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Children pulmonary hypertension

Rachael F Grace, Paola Bianchi, Eduard J van Beers, Stefan W Eber, Bertil Glader, Hassan M Yaish, Jenny M Despotovic, Jennifer A Rothman, Mukta Sharma, Melissa M McNaull, Elisa Fermo, Kimberly Lezon-Geyda, D Holmes Morton, Ellis J Neufeld, Satheesh Chonat, Nina Kollmar, Christine M Knoll, Kevin Kuo, Janet L Kwiatkowski, Dagmar Pospíŝilová, Yves D Pastore, Alexis A Thompson, Peter E Newburger, Yaddanapudi Ravindranath, Winfred C Wang, Marcin W Wlodarski, Heng Wang, Susanne Holzhauer, Vicky R Breakey, Joachim Kunz, Sujit Sheth, Melissa J Rose, Heather A Bradeen, Nolan Neu, Dongjing Guo, Hasan Al-Sayegh, Wendy B London, Patrick G Gallagher, Alberto Zanella, Wilma Barcellini
An international, multicenter registry was established to collect retrospective and prospective clinical data on patients with pyruvate kinase (PK) deficiency, the most common glycolytic defect causing congenital non-spherocytic hemolytic anemia. Medical history and laboratory and radiologic data were retrospectively collected at enrollment in 254 patients with molecularly confirmed PK deficiency. Perinatal complications were common, including anemia requiring transfusions, hyperbilirubinemia, hydrops, and prematurity...
March 16, 2018: Blood
Nathaly M Sweeney, Shareef A Nahas, Shimul Chowdhury, Miguel Del Campo, Marilyn C Jones, David P Dimmock, Stephen F Kingsmore, Rcigm Investigators
Congenital diaphragmatic hernia (CDH) results from incomplete formation of the diaphragm leading to herniation of abdominal organs into the thoracic cavity. CDH is associated with pulmonary hypoplasia, congenital heart disease and pulmonary hypertension. Genetically, it is associated with aneuploidies, chromosomal copy number variants, and single gene mutations. CDH is the most expensive non-cardiac congenital defect: Management frequently requires implementation of Extracorporeal Membrane Oxygenation (ECMO), which increases management expenditures 2...
March 16, 2018: Cold Spring Harbor Molecular Case Studies
Johannes Krämer, Felix Kreuzer, Michael Kaestner, Peter Bride, Fabian von Scheidt, Jannos Siaplaouras, Heiner Latus, Dietmar Schranz, Christian Apitz
Right ventricular (RV) hypertrophy is regarded as the adaptation on chronic RV pressure load in pulmonary hypertension. As the RV Sokolow-Lyon index (RVSLI) is an electrocardiographic marker of RV hypertrophy, we hypothesized that RVSLI might be able to reflect RV pressure load. Therefore, the purpose of this study was to characterize the diagnostic impact of the RVSLI in children with idiopathic pulmonary arterial hypertension (IPAH) in order to assess disease severity and to evaluate its value for the prediction of worse outcome...
March 14, 2018: Pediatric Cardiology
Georgia Brown, Tilman Humpl
PURPOSE OF REVIEW: Pulmonary vascular disease (PVD) complicates the course of many cardiovascular, pulmonary and other systemic diseases in children. The physiological sequelae (pulmonary hypertension and elevated pulmonary vascular resistance) can overwhelm the right ventricle and lead to circulatory collapse. Despite the common end-point, the preceding pathophysiology is complex and variable and requires a tailored approach to diagnosis and management. In this article, we will review the most recent evidence and explore an approach to current controversies in the diagnosis and management of common or challenging patient subgroups...
March 9, 2018: Current Opinion in Pediatrics
S D Yang, P Q Li, Y G Huang, W Li, L Z Ma, L Wu, N Wang, J M Lu, W Q Chen, Guang-Ming Liu, Y M Xiong, Y L Chen, Ying Zhang
Enterovirus A-71 (EV-A71) may be fatal, but the natural history, symptoms, and signs are poorly understood. This study aimed to examine the natural history of fatal EV-A71 infection and to identify the symptoms and signs of early warning of deterioration. This was a clinical observational study of fatal cases of EV-A71 infection treated at five Chinese hospitals between 1 January 2010 and 31 December 2012. We recorded and analysed 91 manifestations of EV-A71 infection in order to identify early prognosis indicators...
March 12, 2018: Epidemiology and Infection
Martin Koestenberger, Alexander Avian, Hannes Sallmon, Andreas Gamillscheg, Gernot Grangl, Stefan Kurath-Koller, Sabrina Schweintzger, Ante Burmas, Georg Hansmann
OBJECTIVE: The right ventricular outflow tract (RVOT) is pivotal for adequate RV function and known to be adversely affected by elevated pulmonary arterial pressure (PAP) in adults with pulmonary hypertension (PH). Aim of this study was to determine the effects of increased RV pressure afterload in children with PH on RVOT size, function, and flow parameters. METHODS: We conducted a transthoracic echocardiographic study in 51 children with PH (median age: 5.3 years; range 1...
