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Pediatric pulmonary hypertension

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https://www.readbyqxmd.com/read/28737623/an-observational-study-of-severe-pertussis-in-100-infants-%C3%A2-120-days-of-age
#1
James D Cherry, Kristen Wendorf, Brooke Bregman, Deborah Lehman, Delma Nieves, John S Bradley, Wilbert H Mason, Linette Sande-Lopez, Merrick Lopez, Myke Federman, Tempe Chen, Dean Blumberg, Samantha Johnston, Hayden T Schwenk, Peggy Weintrub, Kevin K Quinn, Kathleen Winter, Kathleen Harriman
BACKGROUND: Pertussis in young infants is a unique, severe, afebrile, cough illness that is frequently fatal. METHODS: All pertussis cases ≤ 120 days of age admitted to a pediatric intensive care unit (PICU) in California between October 1, 2013 and April 25, 2015 were evaluated. RESULTS: Of 100 pertussis patients ≤ 120 days of age admitted to PICU's, there were 5 deaths. The white blood cell (WBC) counts in the fatal cases were significantly higher than in the non-fatal cases...
July 20, 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28707968/accelerometry-improving-objective-assessments-of-therapeutic-impact-in-pediatric-pulmonary-arterial-hypertension
#2
Eric D Austin, Jeffrey A Feinstein
No abstract text is available yet for this article.
July 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28704161/cardiovascular-safety-of-low-dose-fenfluramine-in-dravet-syndrome-a-review-of-its-benefit-risk-profile-in-a-new-patient-population
#3
An-Sofie Schoonjans, Fabienne Marchau, Bernard P Paelinck, Lieven Lagae, Arnold Gammaitoni, Milka Pringsheim, Martin G Keane, Berten Ceulemans
OBJECTIVE: Dravet syndrome (DS) is a rare, treatment-resistant epilepsy syndrome for which current treatment regimens are often ineffective. Fenfluramine is currently in development for treatment of DS based on reports in the 1980s and 1990s of its antiepileptic activity in pediatric patients with intractable epilepsy. However, fenfluramine was withdrawn from global markets in 1997 following reports of its association with pulmonary hypertension and heart valve disease in adult patients treated for obesity...
July 13, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#4
Roberto Gallotti, Diana E Drogalis-Kim, Gary Satou, Juan Alejos
Pulmonary arterial hypertension (PAH) is a rare and progressive disorder. Current treatment in the pediatric population includes phosphodiesterase 5 inhibitors (PDE-5i), endothelin receptor antagonists (ERA), and both inhaled and intravenous prostacyclin pathway agonists. As of December 22, 2015 the first oral prostacyclin pathway agonist, selexipag (Uptravi(®)), was FDA approved in the US. In this case series, we discuss our single-center experience using selexipag in a pediatric population, composed of both patients with idiopathic PAH, and patients with congenital heart disease and PAH...
July 13, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28701597/randomized-comparative-study-of-intravenous-infusion-of-three-different-fixed-doses-of-milrinone-in-pediatric-patients-with-pulmonary-hypertension-undergoing-open-heart-surgery
#5
Neeraj Kumar Barnwal, Sanjeeta Rajendra Umbarkar, Manjula Sudeep Sarkar, Raylene J Dias
BACKGROUND: Pulmonary hypertension secondary to congenital heart disease is a common problem in pediatric patients presenting for open heart surgery. Milrinone has been shown to reduce pulmonary vascular resistance and pulmonary artery pressure in pediatric patients and neonates postcardiac surgery. We aimed to evaluate the postoperative outcome in such patients with three different fixed maintenance doses of milrinone. METHODOLOGY: Patients were randomized into three groups...
