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Children pulmonary hypertension

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https://www.readbyqxmd.com/read/29788051/proximal-pulmonary-vascular-stiffness-as-a-prognostic-factor-in-children-with-pulmonary-arterial-hypertension
#1
Richard M Friesen, Michal Schäfer, D Dunbar Ivy, Steven H Abman, Kurt Stenmark, Lorna P Browne, Alex J Barker, Kendall S Hunter, Uyen Truong
Aims: Main pulmonary artery (MPA) stiffness and abnormal flow haemodynamics in pulmonary arterial hypertension (PAH) are strongly associated with elevated right ventricular (RV) afterload and associated with disease severity and poor clinical outcomes in adults with PAH. However, the long-term effects of MPA stiffness on RV function in children with PAH remain poorly understood. This study is the first comprehensive evaluation of MPA stiffness in children with PAH, delineating the mechanistic relationship between flow haemodynamics and MPA stiffness as well as the prognostic ability of these measures regarding clinical outcomes...
May 16, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/29781842/application-of-echocardiographic-data-in-patients-with-chronic-kidney-disease
#2
Ruth F Dubin
PURPOSE OF REVIEW: Patients with chronic kidney disease (CKD) are at a high risk for cardiovascular events and mortality, particularly heart failure. Echocardiography is the most commonly used diagnostic imaging modality for heart failure. The purpose of this review is to summarize recent literature that demonstrates how echocardiography may be used to define cardiac structure and function in the CKD population and to identify echocardiographic abnormalities that have utility in predicting clinical outcomes in this population...
May 16, 2018: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/29756719/-pulmonary-hypertension-associated-with-the-human-immunodeficiency-virus-in-children-treatment-with-sildenafil-a-case-report
#3
Carolina Stepffer, Evelyn Gaynor, Mariela López, Norma E González, Micaela Arri, Ana Ms De Dios
Pulmonary hypertension associated with human immunodeficiency virus infection is an extremely rare disease in pediatrics; it requires a high clinical suspicion to reach a diagnosis. Its appearance poses an unfavorable prognostic, but early diagnosis and specific treatment can improve outcomes. We report the clinical case of a fifteen-year-old patient diagnosed with human immunodeficiency virus infection of vertical transmission, without antiretroviral treatment, with cough and progressive exertional dyspnea, associated with signs of right heart failure in which severe pulmonary hypertension was diagnosed...
June 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29750649/cardiac-examination-in-children-with-laron-syndrome-undergoing-mecasermin-therapy
#4
Nurdan Erol, Metin Yıldız, Ayla Güven, Ayse Yıldırım
BACKGROUND: Laron syndrome (LS), which can be defined as primary growth hormone resistance or insensitivity, is a rare genetic disease inherited by an autosomal recessive trait. Although it is undistinguishable from growth hormone deficiency, LS has high levels of growth hormone, but insulin-like growth factor (IGF-1) cannot be synthesized. Mecasermin treatment is the only option for the patients who suffer from LS. This study aims to research cardiac findings of children with LS, who receive treatment with mecasermin...
May 11, 2018: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/29720910/a-retrospective-review-of-infants-receiving-sildenafil
#5
Aliva De, Payal Shah, Jacqueline Szmuszkovicz, Shazia Bhombal, Stanley Azen, Roberta M Kato
OBJECTIVE: The purpose of the study was to assess mortality in an infant population receiving sildenafil. METHODS: A retrospective review of hospitalized infants at Children's Hospital Los Angeles who received sildenafil between 2008 and 2012 was conducted. Patient characteristics, comorbidities, and treatment characteristics were analyzed. Primary outcome was mortality at discharge. Sildenafil dosage ranges were based on the Sildenafil in Treatment-Naïve Children, Aged 1-17 Years, With Pulmonary Arterial Hypertension trial and were categorized as small (<1...
