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Heart disease in children

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https://www.readbyqxmd.com/read/27919346/disease-control-via-intensified-lipoprotein-apheresis-in-three-siblings-with-familial-hypercholesterolemia
#1
Christina Taylan, Andrea Schlune, Thomas Meissner, Karolis Ažukaitis, Floris E A Udink Ten Cate, Lutz T Weber
BACKGROUND: Familial hypercholesterolemia (FH), the prevalent monogenic form of hypercholesterolemia, carries the risk of premature coronary heart disease. Lipoprotein-apheresis is established in children with severe dyslipidemia. We present 3 siblings with a negative/negative residual low-density lipoprotein (LDL) receptor mutation (p.Trp577Arg), unresponsive to drug treatment. OBJECTIVE: Intensified lipoprotein-apheresis is well tolerated and results in permanently low lipid values without harming the health-related quality of life in children...
November 2016: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/27918384/twelve-years-of-kawasaki-disease-in-portugal-epidemiology-in-hospitalized-children
#2
Fátima F Pinto, Sérgio Laranjo, Miguel Mota Carmo, Maria João Brito, Rui Cruz Ferreira
BACKGROUND: Kawasaki disease (KD) became the leading cause of acquired heart disease in developed countries. Reported incidences vary worldwide, but it hasn't been established in Portugal. AIM: To describe the epidemiologic characteristics and estimate incidence rates of KD among hospitalized children in Portugal. METHODS: Descriptive, population-based study, using hospital discharge records with KD diagnosis, for patients <20 years selected from the Hospital Register database from 2000-2011...
November 30, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/27916446/gastrointestinal-complications-associated-with-the-surgical-treatment-of-heart-disease-in-children
#3
Lee P Ferguson, Tarriyo Gandiya, Christos Kaselas, Jigna Sheth, Asif Hasan, Hany Os Gabra
BACKGROUND/PURPOSE: The gastrointestinal system is prone to complications following heart surgery. We sought to determine the incidence and factors associated with gastrointestinal complication after cardiac surgery in children. METHODS: A retrospective review of patients aged <16years that underwent cardiac surgery between 2009 and 2013. Primary outcome was occurrence of gastrointestinal complication within 30days. Multivariable logistic regression was performed to identify variables related to occurrence of gastrointestinal complication...
November 14, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27913750/congenital-heart-block-and-immune-mediated-sensorineural-hearing-loss-possible-cross-reactivity-of-immune-response
#4
C Bason, I Pagnini, A Brucato, S Maestroni, A Puccetti, C Lunardi, R Cimaz
Immune-mediated sensorineural hearing loss may complicate systemic autoimmune diseases. We have previously reported the presence of antibodies directed against inner ear antigens in patients with Cogan syndrome, a disease characterized by sudden hearing loss and interstitial keratitis. Such autoantibodies cross-react with an epitope of SSA/Ro60 protein. Anti-Ro/SSA antibodies in pregnant women cross the placenta and reach the fetal tissues inducing an immune-mediated damage of the cardiac conduction system...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27912926/acute-management-of-refractory-and-unstable-pediatric-supraventricular-tachycardia
#5
Jonathan Lewis, Gaurav Arora, Dana L Tudorascu, Robert W Hickey, Richard A Saladino, Mioara D Manole
OBJECTIVE: To characterize the management of acute pediatric supraventricular tachycardia (SVT), placing special emphasis on infants, patients refractory to adenosine (refractory SVT), and patients with hypotension, poor perfusion, or altered mental status (unstable SVT). STUDY DESIGN: Retrospective cohort study of patients 0-18 years of age without congenital heart disease who presented to our pediatric hospital from January 2003 to December 2012 for the treatment of acute SVT...