July 2017: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/28699846/safety-and-effectiveness-of-tadalafil-in-pediatric-patients-with-pulmonary-arterial-hypertension-a-subgroup-analysis-based-on-japan-post-marketing-surveillance
#6
Hiroyoshi Yamazaki, Noriko Kobayashi, Masanori Taketsuna, Koyuki Tajima, Nahoko Suzuki, Masahiro Murakami
OBJECTIVE: To evaluate the long-term safety and effectiveness of tadalafil in pediatric patients with pulmonary arterial hypertension (PAH) in real-world clinical practice. METHODS: This is an observational surveillance of PAH patients receiving tadalafil in the contracted sites. We performed a subgroup analysis of 391 pediatric PAH patients (<18 years) who were included from 1,704 total patients in this surveillance. Safety was assessed from the frequency of adverse drug reactions (ADRs), discontinuations due to adverse events (AEs), and serious adverse drug reactions (SADRs)...
July 12, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28680572/pulmonary-arterial-hypertension-in-the-usa-an-epidemiological-study-in-a-large-insured-pediatric-population
#7
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
https://www.readbyqxmd.com/read/28680562/addressing-the-challenges-of-phenotyping-pediatric-pulmonary-vascular-disease
#8
REVIEW
Kara N Goss, Allen D Everett, Peter M Mourani, Christopher D Baker, Steven H Abman
Pediatric pulmonary vascular disease (PVD) and pulmonary hypertension (PH) represent phenotypically and pathophysiologically diverse disease categories, contributing substantial morbidity and mortality to a complex array of pediatric conditions. Here, we review the multifactorial nature of pediatric PVD, with an emphasis on improved recognition, phenotyping, and endotyping strategies for pediatric PH. Novel tailored approaches to diagnosis and treatment in pediatric PVD, as well as the implications for long-term outcomes, are highlighted...
March 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28645441/evaluation-and-management-of-pulmonary-hypertension-in-children-with-bronchopulmonary-dysplasia
#9
Usha Krishnan, Jeffrey A Feinstein, Ian Adatia, Eric D Austin, Mary P Mullen, Rachel K Hopper, Brian Hanna, Lew Romer, Roberta L Keller, Jeffrey Fineman, Robin Steinhorn, John P Kinsella, D Dunbar Ivy, Erika Berman Rosenzweig, Usha Raj, Tilman Humpl, Steven H Abman
No abstract text is available yet for this article.
June 20, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28625502/a-computable-phenotype-improves-cohort-ascertainment-in-a-pediatric-pulmonary-hypertension-registry
#10
Alon Geva, Jessica L Gronsbell, Tianxi Cai, Tianrun Cai, Shawn N Murphy, Jessica C Lyons, Michelle M Heinz, Marc D Natter, Nandan Patibandla, Jonathan Bickel, Mary P Mullen, Kenneth D Mandl
OBJECTIVES: To compare registry and electronic health record (EHR) data mining approaches for cohort ascertainment in patients with pediatric pulmonary hypertension (PH) in an effort to overcome some of the limitations of registry enrollment alone in identifying patients with particular disease phenotypes. STUDY DESIGN: This study was a single-center retrospective analysis of EHR and registry data at Boston Children's Hospital. The local Informatics for Integrating Biology and the Bedside (i2b2) data warehouse was queried for billing codes, prescriptions, and narrative data related to pediatric PH...
June 15, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28617685/adult-congenital-heart-disease-magnitude-of-the-problem
#11
Akanksha N Thakkar, Ponraj Chinnadurai, C Huie Lin
PURPOSE OF REVIEW: To define the magnitude of problems faced by patients with adult congenital heart disease (ACHD) and to identify unmet needs for this population. RECENT FINDINGS: The ACHD population is estimated to include more than 1 million people in the United States and continues to grow at a steady rate. Owing to the decline in early mortality in this group, modern medicine is now faced by the long-term complications associated with congenital heart disease such as chronic heart failure, increased endocarditis risk, elevated burden of arrhythmias, pulmonary hypertension, valvular dysfunction, and pregnancy...