March 2018: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29712611/the-development-of-extracorporeal-membrane-oxygenation
#6
Don K Nakayama
Evolving from the development of heart-lung machines for open-heart surgery, extracorporeal membrane oxygenation has reemerged as a rescue modality for patients with acute respiratory failure that cannot be supported by conventional modes of ventilation. The history of extracorporeal membrane oxygenation begins with the discovery of heparin, fundamental to the success of extracorporeal circulation and membrane lungs. Engineers and scientists created suitable artificial membranes that allowed gas exchange while keeping gas and blood phases separate...
April 1, 2018: American Surgeon
https://www.readbyqxmd.com/read/29702711/acute-post-tracheostomy-clinical-decompensations-in-infants-are-there-predictive-markers
#7
Michael F Nyp, Jane B Taylor, Antonio Petralia, Alexandra Oschman, Mike Norberg, Robert A Weatherly, William Truog, Winston Manimtim
OBJECTIVE:  To report on the population of infants receiving a tracheostomy, identify acute post-tracheostomy clinical decompensations, and seek predictive markers associated with acute complications following the placement of a tracheostomy. STUDY DESIGN:  Retrospective deidentified clinical data was provided by the Infant Pulmonary Data Repository at Children's Mercy Hospital, Kansas City. Data from infants undergoing tracheostomy from January 1, 2008 through September 30, 2016 were divided into one of two study groups based on clinical correlations: (1) no acute decompensations within 72 hours post-tracheostomy or (2) acute clinical decompensation defined as sustained escalation of respiratory care within the 72 hours following tracheostomy...
April 27, 2018: American Journal of Perinatology
https://www.readbyqxmd.com/read/29692255/juvenile-scleroderma-what-has-changed-in-the-meantime
#8
Amra Adrovic, Sezgin Sahin, Kenan Barut, Ozgur Kasapcopur
BACKGROUND: Juvenile scleroderma is a rarely seen chronic connective tissue disorder characterized by stiffening of the skin. The frequency of the disease was reported as one per million. According to organ involvement, the disease is divided into two main forms: systemic and localized scleroderma. OBJECTIVES: Since it is uncommon in children, many aspects of the disease remain discussable. With this review, we aimed to revise recent findings and new developments in this rare condition...
April 22, 2018: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/29678139/pulmonary-hemosiderosis-in-children-with-down-syndrome-a-national-experience
#9
Aurelia Alimi, Jessica Taytard, Rola Abou Taam, Véronique Houdouin, Aude Forgeron, Marc Lubrano Lavadera, Pierrick Cros, Isabelle Gibertini, Jocelyne Derelle, Antoine Deschildre, Caroline Thumerelle, Ralph Epaud, Philippe Reix, Michael Fayon, Sylvie Roullaud, Françoise Troussier, Marie-Catherine Renoux, Jacques de Blic, Sophie Leyronnas, Guillaume Thouvenin, Caroline Perisson, Aimé Ravel, Annick Clement, Harriet Corvol, Nadia Nathan
BACKGROUND: Pulmonary hemosiderosis is a rare and complex disease in children. A previous study from the French RespiRare® network led to two important findings: 20% of the children presented with both pulmonary hemosiderosis and Down syndrome (DS), and at least one tested autoantibody was found positive in 50%. This study investigates the relationships between pulmonary hemosiderosis and DS. METHODS: Patients younger than 20 years old and followed for pulmonary hemosiderosis were retrieved from the RespiRare® database...
April 20, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29674337/analysis-of-right-ventricular-myocardial-stiffness-and-relaxation-components-in-children-and-adolescents-with-pulmonary-arterial-hypertension
#10
Yasunobu Hayabuchi, Akemi Ono, Yukako Homma, Shoji Kagami
BACKGROUND: The rate of left ventricular pressure decrease during isovolumic relaxation is traditionally assessed algebraically via 2 empirical indices: the monoexponential and logistic time constants (τE and τL ). Since the pattern of right ventricular (RV) pressure decrease is quite different from that of the left ventricular, we hypothesized that novel kinematic model parameters are more appropriate and useful to evaluate RV diastolic dysfunction. METHODS AND RESULTS: Eight patients with pulmonary arterial hypertension (age 12...
