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Pediatric pulmonary hypertension

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https://www.readbyqxmd.com/read/28928561/periprocedural-management-of-vein-of-galen-aneurysmal-malformation-patients-an-11-year-experience
#1
Ajay Prasad Hrishi, Karen Ruby Lionel
CONTEXT: The vein of Galen aneurysmal malformation (VGAM) is a rare arteriovenous malformation where a dilated median prosencephalic vein provides a low-resistance conduit for intracerebral blood flow resulting in high-output cardiac failure, severe pulmonary hypertension, with or without central nervous system symptoms secondary to hydrocephalus, in the neonatal and pediatric population. AIM: This study aims to analysis of the anesthetic management of this unique subset of patients with VGAM...
July 2017: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/28922348/stem-cell-biology-and-regenerative-medicine-for-neonatal-lung-diseases
#2
REVIEW
Martin Kang, Bernard Thébaud
Lung diseases remain one of the main causes of morbidity and mortality in neonates. Cell therapy and regenerative medicine have the potential to revolutionize the management of life-threatening and debilitating lung diseases that currently lack effective treatments. Over the past decade, the repair capabilities of stem/progenitor cells has been harnessed to prevent/rescue lung damage in experimental neonatal lung diseases. Mesenchymal stromal cells and amnion epithelial cells exert pleiotropic effects and represent ideal therapeutic cells for bronchopulmonary dysplasia, a multifactorial disease...
September 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28917066/effectiveness-of-the-critical-congenital-heart-disease-screening-program-for-early-diagnosis-of-cardiac-abnormalities-in-newborn-infants
#3
Abdulmajid M Almawazini, Hamdi K Hanafi, Hasan A Madkhali, Noura B Majrashi
To evaluate the effectiveness of critical congenital heart disease (CCHD) screening program for early diagnosis of cardiac anomalies in newborn infants.  Methods: This is a hospital-based prospective cross-sectional study conducted in the Pediatric and Neonatology Department, King Fahad Hospital at  Albaha, Saudi Arabia, between February 2016 and February 2017. Results: We screened 2961 (95.4%) of 3103 patients in a nursery unit; 142 (4.6%) patients were not screened. The test was positive in 114 (3.9%) patients and negative in 2847 (96...
October 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28904266/clinical-impact-of-main-pulmonary-artery-dilatation-on-outcome-in-pediatric-idiopathic-and-heritable-pulmonary-arterial-hypertension
#4
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/28900614/echocardiographic-evaluation-of-pulmonary-pressures-and-right-ventricular-function-after-pediatric-cardiac-surgery-a-simple-approach-for-the-intensivist
#5
REVIEW
Maurice Beghetti
Pulmonary hypertension (PH) is diagnosed using cardiac catheterization and is defined as an elevation of mean pulmonary artery pressure (PAP) greater than 25 mmHg. Although invasive hemodynamics remains the gold standard and is mandatory for disease confirmation, transthoracic echocardiography (TTE) is an extremely useful non-invasive and widely available tool that allows for screening and follow-up, in particular, in the acute setting. TTE may be a valuable alternative, allowing for direct measurement and/or indirect assessment of PAP...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28874164/radiation-free-cmr-diagnostic-heart-catheterization-in-children
#6
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/28873355/pulmonary-interstitial-glycogenosis-associated-with-a-spectrum-of-neonatal-pulmonary-disorders
#7
Ernest Cutz, Rose Chami, Sharon Dell, Jacob Langer, David Manson
Primary or isolated pulmonary interstitial glycogenosis (PIG) is a rare disease presenting as tachypnea and hypoxemia during the perinatal period. A diffuse interstitial infiltrate with focal hyperinflation is visible on chest imaging. The biopsy findings include diffuse expansion of the interstitium by spindle-shaped cells with pale cytoplasm that, on electron microscopy (EM), are poorly differentiated mesenchymal cells containing abundant monoparticulate glycogen. This glycogenosis appears to be a transient abnormality, usually with a favorable prognosis...