March 5, 2018: Echocardiography
Andreas Krause, Jochen Zisowsky, Jasper Dingemanse
BACKGROUND: Macitentan is the first endothelin receptor antagonist with demonstrated efficacy on morbidity and mortality in pulmonary arterial hypertension (PAH) in the pivotal study SERAPHIN. METHODS: The pharmacokinetics (PK) of macitentan and its active metabolite, ACT-132577, were characterized in a population model. Efficacy and hemodynamics (pharmacodynamics, PD) were related to PK based on PK/PD modeling. RESULTS: Sex, age, and body weight influenced the PK to a statistically significant extent...
February 28, 2018: Pulmonary Pharmacology & Therapeutics
Lia Gore, Pamela R Kearns, Maria Lucia de Martino Lee, Carmino Antonio De Souza, Yves Bertrand, Nobuko Hijiya, Linda C Stork, Nack-Gyun Chung, Rocio Cardenas Cardos, Tapan Saikia, Franca Fagioli, Jong Jin Seo, Judith Landman-Parker, Donna Lancaster, Andrew E Place, Karen R Rabin, Mariana Sacchi, Rene Swanink, C Michel Zwaan
Purpose Safe, effective treatments are needed for pediatric patients with chronic myeloid leukemia in chronic phase (CML-CP). Dasatinib is approved for treatment of adults and children with CML-CP. A phase I study determined suitable dosing for children with Philadelphia chromosome-positive (Ph+) leukemias. Methods CA180-226/NCT00777036 is a phase II, open-label, nonrandomized prospective trial of patients < 18 years of age receiving dasatinib. There are three cohorts: (1) imatinib-resistant/intolerant CML-CP, (2) imatinib-resistant/intolerant CML in accelerated/blast phase or Ph+ acute lymphoblastic leukemia (n = 17), and (3) newly diagnosed CML-CP treated with tablets or powder for oral suspension...
March 2, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Michal Schäfer, Kathryn K Collins, Lorna P Browne, D Dunbar Ivy, Steven Abman, Richard Friesen, Benjamin Frank, Brian Fonseca, Michael DiMaria, Kendall S Hunter, Uyen Truong, Johannes C von Alvensleben
BACKGROUND: Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony...
February 6, 2018: Journal of Heart and Lung Transplantation
Mohsen S Elalfy, Omneya Ibrahim Youssef, Marwa M R Deghedy, Mariam M Abdel Naby
Cardiovascular complications are well recognized in β-thalassemia and sickle cell disease (SCD). The objective of this study was to evaluate left ventricular (LV) structural and functional changes and their relationship to sleep-disordered breathing (SDB) in children with β-thalassemia and SCD. One hundred patients recruited from the hematology clinic were subjected to Pittsburgh Sleep Quality Index score; 26 patients had positive score (Pittsburgh Sleep Quality Index ≥5) (15 β-thalassemia major and 11 SCD) and were compared with 25 age-matched and sex-matched controls...
February 28, 2018: Journal of Pediatric Hematology/oncology
Michael L O'Byrne, Kevin F Kennedy, Joshua P Kanter, John T Berger, Andrew C Glatz
BACKGROUND: Cardiac catheterization is the gold standard for assessment and follow-up of patients with pulmonary hypertension (PH). To date, there are limited data about the factors that influence the risk of catastrophic adverse events after catheterization in this population. METHODS AND RESULTS: A retrospective multicenter cohort study was performed to measure risk of catastrophic adverse outcomes after catheterization in children and young adults with PH and identify risk factors for these outcomes...
February 28, 2018: Journal of the American Heart Association
Deborah R Liptzin, Steven H Abman, Ann Giesenhagen, D Dunbar Ivy
Liptzin, Deborah R., Steven H. Abman, Ann Giesenhagen, and D. Dunbar Ivy. An approach to children with pulmonary edema at high altitude. High Alt Med Biol. 00:000-000, 2018. INTRODUCTION: Diagnosis of high-altitude illness can be more challenging in children, especially those who are preverbal. Families often travel to high elevations for family vacations, either for skiing, hiking, and/or camping. They may present to their primary care providers looking for anticipatory guidance before travel or may follow-up after developing high-altitude illness...
