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https://www.readbyqxmd.com/read/28912187/pediatric-cardiomyopathies
#1
REVIEW
Teresa M Lee, Daphne T Hsu, Paul Kantor, Jeffrey A Towbin, Stephanie M Ware, Steven D Colan, Wendy K Chung, John L Jefferies, Joseph W Rossano, Chesney D Castleberry, Linda J Addonizio, Ashwin K Lal, Jacqueline M Lamour, Erin M Miller, Philip T Thrush, Jason D Czachor, Hiedy Razoky, Ashley Hill, Steven E Lipshultz
Pediatric cardiomyopathies are rare diseases with an annual incidence of 1.1 to 1.5 per 100 000. Dilated and hypertrophic cardiomyopathies are the most common; restrictive, noncompaction, and mixed cardiomyopathies occur infrequently; and arrhythmogenic right ventricular cardiomyopathy is rare. Pediatric cardiomyopathies can result from coronary artery abnormalities, tachyarrhythmias, exposure to infection or toxins, or secondary to other underlying disorders. Increasingly, the importance of genetic mutations in the pathogenesis of isolated or syndromic pediatric cardiomyopathies is becoming apparent...
September 15, 2017: Circulation Research
https://www.readbyqxmd.com/read/28904266/clinical-impact-of-main-pulmonary-artery-dilatation-on-outcome-in-pediatric-idiopathic-and-heritable-pulmonary-arterial-hypertension
#2
Satoshi Ikehara, Shinichi Takatsuki, Tomotaka Nakayama, Kazuyuki Naoi, Hiroyuki Matsuura, Tsutomu Saji
BACKGROUND: Few studies have investigated the clinical impact of pulmonary artery (PA) dilatation on outcomes in pediatric pulmonary arterial hypertension (PAH).Methods and Results:This study investigated the clinical outcomes of idiopathic or heritable PAH in 66 children aged <18 years at diagnosis. Main PA/thorax (MPA/T) ratio was measured on chest radiography in PAH patients. Patients were divided into 2 groups based on MPA/T ratio, and compared with a control group of 166 age- and gender-matched healthy children...
September 13, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28877973/heritable-pulmonary-hypertension-from-bench-to-bedside
#3
REVIEW
Barbara Girerd, Jason Weatherald, David Montani, Marc Humbert
Mutations in the BMPR2 gene, and more rarely in ACVRL1, endoglin, caveolin-1, KCNK3 and TBX4 genes predispose to heritable pulmonary arterial hypertension, an autosomal dominant disease with incomplete penetrance. Bi-allelic mutations in the EIF2AK4 gene predispose to heritable pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis, an autosomal recessive disease with an unknown penetrance.In France, the national pulmonary hypertension referral centre offers genetic counselling and testing to adults and children...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28843329/safety-and-feasibility-of-the-transcatheter-approach-to-create-a-reverse-potts-shunt-in-children-with-idiopathic-pulmonary-arterial-hypertension
#4
Younes Boudjemline, Aleksander Sizarov, Sophie Malekzadeh-Milani, Cristian Mirabile, Marien Lenoir, Diala Khraiche, Marilyne Lévy, Damien Bonnet
BACKGROUND: The reversed Potts shunt improves right ventricular (RV) function in patients with suprasystemic pulmonary arterial hypertension (PAH). The proximity of the left pulmonary artery (LPA) to the descending aorta (DAo) permits the creation of a transcatheter connection. We sought to assess the safety, feasibility, and hemodynamic efficacy of the transcatheter Potts shunt (TPS) in children. METHODS: The TPS procedure was performed using radiofrequency energy for vessel perforation and deployment of a covered stent to connect the DAo and LPA...
September 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28840735/-risk-factors-for-cardiovascular-diseases-in-the-descendants-of-patients-after-early-myocardial-infarction
#5
Markéta Mateřánková, Petra Karnosová, Jitka Mlíková Seidlerová, Jan Filipovský, Otto Mayer
GOAL: The cardiovascular diseases (CVDs) developing as the result of atherosclerosis are among the most frequent causes of morbidity and mortality within the Czech Republic and elsewhere. Genetic predisposition for cardiovascular diseases is amplified in the presence of routine risk factors which can be influenced. Our aim was to establish whether the level of the risk factors for ICHS already differs in the population of healthy descendants of the patients after early myocardial infarction, as opposed to the control group of examined individuals...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28836059/posterior-reversible-encephalopathy-syndrome-in-children-with-kidney-disease
#6
REVIEW
Cristina Gavrilovici, Ingrith Miron, Luminiţa Voroneanu, Silvia Bădărau, Magdalena Stârcea
Posterior reversible encephalopathy syndrome (PRES) has been described as a neurological condition observed in a variety of clinical settings and is characterized by focal neurological deficits, seizures, headaches, altered mental status, and visual impairment, associated with transient typical lesions on neuroimaging, predominantly in the posterior part of the brain. The most common risk factors for PRES are hypertension, renal diseases, and the use of calcineurin inhibitors. The incidence of PRES in children with renal disorders varies between 4 and 9%, according to different reports...