June 15, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/28611076/learning-a-comorbidity-driven-taxonomy-of-pediatric-pulmonary-hypertension
#12
Mei-Sing Ong, Mary P Mullen, Eric D Austin, Peter Szolovits, Marc Natter, Alon Geva, Tianxi Cai, Sek Won Kong, Kenneth D Mandl
Rationale: Pediatric pulmonary hypertension (PH) is a heterogeneous condition with varying natural history and therapeutic response. Precise classification of PH subtypes is therefore crucial for individualizing care. However, gaps remain in our understanding of the spectrum of PH in children. Objective: We seek to study the manifestations of PH in children, and to assess the feasibility of applying a network-based approach to discern disease subtypes from comorbidity data recorded in longitudinal datasets...
June 13, 2017: Circulation Research
https://www.readbyqxmd.com/read/28609431/polymorphisms-in-urea-cycle-enzyme-genes-are-associated-with-persistent-pulmonary-hypertension-of-the-newborn
#13
Dinushan C Kaluarachchi, Caitlin J Smith, Jonathan M Klein, Jeffrey C Murray, John M Dagle, Kelli K Ryckman
BACKGROUND: Persistent pulmonary hypertension of the newborn (PPHN) is characterized by elevated pulmonary vascular resistance. Endogenous nitric oxide is critical for regulation of pulmonary vascular resistance. Nitric oxide is generated from L -arginine, supplied by the urea cycle (UC). We hypothesized that polymorphisms in UC enzyme genes and low concentrations of UC intermediates are associated with PPHN. METHODS: We performed a family based candidate gene analysis to study 48 single nucleotide polymorphisms (SNPs) in 6 UC enzyme genes...
June 13, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28597754/characteristics-of-pediatric-pulmonary-hypertension-trials-registered-on-clinicaltrials-gov
#14
Jordan D Awerbach, Richard A Krasuski, Kevin D Hill
The investigation of pediatric pulmonary hypertension (PH) drugs has been identified as a high priority by the United States National Institutes of Health (NIH). Studying pediatric PH is challenging due to the rare and heterogeneous nature of the disease. We sought to define the pediatric PH clinical trials landscape, to evaluate areas of trial success or failure, and to identify potential obstacles to the study of pediatric PH drugs. Interventional pediatric (ages 0-17 years) PH trials registered on ClinicalTrials...
April 2017: Pulmonary Circulation
https://www.readbyqxmd.com/read/28595211/-closure-of-wide-patent-ductus-arteriosus-using-a-fenestrated-muscular-vsd-occluder-device-in-a-pediatric-patient-with-down-syndrome-and-pulmonary-hypertension
#15
Osman Güvenç, Murat Saygı, İbrahim Halil Demir, Ender Ödemiş
Patients with wide patent ductus arteriosus and significant pulmonary hypertension not treated in time constitute a significant problem for cardiologists. For these patients, tests that could aid in decision-making for further planning include reversibility and balloon occlusion tests performed in the catheterization laboratory. Devices developed for the closure of ductus as well as different devices with off-label use may be employed in patients scheduled for transcatheter occlusion. When result of reversibility test is borderline positive, the use of fenestrated device may be applicable for selected patients...
June 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28590293/pediatric-pulmonary-arterial-hypertension-on-the-eve-of-growing-up
#16
Johannes M Douwes, Rolf M F Berger
PURPOSE OF REVIEW: Current recommendations for diagnosis and treatment of pulmonary arterial hypertension (PAH) during childhood are expert opinion based, because of lacking pediatric data. In recent years, however, important pediatric data have emerged on PAH. RECENT FINDINGS: PAH in children shows similarities as well as differences compared to adults. Neonates and children know specific clinical presentations and a hemodynamic profile that differs from adults with PAH...
June 5, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28548215/protein-z-and-endothelin-1-genetic-polymorphisms-in-pediatric-egyptian-sickle-cell-disease-patients
#17
Mervat M Khorshied, Nohair S Mohamed, Rania S Hamza, Rasha M Ali, Mona K El-Ghamrawy
BACKGROUND: Sickle cell disease (SCD) is a monogenic disease associated with multisystem morbidity. Vasculopathy caused by delicate imbalance between coagulation and endothelial systems plays a pivotal role in disease course. As Protein Z and Endothelin-1 genetic polymorphisms may increase the thrombotic risk, the aim of the current work was to verify the possible impact of Protein Z (PROZ G79A) and Endothelin-1 (EDN1 G5665T) polymorphisms on the clinic-laboratory features of the SCD in a cohort of Egyptian pediatric patients...