November 22, 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27904570/existence-of-mutations-in-the-homeodomain-encoding-region-of-nkx2-5-gene-in-iranian-patients-with-tetralogy-of-fallot
#6
Majid Kheirollahi, Fereshteh Khosravi, Saeideh Ashouri, Alireza Ahmadi
BACKGROUND: Tetralogy of Fallot (TOF), the most common cyanotic heart defect and one of the most common congenital heart diseases, occurs mostly sporadically and nonsyndromically. The underlying molecular genetic mechanism is not known. Therefore, the existence of mutations in the homeodomain-encoding region of NKX2.5 gene in Iranian patients with tetralogy of Fallot is evaluated. MATERIALS AND METHODS: In the present study, we analyzed the peripheral blood samples of27 patients in order to find any mutation in the 180 bp homeodomain-encoding region of NKX2...
2016: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/27901294/performance-of-functional-fibrinogen-thromboelastography-in-children-undergoing-congenital-heart-surgery
#7
Nischal K Gautam, Chunyan Cai, Olga Pawelek, Muhammad B Rafique, Davide Cattano, Evan G Pivalizza
BACKGROUND: Functional Fibrinogen assay of the Thromboelastography (FFTEG), a whole blood viscoelastic hemostatic assay, has been used to estimate fibrinogen levels in adult patients undergoing major surgery but its performance in pediatric patients undergoing cardiac surgery requires evaluation. In this study, we evaluate the correlation between FFTEG parameters and standard laboratory tests for fibrinogen and platelet counts before and after cardiopulmonary bypass in children undergoing repair for congenital heart disease...
November 30, 2016: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/27899865/necrotizing-pneumonia-caused-by-h1n1-virus-in-a-child-with-total-anomalous-pulmonary-venous-connection-after-cardiac-surgery
#8
Mehmet G Ramoğlu, Tayfun Uçar, Tanıl Kendirli, Zeynep Eyileten, Semra Atalay
Total anomalous pulmonary venous connection is a rare form of congenital heart disease, occurring in only 1.5% of children with congenital heart disease. Although the mortality and morbidity of total anomalous pulmonary venous connection have decreased dramatically due to improvements in surgery, postoperative pulmonary venous obstruction is still a cause of late mortality in patients with corrected total anomalous pulmonary venous connection. Influenza A H1N1, the most common cause of human influenza in 2009, may cause pneumonia presenting with increased disease severity...
November 2016: Acta Cardiologica Sinica
https://www.readbyqxmd.com/read/27898687/effects-of-chronologic-age-and-young-child-exposure-on-respiratory-syncytial-virus-disease-among-us-preterm-infants-born-at-32-to-35-weeks-gestation
#9
Eric A F Simões, Evan J Anderson, Xionghua Wu, Christopher S Ambrose
OBJECTIVE: To estimate the incidence of respiratory syncytial virus (RSV) disease as a function of chronologic age and exposure to young children in US preterm infants. METHODS: In the RSV Respiratory Events among Preterm Infants Outcomes and Risk Tracking (REPORT) study, preterm infants born at 32-35 weeks gestational age (wGA) were enrolled from 188 US clinics and followed September-May of 2009-2010 or 2010-2011. Infants with medically-attended acute respiratory illness had nasal/pharyngeal swabs collected for viral testing...
2016: PloS One
https://www.readbyqxmd.com/read/27898601/fontan-fenestration-closure-prior-to-posterior-spinal-fusion-in-patients-with-single-ventricle-heart-disease
#10
Matthew C Schwartz, David Nykanen, Craig Fleishman, Hamish M Munro, Jonathan Phillips, Raymond D Knapp, Donald Felix
STUDY DESIGN: Case series. OBJECTIVE: To describe transcatheter closure of the Fontan fenestration prior to posterior spinal fusion in two children to prevent paradoxical venous air embolism during the operation. SUMMARY OF BACKGROUND DATA: Scoliosis is common among patients with single-ventricle congenital heart disease who have undergone Fontan operation and spinal surgery can offer physiologic benefits. Venous air embolism is a rare, but important reported complication during spinal surgery performed in the prone position...
