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Children pulmonary hypertension

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https://www.readbyqxmd.com/read/28910308/pulmonary-hypertension-among-5-to-18-year-old-children-with-sickle-cell-anaemia-in-nigeria
#1
Ogochukwu J Sokunbi, Ekanem N Ekure, Edamisan O Temiye, Roosevelt Anyanwu, Christy A N Okoromah
BACKGROUND: Pulmonary hypertension (PHT) is a significant cause of mortality in patients with sickle cell disease (SCD). Few studies on PHT in SCD have been carried out in children. This study aimed to estimate the prevalence of PHT in children with sickle cell anaemia (SCA) and determine its clinical and laboratory correlates. METHODS: In this cross sectional study, evaluation involved obtaining bio-data, history and physical examination findings in 175 SCA subjects with haemoglobin genotype SS aged 5 to 18 years and 175 age and sex matched controls with haemoglobin genotype AA...
2017: PloS One
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/28879059/pulmonary-hypertension-and-cardiac-hypertrophy-in-children-recipients-of-orthotopic-living-related-liver-transplantation
#3
Magd A Kotb, Inas Abd El Satar, Ahmed M Badr, Nancy H Anis, Hoda Abd El Rahman Ismail, Alaa F Hamza, Hesham M Abdelkader
Surgical stress, liberation of cytokines associated with re-perfusion injury, and long standing use of immune suppressive medications in children recipients of orthotopic living related liver transplantation (OLRLT) pose cardiovascular risk. Reported cardiovascular adverse effects vary from left ventricular wall thickening, hypertrophic cardiomyopathy to resting ECG abnormalities, asymptomatic ST depression following increased heart rate and ventricular arrhythmias. Twenty-five consecutive children recipients of OLRLT were assessed by conventional 2-D, M-mode echocardiography and Doppler...
November 2017: Journal of Advanced Research
https://www.readbyqxmd.com/read/28877973/heritable-pulmonary-hypertension-from-bench-to-bedside
#4
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/28874164/radiation-free-cmr-diagnostic-heart-catheterization-in-children
#5
Kanishka Ratnayaka, Joshua P Kanter, Anthony Z Faranesh, Elena K Grant, Laura J Olivieri, Russell R Cross, Ileen F Cronin, Karin S Hamann, Adrienne E Campbell-Washburn, Kendall J O'Brien, Toby Rogers, Michael S Hansen, Robert J Lederman
BACKGROUND: Children with heart disease may require repeated X-Ray cardiac catheterization procedures, are more radiosensitive, and more likely to survive to experience oncologic risks of medical radiation. Cardiovascular magnetic resonance (CMR) is radiation-free and offers information about structure, function, and perfusion but not hemodynamics. We intend to perform complete radiation-free diagnostic right heart catheterization entirely using CMR fluoroscopy guidance in an unselected cohort of pediatric patients; we report the feasibility and safety...
September 6, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/28872749/outstanding-contribution-to-pediatric-anesthesiology-an-interview-with-dr-robert-h-friesen
#6
Mark D Twite, Richard J Ing, Christopher S Nichols, Myron Yaster
Dr. Robert H. Friesen, (1946-) Professor of Anesthesiology, Children's Hospital Colorado, University of Colorado, Anschutz Medical Campus, has played a pivotal and pioneering role in the development of pediatric and congenital cardiac anesthesiology. His transformative research included the study of the hemodynamic effects of inhalational and intravenous anesthetic agents in the newborn and the effects of anesthetic agents on pulmonary vascular resistance in patients with pulmonary hypertension. As a model clinician-scientist, educator, and administrator, he changed the practice of pediatric anesthesia and shaped the careers of hundreds of physicians-in-training, imbuing them with his core values of honesty, integrity, and responsibility...
September 5, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28852817/risk-factors-for-heart-failure-and-its-costs-among-children-with-complex-congenital-heart-disease-in-a-medicaid-cohort
#7
Deipanjan Nandi, Joseph W Rossano, Yinding Wang, Jeanette M Jerrell
Little research attention has been paid to the occurrence of heart failure (HF) in children with complex congenital heart diseases (CHDs). Herein, we describe the prevalence, risk factors, and costs associated with HF in complex CHD. Patients aged ≤17 years and diagnosed with a complex CHD on multiple service visits over a 15-year period in the SC Medicaid dataset (1996-2010) were tracked and analyzed. The cohort included 2999 unduplicated patients; 51.0% were male; 34.4% were African American. HF was diagnosed in 7...
August 29, 2017: Pediatric Cardiology
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
#8
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/28838436/early-postoperative-problems-after-lung-transplantation-first-year-experiences-in-light-of-the-newly-established-national-hungarian-lung-transplantation-program
#9
I Madurka, J Elek, N Schönauer, T Bartók, K Kormosói-Tóth, P Radeczky, B Gieszer, Á Ghimessy, G Lang, W Klepetko, F Rényi-Vámos
Until December 2015, Hungarian patients' lung transplantations (LTXs) were done at the Medical University of Vienna. After several years of preparation, the National Hungarian Lung Transplantation Program was launched and the first milestone LTX was performed in Budapest on December 12, 2015. During the first 12 months, 18 lung transplantations took place in Hungary, including the first one. Data were retrospectively collected to analyze the early postoperative problems of the first 18 LTX patients of the newly launched Hungarian National Lung Transplantation Program...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28825767/clinical-and-parental-status-of-patients-with-congenital-heart-disease-associated-pulmonary-arterial-hypertension
#10
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/28822439/-multisystemic-smooth-muscle-dysfunction-syndrome-in-children-a-case-report-and-literature-review
#11
Y L Zhou, Y Y Zhang, B L Cheng, D Xu, L F Tang, Z M Chen
Objective: To analyze the clinical characteristics and diagnosis of multisystemic smooth muscle dysfunction syndrome(MSMDS). Method: Clinical data of a case diagnosed as MSMDS and hospitalized in our hospital in July 2016 was retrospectively analyzed. Literature search was performed at databases of PubMed, Wanfang, China National Knowledge Infrastructure and VIP with the key words "multisystemic smooth muscle dysfunction syndrome" "ACTA2" . The literature retrieval was confined from January 1980 to November 2016...
August 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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
#12
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/28791823/echocardiographic-characteristics-of-pulmonary-arterial-hypertension-in-children-with-horizontally-transmitted-hiv
#13
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
#14
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
#15
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/28755186/liver-transplantation-for-hereditary-tyrosinaemia-type-1-in-the-united-kingdom
#16
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
#17
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
#18
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
#19
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
#20
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
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