April 19, 2018: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29650343/safety-efficacy-and-management-of-subcutaneous-treprostinil-infusions-in-the-treatment-of-severe-pediatric-pulmonary-hypertension
#11
Marilyne Levy, Maria-Jesus Del Cerro, Sophie Nadaud, Karunakar Vadlamudi, Elizabeth Colgazier, Jeff Fineman, Damien Bonnet, Ian Adatia
BACKGROUND: Continuous intravenous epoprostenol was the first treatment approved for pulmonary arterial hypertension (PAH) but administration through a central venous line carries risks of thrombosis and sepsis, particularly in children. We sought to evaluate the safety, efficacy and management of subcutaneous (SC) treprostinil in children with PAH. METHODS: Fifty-six children (median age 65, range 1-200 months) were treated with SC treprostinil. Clinical status, echocardiography, NT-proBNP, and site pain and infection were evaluated...
March 15, 2018: International Journal of Cardiology
https://www.readbyqxmd.com/read/29602613/noninvasive-echocardiographic-measures-of-pulmonary-vascular-resistance-in-children-and-young-adults-with-cardiomyopathy
#12
Dor Markush, Robert D Ross, Ronald Thomas, Sanjeev Aggarwal
BACKGROUND: Patients with cardiomyopathy (CM) are at increased risk for pulmonary hypertension (PH). Data are lacking on the use of noninvasive PH measures by echocardiography in patients with CM. The aim of this study was to evaluate the correlation between Doppler-derived echocardiographic indices and catheterization-based measurement of pulmonary vascular resistance (PVR) in children and young adults with CM. METHODS: Imaging studies were retrospectively reviewed from pediatric patients with CM who underwent both echocardiography and cardiac catheterization within a 72-hour period...
March 27, 2018: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29596267/a-stewardship-program-to-optimize-the-use-of-inhaled-nitric-oxide-in-pediatric-critical-care
#13
Tanya Di Genova, Christina Sperling, Ashley Gionfriddo, Zelia Da Silva, Leanne Davidson, Jason Macartney, Michael Finelli, Robert P Jankov, Peter C Laussen
PURPOSE: Inhaled nitric oxide (iNO) is a pulmonary vasodilator that is approved for use in term and near-term neonates with hypoxic respiratory failure associated with evidence of pulmonary hypertension. However, it is commonly used in infants and children to treat a variety of other cardiopulmonary diseases associated with pulmonary hypertension and hypoxic respiratory failure. In critically ill children, iNO therapy may be continued for a prolonged period, and this increases the risk for adverse consequences including toxicity and unnecessary costs...
April 2018: Quality Management in Health Care
https://www.readbyqxmd.com/read/29592985/caffeine-to-prevent-respiratory-failure-and-improve-outcome-in-infant-pertussis
#14
John Evered, Eric Pfeifer, Matthew Gracianette
Pertussis remains a dangerous disease for children around the world, especially for infants less than 6 months old. In this age group, high mortality and morbidity have been linked to the effects of the pertussis toxin, including lymphocytosis, pulmonary hyperviscosity and pulmonary hypertension. This paper reports on an infant with pertussis who received therapeutic caffeine. Caffeine might improve outcomes in pertussis by preventing apnoea, improving respiratory drive and decreasing pulmonary complications...
