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Pediatric pulmonary hypertension

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https://www.readbyqxmd.com/read/28548215/protein-z-and-endothelin-1-genetic-polymorphisms-in-pediatric-egyptian-sickle-cell-disease-patients
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28506578/-congenital-heart-disease-in-adolescents-and-adults-management-in-a-general-cardiology-department-in-senegal
#5
A Mbaye, M Bodian, A A Ngaïdé, H Abdourafiq, M C B O Leye, S Savodogo, F Aw, M Ndiaye, I Kouamé, K Babaka, M Dioum, N D Gaye, S A Sarr, M B Ndiaye, A D Kane, A Kane
BACKGROUND: Congenital heart diseases in adults include malformations treated in childhood that decompensate secondarily and those asymptomatic at birth, appear later. This study aims to identify congenital heart diseases in adults in general cardiology department of Senegal and to assess clinical presentations, treatment and outcomes. METHODS: We conducted a cross-sectional and descriptive study based on the records of patients aged at least 16 years and followed for congenital heart disease in the cardiology department of the General Hospital of Grand-Yoff in Dakar between May 2003 and March 2015...
May 12, 2017: Annales de Cardiologie et D'angéiologie
https://www.readbyqxmd.com/read/28481069/-laparoscopic-adrenalectomy-advantages-of-the-minimally-invasive-approach
#6
P Rodríguez Iglesias, J Gómez-Chacón Villalba, L Rodríguez Caraballo, P Ortolá Fortes, J Cortés Sáez, A Marco Macián, J J Vila Carbó
AIM: To describe our experience as a tertiary center on the use of laparoscopic adrenal surgery in children. MATERIAL AND METHODS: A descriptive, retrospective study of patients with pathologic adrenal masses undergoing laparoscopic adrenal surgery, between 2012 and 2015. Epidemiological variables, surgical technique, complications and follow-up were studied. RESULTS: Nine patients were studied with a median age of 62 months (5-184). In 3 patients (33...
October 10, 2016: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28475298/a-modified-noninvasive-screening-protocol-for-pulmonary-hypertension-in-children-with-sickle-cell-disease-who-should-be-sent-for-invasive-evaluation
#7
Christian Lilje, Jordan Harry, Kelly K Gajewski, Renee V Gardner
BACKGROUND: Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. OBJECTIVES: We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies...
May 5, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28456396/long-term-outcomes-of-children-with-trisomy-13-and-18-after-congenital-heart-disease-interventions
#8
Jennifer K Peterson, Lazaros K Kochilas, Kirsti G Catton, James H Moller, Shaun P Setty
BACKGROUND: The purpose of this study is to report short- and long-term outcomes after congenital heart defect (CHD) interventions in patients with trisomy 13 or 18. METHODS: A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) identified children with trisomy 13 or 18 with interventions for CHD between 1982 and 2008. Long-term survival and cause of death were obtained through linkage with the National Death Index. RESULTS: A total of 50 patients with trisomy 13 and 121 patients with trisomy 18 were enrolled in PCCC between 1982 and 2008; among them 29 patients with trisomy 13 and 69 patients with trisomy 18 underwent intervention for CHD...
June 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28425226/a-review-of-pediatric-pulmonary-hypertension-with-new-guidelines
#9
Serdar Kula, Ayhan Pektaş
This study aims to review pediatric pulmonary hypertension (PH) by comparing the guidelines of the European Society of Cardiology (ESC)/European Respiratory Society (ERS), the American Heart Association (AHA)/American Thoracic Society (ATS), and the European Pediatric Pulmonary Vascular Disease Network (EPPVDN). All three sets of guidelines define PH as having a mean pulmonary artery pressure of ≥25 mmHg and accept the validity of the World Health Organization (WHO) classification system. Every child with a high index of suspicion for PH should undergo an initial work-up of chest X-rays, electrocardiography, and echocardiography...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28416430/-extracorporeal-membrane-oxygenation-in-critically-ill-neonates-and-children
#10
J Rambaud, J Guilbert, I Guellec, S Jean, A Durandy, M Demoulin, A Amblard, R Carbajal, P-L Leger
Extracorporeal membrane oxygenation is used as a last resort during neonatal and pediatric resuscitation in case of refractory circulatory or respiratory failure under maximum conventional therapies. Different types of ECMO can be used depending on the initial failure. The main indications for ECMO are refractory respiratory failure (acute respiratory distress syndrome, status asthmaticus, severe pneumonia, meconium aspiration syndrome, pulmonary hypertension) and refractory circulatory failure (cardiogenic shock, septic shock, refractory cardiac arrest)...
April 14, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28377467/safety-with-echocardiographic-contrast-agents
#11
REVIEW
Preetham R Muskula, Michael L Main
In October 2007, the Food and Drug Administration mandated significant revisions to product labeling for the commercially available echocardiographic contrast agents (ECA) Definity and Optison after spontaneous healthcare provider reports of 4 patient deaths and ≈190 severe cardiopulmonary reactions occurring in close temporal relationship to ECA administration. Since then, multiple large ECA safety studies have been published and have included outpatients, hospitalized patients (including the critically ill), patients undergoing stress echocardiography, and patients with pulmonary hypertension...
April 2017: Circulation. Cardiovascular Imaging
https://www.readbyqxmd.com/read/28375639/prognostic-significance-of-reduced-blood-pressure-response-to-exercise-in-pediatric-pulmonary-arterial-hypertension
#12
Hong-Da Zhang, Zi-Chao Lv, Li-Ting Wang, Alexander Rothman, Tian-Yu Lian, Yang-Yang He, Yan Wu, Allan Lawrie, Maurice Beghetti, Zhi-Cheng Jing
No abstract text is available yet for this article.