December 1, 2016: Spine
https://www.readbyqxmd.com/read/27894758/the-impact-of-cardiac-risk-factors-on-short-term-outcomes-for-children-undergoing-a-ladd-procedure
#11
Luke R Putnam, Kathryn T Anderson, KuoJen Tsao, Lillian S Kao, Jane A Lugo, Kevin P Lally, Akemi L Kawaguchi
BACKGROUND/PURPOSE: The purpose of this study was to describe the outcomes of children with and without congenital heart disease who undergo a Ladd procedure. METHODS: The 2012-2014 National Surgical Quality Improvement Program Pediatric (NSQIP-P) data were queried for patients undergoing a Ladd procedure. Utilizing NSQIP-P definitions, patients were categorized into four cardiac risk groups (none, minor, major, severe) based on severity of cardiac anomalies, previous cardiac procedure(s), and ongoing cardiac dysfunction...
October 21, 2016: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/27887664/translating-sickle-cell-guidelines-into-practice-for-primary-care-providers-with-project-echo
#12
Lisa M Shook, Christina B Farrell, Karen A Kalinyak, Stephen C Nelson, Brandon M Hardesty, Angeli G Rampersad, Kay L Saving, Wanda J Whitten-Shurney, Julie A Panepinto, Russell E Ware, Lori E Crosby
BACKGROUND: Approximately 100,000 persons with sickle cell disease (SCD) live in the United States, including 15,000 in the Midwest. Unfortunately, many patients experience poor health outcomes due to limited access to primary care providers (PCPs) who are prepared to deliver evidence-based SCD care. Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional network established to improve care and outcomes for individuals with SCD living in Indiana, Illinois, Michigan, Minnesota, Ohio, and Wisconsin...
2016: Medical Education Online
https://www.readbyqxmd.com/read/27885814/trends-microbiology-and-outcomes-of-infective-endocarditis-in-children-during-2000-2010-in-the-united-states
#13
Shipra Gupta, Ankit Sakhuja, Eric McGrath, Basim Asmar
BACKGROUND: We studied the incidence, trend, underlying conditions, microbiology, and outcomes of infective endocarditis (IE) in children during 11 years using Nationwide Inpatient Sample (NIS) database. This is the largest all-payer inpatient care database in the United States containing data for more than 8 million hospital stays from over 1000 hospitals. METHODS: NIS data from 2000 to 2010 of primary discharge diagnosis of IE in children aged ≤19 years old were studied...
November 25, 2016: Congenital Heart Disease
https://www.readbyqxmd.com/read/27883978/outcomes-of-borderline-rheumatic-heart-disease-a-prospective-cohort-study
#14
Geneviève Bertaina, Bernard Rouchon, Bertrand Huon, Nina Guillot, Corinne Robillard, Baptiste Noël, Marie Nadra, Christophe Tribouilloy, Eloi Marijon, Xavier Jouven, Mariana Mirabel
CONTEXT AND AIMS: The advent of systematic screening for rheumatic heart disease (RHD) by echocardiography in endemic regions has led to a new entity: borderline RHD. The pathogenicity and natural history of borderline RHD needs to be addressed. The aim of this study was to assess the outcomes of children detected by echocardiography as having borderline RHD. METHODS: Schoolchildren in 4th grade (i.e., aged 9-10years) who were prospectively echo-screened for RHD (2012-2014) in Nouméa, New Caledonia, were asked to participate...
November 14, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27878778/elevated-serum-levels-of-ghrelin-and-tnf-%C3%AE-in-patients-with-cyanotic-and-acyanotic-congenital-heart-disease
#15
Sai Zhang, Gong-Liang Guo, Li-Li Yang, Li-Qun Sun
BACKGROUND: The levels of ghrelin and tumor necrosis factor alpha (TNF-α) are considered biological markers of congenital heart diseases (CHD). The present meta-analysis was conducted to investigate the clinical significance of serum levels of ghrelin and TNF-α in children with (CHD). METHODS: Chinese and English scientific literature databases were searched to retrieve published studies relevant to ghrelin, TNF-α and CHD. Manual search was additionally employed to identify other relevant studies from cross-references...