March 28, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29580786/neurodevelopmental-outcomes-in-cdh-survivors-a-single-institution-s-experience
#15
Joseph T Church, Rodrigo Mon, Tiffany Wright, Megan A Coughlin, Maria Ladino-Torres, Christopher Tapley, Heather Bowen, Niki Matusko, George B Mychaliska
PURPOSE: Survivors of congenital diaphragmatic hernia (CDH) face high morbidity. We studied the neurodevelopmental outcomes of CDH survivors at a single institution. METHODS: CDH survivors born July 2006-March 2016 at a free-standing children's hospital were reviewed. Neurodevelopment was assessed using the Peabody Developmental Motor Scales (PDMS-2) broken into gross, fine, and total motor quotients. Data collected included prenatal variables (liver herniation, defect laterality, observed:expected total fetal lung volume (o:eTFLV) on MRI), birth demographics (sex, race, estimated gestational age (EGA), birth weight (BtWt), 5 min APGAR, associated anomalies), and therapies/hospital course (HFOV/HFJV, ECMO, timing of repair, pulmonary hypertension (PHTN) severity, length of stay, ventilator days)...
March 5, 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29575641/nt-pro-bnp-a-marker-for-worsening-respiratory-status-and-mortality-in-infants-and-young-children-with-pulmonary-hypertension
#16
Shahnawaz M Amdani, Muhammad Umair M Mian, Ron L Thomas, Robert D Ross
AIM: To evaluate predictors of morbidity and mortality in pediatric patients with pulmonary hypertension (PH), laboratory and echocardiographic measures of PH were analyzed. METHODS: A retrospective review of all infants and children < 2 years of age with PH from January 2011 to August 2016 was conducted. Correlations were determined using Spearman's rank correlation coefficients. Differences in characteristics between survivors and nonsurvivors were analyzed and Kaplan-Meier survival curves were generated...
March 25, 2018: Congenital Heart Disease
https://www.readbyqxmd.com/read/29570688/diagnosis-evaluation-and-treatment-of-pulmonary-arterial-hypertension-in-children
#17
REVIEW
Benjamin S Frank, D Dunbar Ivy
Pulmonary Hypertension (PH), the syndrome of elevated pressure in the pulmonary arteries, is associated with significant morbidity and mortality for affected children. PH is associated with a wide variety of potential underlying causes, including cardiac, pulmonary, hematologic and rheumatologic abnormalities. Regardless of the cause, for many patients the natural history of PH involves progressive elevation in pulmonary arterial resistance and pressure, right ventricular dysfunction, and eventually heart failure...
March 23, 2018: Children
https://www.readbyqxmd.com/read/29569581/genetic-basis-for-childhood-interstitial-lung-disease-among-japanese-infants-and-children
#18
Itaru Hayasaka, Kazutoshi Cho, Takuma Akimoto, Masahiko Ikeda, Yutaka Uzuki, Masafumi Yamada, Koh Nakata, Itsuko Furuta, Tadashi Ariga, Hisanori Minakami
BackgroundGenetic variants responsible for childhood interstitial lung disease (chILD) have not been studied extensively in Japanese patients.MethodsThe study population consisted of 62 Japanese chILD patients. Twenty-one and four patients had pulmonary hypertension resistant to treatment (PH) and hypothyroidism, respectively. Analyses of genetic variants were performed in all 62 patients for SFTPC and ABCA3, in all 21 PH patients for FOXF1, and in a limited number of patients for NKX2.1.ResultsCausative genetic variants for chILD were identified in 11 (18%) patients: SFTPC variants in six, NKX2...
February 2018: Pediatric Research
https://www.readbyqxmd.com/read/29549173/clinical-spectrum-of-pyruvate-kinase-deficiency-data-from-the-pyruvate-kinase-deficiency-natural-history-study
#19
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 Pospíšilová, 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 nonspherocytic hemolytic anemia. Medical history and laboratory and radiologic data were retrospectively collected at enrollment for 254 patients with molecularly confirmed PK deficiency. Perinatal complications were common, including anemia that required transfusions, hyperbilirubinemia, hydrops, and prematurity...
May 17, 2018: Blood
https://www.readbyqxmd.com/read/29549119/the-case-for-early-use-of-rapid-whole-genome-sequencing-in-management-of-critically-ill-infants-late-diagnosis-of-coffin-siris-syndrome-in-an-infant-with-left-congenital-diaphragmatic-hernia-congenital-heart-disease-and-recurrent-infections
#20
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
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