April 4, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28338487/the-evolution-of-disease-chronic-lung-disease-of-infancy-and-pulmonary-hypertension
#13
Michael C Tracy, David N Cornfield
PURPOSE OF REVIEW: Bronchopulmonary dysplasia (BPD) or chronic lung disease of infancy BPD was originally described 50 years ago, in 1967 by Northway et al. This article possesses two fundamental objectives to provide: a brief historical perspective on BPD; and an update relative to current notions of epidemiology, pathophysiology, evaluation, and clinical management of BPD complicated by vascular disease. The review highlights areas of consensus and ongoing uncertainty. RECENT FINDINGS: The clinical cause and presentation of infants with BPD has evolved over the past several decades...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#14
Michaël Levy, Nolwenn Le Sache, Mostafa Mokhtari, Guy Fagherazzi, Gaelle Cuzon, Benjamin Bueno, Virginie Fouquet, Alexandra Benachi, Sergio Eleni Dit Trolli, Pierre Tissieres
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. METHODS: This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28301910/soluble-fms-like-tyrosine-kinase-1-as-a-link-between-angiogenesis-and-endothelial-dysfunction-in-pediatric-patients-with-%C3%AE-thalassemia-intermedia
#15
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Omneya Ibrahim Youssef, Mohamed ElSayed Ali
Endothelial damage has been implicated in the pathogenesis of vascular complications in β-thalassemia intermedia (β-TI). Soluble fms-like tyrosine kinase 1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. Soluble fms-like tyrosine kinase 1 is an antiangiogenic protein that induces endothelial dysfunction by adhering to and inhibiting VEGF and placenta growth factor. The aim of this study was to assess the level of sFLT-1 in 35 children and adolescents with β-TI, correlating it with markers of hemolysis and iron overload as well as cardiopulmonary complications...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28296782/the-safety-and-efficacy-of-dexmedetomidine-remifentanil-in-children-undergoing-flexible-bronchoscopy-a-retrospective-dose-finding-trial
#16
Xia Li, Xue Wang, Shuguang Jin, Dongsheng Zhang, Yanuo Li
Flexible bronchoscopy is more and more used for diagnosis and management of various pulmonary diseases in pediatrics. As poor coordination of children, the procedure is usually performed under general anesthesia with spontaneous or controlled ventilation to increase children and bronchoscopists' safety and comfort. Previous studies have reported that dexmedetomidine (DEX) could be safely and effectively used for flexible bronchoscopy in both adulate and children. However, there is no trial to evaluate the dose-finding of safety and efficacy of dexmedetomidine-remifentanil (DEX-RF) in children undergoing flexible bronchoscopy...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28271596/team-based-approach-to-identify-cardiac-toxicity-in-critically-ill-hematopoietic-stem-cell-transplant-recipients
#17
Christopher E Dandoy, Sonata Jodele, Zachary Paff, Russel Hirsch, Thomas D Ryan, John L Jefferies, Michelle Cash, Seth Rotz, Abigail Pate, Michael D Taylor, Javier El-Bietar, Kasiani C Myers, Gregory Wallace, Adam Nelson, Michael Grimley, Thomas Pfeiffer, Adam Lane, Stella M Davies, Ranjit S Chima
INTRODUCTION: We observed pulmonary hypertension (PH), pericardial effusions, and left ventricular systolic dysfunction (LVSD) in multiple critically ill hematopoietic stem cell transplant (HSCT) recipients. We implemented routine structured echocardiography screening for HSCT recipients admitted to the pediatric intensive care unit (PICU) using a standardized multidisciplinary process. METHODS: HSCT recipients admitted to the PICU with respiratory distress, hypoxia, shock, and complications related to transplant-associated thrombotic microangiopathy were screened on admission and every 1-2 weeks thereafter...
March 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28247848/relation-of-cytokine-profile-to-clinical-and-hemodynamic-features-in-young-patients-with-congenital-heart-disease-and-pulmonary-hypertension
#18
Leína Zorzanelli, Nair Maeda, Mariana Clavé, Ana Thomaz, Filomena Galas, Marlene Rabinovitch, Antonio Lopes
In congenital heart disease, severity of pulmonary hypertension and operability is defined by noninvasive parameters (clinical history, physical examination, and echocardiography) and sometimes, cardiac catheterization. We investigated how circulating levels of inflammatory mediators correlate with such parameters in a young pediatric population (age, 2.0 months to 3.1 years) and the effects of preoperative pulmonary vasodilator therapy with sildenafil. Cytokines were analyzed in serum using chemiluminescence signals...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28246340/tsujii-n-shiraishi-i-kokame-k-et-al-severe-hemolysis-and-pulmonary-hypertension-in-a-neonate-with-upshaw-schulman-syndrome-pediatrics-2016-138-6-e20161565
#19
(no author information available yet)
No abstract text is available yet for this article.
March 2017: Pediatrics
https://www.readbyqxmd.com/read/28235796/pulmonary-hypertension-therapy-and-a-systematic-review-of-efficacy-and-safety-of-pde-5-inhibitors
#20
REVIEW
Chinwe Unegbu, Corina Noje, John D Coulson, Jodi B Segal, Lewis Romer
Pulmonary hypertension (PH) is a syndrome that is of growing concern to pediatricians worldwide. Recent data led to concerns about the safety of phosphodiesterase type 5 (PDE5) inhibitors in children and a US Food and Drug Administration safety advisory. Our objective is to provide insight into therapies for PH in children and to systematically review the comparative effectiveness and safety of PDE5 inhibitors in the management of pediatric patients with PH. We searched the following databases through February 2015: Medline, Embase, SCOPUS, and the Cochrane Central Register of Controlled Trials...
March 2017: Pediatrics
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