August 23, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/28825767/clinical-and-parental-status-of-patients-with-congenital-heart-disease-associated-pulmonary-arterial-hypertension
#7
Amiram Nir, Neville Berkman, Sagui Gavri
BACKGROUND: Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity is associated with increased morbidity and mortality. OBJECTIVES: To evaluate the clinical and demographic characteristics of adults with congenital heart diseases (ADCHD) and PAH at a single center. METHODS: A prospective registry of all patients with PAH was conducted between 2009 and 2015. RESULTS: Thirty-two patients were identified...
August 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28819713/plasma-growth-differentiation-factor-15-is-a-potential-biomarker-for-pediatric-pulmonary-arterial-hypertension-associated-with-congenital-heart-disease
#8
Gang Li, Yan Li, Xiao-Qiu Tan, Peng Jia, Jian Zhao, Dong Liu, Ting Wang, Bin Liu
We aimed to investigate plasma growth differentiation factor-15 (GDF-15) levels in pediatric pulmonary arterial hypertension secondary to congenital heart disease (PAH-CHD), and assess the association with hemodynamic parameters. Plasma GDF-15 levels were measured in children with PAH-CHD (n = 46) and compared to children with CHD without PAH (n = 39). Normal individuals (n = 30) served as health control group. Plasma GDF-15 levels were significantly elevated in patients with PAH-CHD compared with those with CHD without PAH (median 1415 ng/L, interquartile range [IQR] 926...
August 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28799999/features-of-cardiovascular-disease-in-low-income-and-middle-income-countries-in-adults-and-children-living-with-hiv
#9
Andrew W McCrary, Chidozie U Nduka, Saverio Stranges, Gerald S Bloomfield
PURPOSE OF REVIEW: The current article addresses crucial issues in identifying risk of cardiovascular disease (CVD) in people living with HIV in low-income and middle-income countries (LMICs). These issues are in need of urgent attention to advance our knowledge and inform actions to mitigate CVD in this population. We address CVDs in adults living with HIV as well as the unique aspects pertaining to children living with HIV (CLHIV), a group sorely under-represented in this field. RECENT FINDINGS: CVDs affecting adults such as hypertension, dyslipidemia, coronary artery disease, and heart failure, in addition to myocardial dysfunction, vascular diseases, and autoimmune phenomena are also being reported in CLHIV...
August 10, 2017: Current Opinion in HIV and AIDS
https://www.readbyqxmd.com/read/28791823/echocardiographic-characteristics-of-pulmonary-arterial-hypertension-in-children-with-horizontally-transmitted-hiv
#10
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
#11
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
#12
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 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28745124/dysfunction-of-the-foetal-arterial-duct-results-in-a-wide-spectrum-of-cardiovascular-pathology
#13
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
#14
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/28717934/why-should-we-screen-for-arterial-hypertension-in-children-and-adolescents
#15
EDITORIAL
Mieczysław Litwin
No abstract text is available yet for this article.
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28716800/relationship-between-vascular-resistance-and-sympathetic-nerve-fiber-density-in-arterial-vessels-in-children-with-sleep-disordered-breathing
#16
Anna Kontos, Kurt Lushington, James Martin, Quenten Schwarz, Ryan Green, David Wabnitz, Xiangjun Xu, Elke M Sokoya, Scott Willoughby, Mathias Baumert, Antonio Ferrante, Melissa La Forgia, Declan Kennedy
BACKGROUND: Sleep disordered breathing in children is associated with increased blood flow velocity and sympathetic overactivity. Sympathetic overactivity results in peripheral vasoconstriction and reduced systemic vascular compliance, which increases blood flow velocity during systole. Augmented blood flow velocity is recognized to promote vascular remodeling. Importantly, increased vascular sympathetic nerve fiber density and innervation in early life plays a key role in the development of early-onset hypertension in animal models...
July 17, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28695435/evaluation-of-development-of-subclinical-atherosclerosis-in-children-with-uveitis
#17
Seçil Conkar, Suzan Güven Yılmaz, İlker Özgür Koska, Afig Berdeli, Sevgi Mir
Uveitis is a chronic inflammatory disease. Chronic inflammation has been shown to have a role in pathogenesis of atherosclerosis. Atherosclerosis is the most important risk factor of cardiovascular diseases and is shown to start as early as childhood. In this study, we investigated the presence of subclinical atherosclerosis in children with uveitis. Seventy five patients who were diagnosed as having uveitis in ophthalmology and pediatric rheumatology clinics were included in the study. Patients with hypertension, obesity, dyslipidemia, diabetes, and with history of early cardiovascular disease were excluded...
July 10, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28693788/fetal-growth-restriction-with-brain-sparing-neurocognitive-and-behavioral-outcomes-at-12-years-of-age
#18
Fenny Beukers, Cornelieke S H Aarnoudse-Moens, Mirjam M van Weissenbruch, Wessel Ganzevoort, Johannes B van Goudoever, Aleid G van Wassenaer-Leemhuis
OBJECTIVE: To study neurocognitive functions and behavior in children with a history of fetal growth restriction (FGR) with brain sparing. We hypothesized that children with FGR would have poorer outcomes on these domains. STUDY DESIGN: Subjects were 12-year-old children with a history of FGR born to mothers with severe early-onset hypertensive pregnancy disorders (n = 96) compared with a normal functioning full term comparison group with a birth weight ≥2500 g (n = 32)...
September 2017: Journal of 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
#19
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
#20
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
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