September 2, 2017: Human Pathology
https://www.readbyqxmd.com/read/28872749/outstanding-contribution-to-pediatric-anesthesiology-an-interview-with-dr-robert-h-friesen
#8
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/28859030/treatment-of-refractory-infantile-hemangiomas-and-pulmonary-hypertension-with-sirolimus-in-a-pediatric-patient
#9
Kelley K Hutchins, Robert D Ross, Daisuke Kobayashi, Alissa Martin, Madhvi Rajpurkar
Infantile hemangioma is a benign vascular neoplasm that spontaneously involutes over time. Management, when needed, consists of medications, laser treatment and surgical excision. We describe a 3-year-old girl who presented shortly after birth with diffuse cutaneous hemangiomas, hepatosplenomegaly with liver lesions, anemia, and acute heart failure. She was diagnosed with hepatic and cutaneous infantile hemangioma based on skin biopsy. She developed progressive pulmonary hypertension with numerous pulmonary nodules suspicious for pulmonary arteriovenous malformations...
August 30, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28832796/transverse-sternal-split-a-safe-mini-invasive-approach-for-perventricular-device-closure-of-ventricular-septal-defect
#10
Pankaj Garg, Arvind Kumar Bishnoi, Ketav Lakhia, Jigar Surti, Sumbul Siddiqui, Parth Solanki, Himani Pandya
Objective: Perventricular device closure of ventricular septal defect through midline sternotomy avoids the cardiopulmonary bypass, however, lacks the cosmetic advantage. Perventricular device closure of ventricular septal defect with transverse split sternotomy was performed to add the cosmetic advantage of mini-invasive technique. Methods: Thirty-six pediatric patients with mean age 7.14±3.24 months and weight 5.00±0.88 kg were operated for perventricular device closure of ventricular septal defect through transverse split sternotomy in 4th intercostal space under transesophageal echocardiography guidance...
May 2017: Brazilian Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/28820870/controversies-in-the-identification-and-management-of-acute-pulmonary-hypertension-in-preterm-neonates
#11
REVIEW
Regan E Giesinger, Kiran More, Jodie Odame, Amish Jain, Robert P Jankov, Patrick J McNamara
It is increasingly recognized that the abnormal physiologic consequences of pulmonary hypertension (PH) may contribute to poor cardiopulmonary health in premature babies. Conflicting literature has led to clinical uncertainty, pathological misinterpretation and variability in treatment approaches among practitioners. There are several disorders with overlapping and interrelated presentations and other disorders with a similar clinical phenotype but diverse pathophysiological contributors. In this review, we provide a diagnostic approach for acute HRF in the preterm neonate, outline the pathophysiological conditions that may present as acute PH and discuss the implications of high pulmonary vascular resistance (PVR) on the cardiovascular system...
August 18, 2017: Pediatric Research
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/28819545/circulating-mirnas-in-pediatric-pulmonary-hypertension-show-promise-as-biomarkers-of-vascular-function
#13
Vitaly O Kheyfets, Carmen C Sucharov, Uyen Truong, Jamie Dunning, Kendall Hunter, Dunbar Ivy, Shelley Miyamoto, Robin Shandas
BACKGROUND/OBJECTIVES: The objective of this study was to evaluate the utility of circulating miRNAs as biomarkers of vascular function in pediatric pulmonary hypertension. METHOD: Fourteen pediatric pulmonary arterial hypertension patients underwent simultaneous right heart catheterization (RHC) and blood biochemical analysis. Univariate and stepwise multivariate linear regression was used to identify and correlate measures of reactive and resistive afterload with circulating miRNA levels...
2017: Oxidative Medicine and Cellular Longevity
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/28775006/uncovering-small-secrets-in-big-data-sets-how-math-can-identify-biology-in-rare-conditions-pediatric-pulmonary-hypertension
#15
EDITORIAL
Mardi Gomberg-Maitland, Rogerio Souza
No abstract text is available yet for this article.
August 4, 2017: Circulation Research
https://www.readbyqxmd.com/read/28737623/an-observational-study-of-severe-pertussis-in-100-infants-%C3%A2-120-days-of-age
#16
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
#17
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
#18
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 anti-epileptic 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...
October 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28702718/single-center-experience-using-selexipag-in-a-pediatric-population
#19
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
#20
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
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