November 23, 2016: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/27878634/relationship-between-habitual-exercise-and-performance-on-cardiopulmonary-exercise-testing-differs-between-children-with-single-and-biventricular-circulations
#16
Michael L O'Byrne, Sanyukta Desai, Megan Lane, Michael McBride, Stephen Paridon, Elizabeth Goldmuntz
Increasing habitual exercise has been associated with improved cardiopulmonary exercise testing (CPET) performance, specifically maximal oxygen consumption in children with operatively corrected congenital heart disease. This has not been studied in children following Fontan palliation, a population in whom CPET performance is dramatically diminished. A single-center cross-sectional study with prospective and retrospective data collection was performed that assessed habitual exercise preceding a clinically indicated CPET in children and adolescents with Fontan palliation, transposition of the great arteries following arterial switch operation (TGA), and normal cardiac anatomy without prior operation...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27878628/global-deformation-parameters-response-to-exercise-in-adolescents-with-repaired-tetralogy-of-fallot
#17
Timur Mese, Baris Guven, Murat Muhtar Yilmazer, Mustafa Demirol, Şenay Çoban, Cem Karadeniz
Exercise stress echocardiography is rarely used in the follow-up of children with congenital heart disease following surgical repair. There are no data on global longitudinal strain (GLS) and global circumferential strain (GCS) during exercise in adolescents with repaired tetralogy of Fallot (TOF). The purpose of this prospective study was to investigate GLS and GCS using speckle tracking two-dimensional echocardiography (2D-STE) at rest and during exercise in adolescents with repaired TOF. Twenty patients with repaired TOF and 20 controls were included...
November 23, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27872994/outcomes-of-tracheostomy-in-children-requiring-surgery-for-congenital-heart-disease
#18
Laura A Ortmann, Winston M Manimtim, Charisse I Lachica
Outcomes after discharge in children requiring tracheostomy after cardiac surgery have not been fully described. A retrospective, single-center study was performed on all children <18 years of age requiring both tracheostomy and surgery for congenital heart disease from January 2002 to May 2015. Forty-six tracheostomies were placed after surgery and four before. Single-ventricle anatomy was present in 12 (33%) patients. Incidence of tracheostomy after heart surgery increased from 0.8% the first half of the study period to 2% the second half...
November 21, 2016: Pediatric Cardiology
https://www.readbyqxmd.com/read/27871578/children-with-heart-disease-risk-stratification-for-non-cardiac-surgery
#19
Angela K Saettele, Jacob L Christensen, Kelly L Chilson, David J Murray
STUDY OBJECTIVE: Children with congenital or acquired heart disease have an increased risk of anesthesia related morbidity and mortality. The child's anesthetic risk is related to the severity of their underlying cardiac disease, associated comorbidities, and surgical procedure. The goal of this project was to determine the ease of use of a preoperative risk stratification tool for assigning pediatric cardiac staff and to determine the relative frequency that children with low, moderate, and high risk cardiac disease present for non-cardiac surgery at a tertiary pediatric hospital...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27871282/a-mother-s-choice-a-qualitative-study-of-mothers-health-seeking-behaviour-for-their-children-with-acute-diarrhoea
#20
Lucy Cunnama, Ayako Honda
BACKGROUND: Diarrhoea presents a considerable health risk to young children and is one of the leading causes of infant mortality. Although proven cost-effective interventions exist, South Africa is yet to reach the Sustainable Development Goals set for the elimination of preventable under-five mortality and water-borne diseases. The rural study area in the Eastern Cape of South Africa continues to have a parallel health system comprising traditional and modern healthcare services. It is in this setting that this study aimed to qualitatively examine the beliefs surrounding and perceived quality of healthcare accessed for children's acute diarrhoea...
November 21, 2016: BMC Health Services Research
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