CPU) during the retrieval and transport of critically ill term and near term newborns. METHODS: A neonatologist with portable ultrasound accompanied a sample of newborn retrievals to perform cardiac and cerebral ultrasound before and after transportation. RESULTS: A total of fifty-five babies were studied. Median birthweight: 3350 g (2220-5030 g). CPU led to a change in the planned receiving hospital in ten babies...
December 2016: Acta Paediatrica
https://www.readbyqxmd.com/read/27587546/genetic-analyses-in-a-cohort-of-children-with-pulmonary-hypertension
#20
Marilyne Levy, Mélanie Eyries, Isabelle Szezepanski, Magalie Ladouceur, Sophie Nadaud, Damien Bonnet, Florent Soubrier
The prevalence of germline mutations in paediatric pulmonary hypertension (PH) is poorly documented. The objective of this study was to determine the mutation frequency in PH genes in a paediatric cohort and describe the clinical characteristics of mutation carriers.The study involved 66 index cases with PH: 35 children with idiopathic pulmonary arterial hypertension (IPAH); five children with familial PAH (FPAH); three children with pulmonary veno-occlusive disease (PVOD); and 23 children with PAH associated with congenital heart disease (APAH-CHD)...
October 2016: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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