February 22, 2018: High Altitude Medicine & Biology
M Louise Morrison, Corrina McMahon, Riona Tully, Noelle Enright, Ricardo Pignatelli, Jeffrey A Towbin, Colin J McMahon
OBJECTIVES: Incidence of sickle cell disease (SCD) in Ireland has dramatically increased. Disease survival has also steadily improved however cardiovascular manifestations remain important causes of morbidity. These include reports of left ventricular hypertrabeculation (LVHT)/noncompaction. We sought to investigate the prevalence of LVHT among a large cohort of children with SCD. METHODS: We retrospectively reviewed the records of all patients with a diagnosis of SCD who had undergone surveillance echocardiography at Our Lady's Children's Hospital Crumlin (OLCHC) from 1998 to 2015...
February 21, 2018: Congenital Heart Disease
Manish Aggarwal, R Mark Grady
Pulmonary hypertension (PH) is an increasingly recognized problem in children, particularly within tertiary pediatric hospitals. This increase is, in large part, due to ever improving survival among previously fatal conditions, such as extreme prematurity and complicated congenital heart disease. This increased recognition has paralleled burgeoning pharmacologic and interventional PH-specific treatment options. Unfortunately, most PH-specific therapies have not been tested in children with rigorous, randomized, controlled trials...
February 20, 2018: Current Treatment Options in Cardiovascular Medicine
Christian Apitz, Dietmar Schranz
No abstract text is available yet for this article.
February 16, 2018: Respiration; International Review of Thoracic Diseases
D Thiwanka Wijeratne, Katherine Lajkosz, Susan B Brogly, M Diane Lougheed, Li Jiang, Ahmad Housin, David Barber, Ana Johnson, Katharine M Doliszny, Stephen L Archer
BACKGROUND: The World Health Organization recognizes 5 groups of pulmonary hypertension (PH), categorized by pathogenesis or comorbidity: 1-pulmonary arterial hypertension 2-left-heart disease, 3-lung disease and hypoxia 4-chronic thromboembolic disease, and 5-miscellaneous. The epidemiology of PH, apart from group 1, is largely unknown. METHODS AND RESULTS: We describe incidence, prevalence, comorbidities, mortality and prescribing patterns for groups 1 to 4 PH from 1993 to 2012...
February 2018: Circulation. Cardiovascular Quality and Outcomes
Chung-Ting Hsu, Chao-Huei Chen, Ming-Chih Lin, Teh-Ming Wang, Ya-Chi Hsu
BACKGROUND: Premature infants are at high risk for developmental delay and cognitive dysfunction. Besides medical conditions, growth restriction is regarded as an important risk factor for cognitive and neurodevelopmental dysfunction throughout childhood and adolescence and even into adulthood. In this study, we analyzed the relationship between post-discharge body weight and psychomotor development using a nationwide dataset. MATERIALS AND METHODS: This was a nationwide cohort study conducted in Taiwan...
2018: PloS One
Qiangqiang Li, Konstantinos Dimopoulos, Chen Zhang, Yan Zhu, Qian Liu, Hong Gu
Inhaled prostacyclin analogue iloprost is currently utilized in adult patients with pulmonary arterial hypertension (PAH), but little information is available on its use in the pediatric population. This study evaluated the safety and acute haemodynamic effects of inhaled iloprost in children with PAH associated with congenital heart disease (CHD). Children with PAH-CHD who underwent cardiac catheterization and iloprost administration in our catheter laboratory between June 2007 and October 2015 were included...
February 9, 2018: Pediatric Cardiology
Jonathan I Silverberg, Lauren Kwa, Michael C Kwa, Anne E Laumann, Kaveh Ardalan
Objective: JDM is associated with multiple potential risk factors for cardiovascular disease, including reduced heart rate variability, systolic/diastolic cardiac dysfunction, abnormal brachial artery reactivity and metabolic syndrome. However, little is known about cardiovascular risk in JDM. We sought to examine the association between JDM and cardiovascular risk factors and disease in US children. Methods: Data from the 2002-12 National Inpatient Sample was analysed, including ∼20% of all US hospitalizations (n = 14 535 620 paediatric hospitalizations)...
January 24, 2018: Rheumatology
Kriti Puri, Peter Kazembe, Treasure Mkaliainga, Msandeni Chiume, Antonio G Cabrera, Amy Sims Sanyahumbi
Malawi is one of the poorest nations in the world, ranked 151st among 195 countries by the World Bank, with an under-5-year mortality rate of 63 per 1000 live births. There are no previous studies describing the spectrum of inpatient pediatric cardiology consultations in sub-Saharan Africa. A descriptive cohort study was performed at Kamuzu Central Hospital (KCH), a tertiary care hospital in Lilongwe, Malawi. Demographic, anthropometric, and clinical information for all cardiology consults patients aged 0-18 years admitted to the children's wards over a period of 1 month was reviewed...
January 25, 2018: Congenital Heart Disease
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