May 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28546372/inhaled-pulmonary-vasodilators-are-there-indications-within-the-pediatric-icu
#18
Bradley A Kuch, Alvin L Saville, Joan Sanchez De Toledo, Shekhar T Venkataraman
Inhaled nitric oxide (INO) is only FDA-cleared for neonates (> 34 weeks gestation) with hypoxic respiratory failure-associated pulmonary hypertension. Off-label use of INO is common in the pediatric population despite a lack of evidence regarding survival benefit, questioning whether the therapy should be considered outside the neonatal period. A lack of definitive evidence combined with increasing health-care costs has led to the use of less costly inhaled prostacyclin as an alternative to INO, presenting unique patient safety concerns...
June 2017: Respiratory Care
https://www.readbyqxmd.com/read/28523862/transatlantic-medical-consultation-and-second-opinion-in-pediatric-cardiology-has-benefit-past-patient-care-a-case-study-in-videoconferencing
#19
Lubica Kovacikova, Martin Zahorec, Peter Skrak, Brian D Hanna, R Lee Vogel
BACKGROUND: Telemedicine is a rapidly evolving form of modern information and communication technology used to deliver clinical services and educational activities. OBJECTIVE: The aim of this article is to report and analyze our experience with transatlantic consultation via videoconferencing in pediatric cardiology. METHODS: In February, 2013, videoconferencing project was launched between a medium-volume pediatric cardiac center in Bratislava, Slovakia and subspecialty experts from a high-volume pediatric cardiac program at The Children's Hospital of Philadelphia (CHOP), USA...
May 19, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28509730/american-college-of-critical-care-medicine-clinical-practice-parameters-for-hemodynamic-support-of-pediatric-and-neonatal-septic-shock
#20
Alan L Davis, Joseph A Carcillo, Rajesh K Aneja, Andreas J Deymann, John C Lin, Trung C Nguyen, Regina S Okhuysen-Cawley, Monica S Relvas, Ranna A Rozenfeld, Peter W Skippen, Bonnie J Stojadinovic, Eric A Williams, Tim S Yeh, Fran Balamuth, Joe Brierley, Allan R de Caen, Ira M Cheifetz, Karen Choong, Edward Conway, Timothy Cornell, Allan Doctor, Marc-Andre Dugas, Jonathan D Feldman, Julie C Fitzgerald, Heidi R Flori, James D Fortenberry, Ana Lia Graciano, Bruce M Greenwald, Mark W Hall, Yong Yun Han, Lynn J Hernan, Jose E Irazuzta, Elizabeth Iselin, Elise W van der Jagt, Howard E Jeffries, Saraswati Kache, Chhavi Katyal, Niranjan Tex Kissoon, Alexander A Kon, Martha C Kutko, Graeme MacLaren, Timothy Maul, Renuka Mehta, Fola Odetola, Kristine Parbuoni, Raina Paul, Mark J Peters, Suchitra Ranjit, Karin E Reuter-Rice, Eduardo J Schnitzler, Halden F Scott, Adalberto Torres, Jacki Weingarten-Abrams, Scott L Weiss, Jerry J Zimmerman, Aaron L Zuckerberg
OBJECTIVES: The American College of Critical Care Medicine provided 2002 and 2007 guidelines for hemodynamic support of newborn and pediatric septic shock. Provide the 2014 update of the 2007 American College of Critical Care Medicine "Clinical Guidelines for Hemodynamic Support of Neonates and Children with Septic Shock." DESIGN: Society of Critical Care Medicine members were identified from general solicitation at Society of Critical Care Medicine Educational and Scientific Symposia (2006-2014)...
June 2017: Critical Care Medicine
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