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https://www.readbyqxmd.com/read/28100970/sickle-cell-disease-with-cyanotic-congenital-heart-disease-long-term-outcomes-in-5-children
#1
Glen J Iannucci, Olufolake A Adisa, Matthew E Oster, Michael McConnell, William T Mahle
Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction...
December 2016: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28099323/anesthesia-and-databases-pediatric-cardiac-disease-as-a-role-model
#2
David F Vener, Sara K Pasquali, Emad B Mossad
Large data sets have now become ubiquitous in clinical medicine; they are particularly useful in high-acuity, low-volume conditions such as congenital heart disease where data must be collected from many centers. These data fall into 2 categories: administrative data arising from hospital admissions and charges and clinical data relating to specific diseases or procedures. In congenital cardiac diseases, there are now over a dozen of these data sets or registries focusing on various elements of patient care...
February 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28098429/natural-history-of-nonimmune-mediated-thrombocytopenia-and-acute-kidney-injury-in-pediatric-open-heart-surgery
#3
Shannon Tew, Manuel L Fontes, Nathaniel H Greene, Miklos D Kertai, George Ofori-Amanfo, Robert D B Jaquiss, Andrew J Lodge, Warwick A Ames, Hercilia Mayumi Homi, Kelly A Machovec, Edmund H Jooste
OBJECTIVE: Thrombocytopenia and acute kidney injury (AKI) are common following pediatric cardiac surgery with cardiopulmonary bypass (CPB). However, the relationship between postoperative nadir platelet counts and AKI has not been investigated in the pediatric population. Our objective was to investigate this relationship and examine independent predictors of AKI. DESIGN: After IRB approval, we performed a retrospective review of the institution's medical records and database...
January 18, 2017: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/28098235/molecular-characterization-of-pediatric-restrictive-cardiomyopathy-from-integrative-genomics
#4
Tara N Rindler, Robert B Hinton, Nathan Salomonis, Stephanie M Ware
Pediatric restrictive cardiomyopathy (RCM) is a genetically heterogeneous heart disease with limited therapeutic options. RCM cases are largely idiopathic; however, even within families with a known genetic cause for cardiomyopathy, there is striking variability in disease severity. Although accumulating evidence implicates both gene expression and alternative splicing in development of dilated cardiomyopathy (DCM), there have been no detailed molecular characterizations of underlying pathways dysregulated in RCM...
January 18, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28097735/autoimmune-enteropathy-and-hepatitis-in-pediatric-heart-transplant-recipient
#5
Kimberly Lewis, Ryan Butts, J Antonio Quiros, Michelle Hudspeth, Katherine Twombley, Andrew Savage, Sally Self, Ali Burnette, Shaoli Sun
AIE is a rare disorder in children that presents with severe diarrhea and malabsorption, caused by immune-mediated damage to intestinal mucosa. AIE is often associated with various syndromes of immunodeficiency including IPEX syndrome (immune dysregulation, polyendocrinopathy and enteropathy, X-linked). Dysfunctional T regulatory cells are the source of pathology in both IPEX syndrome and AIE as they are essential in maintaining tolerance to self-antigens and eliminating autoreactive B cells. This case report describes a 10-year-old cardiac transplant and total thymectomy patient on chronic immunosuppression with tacrolimus that presented with AIE and extraintestinal manifestations of cyclical hepatitis...
January 17, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28097194/the-mystery-of-the-z-score
#6
REVIEW
Alexander E Curtis, Tanya A Smith, Bulat A Ziganshin, John A Elefteriades
Reliable methods for measuring the thoracic aorta are critical for determining treatment strategies in aneurysmal disease. Z-scores are a pragmatic alternative to raw diameter sizes commonly used in adult medicine. They are particularly valuable in the pediatric population, who undergo rapid changes in physical development. The advantage of the Z-score is its inclusion of body surface area (BSA) in determining whether an aorta is within normal size limits. Therefore, Z-scores allow us to determine whether true pathology exists, which can be challenging in growing children...
August 2016: Aorta (Stamford, Conn.)
https://www.readbyqxmd.com/read/28095022/estimating-effective-dose-of-radiation-from-pediatric-cardiac-ct-angiography-using-a-64-mdct-scanner-new-conversion-factors-relating-dose-length-product-to-effective-dose
#7
Sigal Trattner, Anjali Chelliah, Peter Prinsen, Carrie B Ruzal-Shapiro, Yanping Xu, Sachin Jambawalikar, Maxwell Amurao, Andrew J Einstein
OBJECTIVE: The purpose of this study is to determine the conversion factors that enable accurate estimation of the effective dose (ED) used for cardiac 64-MDCT angiography performed for children. MATERIALS AND METHODS: Anthropomorphic phantoms representative of 1- and 10-year-old children, with 50 metal oxide semiconductor field-effect transistor dosimeters placed in organs, underwent scanning performed using a 64-MDCT scanner with different routine clinical cardiac scan modes and x-ray tube potentials...
January 17, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28094679/a-retrospective-analysis-of-a-pediatric-tele-echocardiography-service-to-treat-triage-and-reduce-trans-pacific-transport
#8
Christopher A Rouse, Brandon T Woods, C Becket Mahnke
Introduction Tele-echocardiography can ensure prompt diagnosis and prevent the unnecessary transport of infants without critical congenital heart disease, particularly at isolated locations lacking access to tertiary care medical centers. Methods We retrospectively reviewed all infants who underwent tele-echocardiography at a remote 16-bed level IIIB NICU from June 2005 to March 2014. Tele-echocardiograms were completed by cardiac sonographers in Okinawa, Japan, and transmitted asynchronously for review by pediatric cardiologists in Hawaii...
January 1, 2017: Journal of Telemedicine and Telecare
https://www.readbyqxmd.com/read/28093988/adherence-to-secondary-prophylaxis-for-acute-rheumatic-fever-and-rheumatic-heart-disease-a-systematic-review
#9
Priya Minaxi Kevat, Benjamin M Reeves, Alan R Ruben, Ronny Gunnarsson
BACKGROUND: Optimal delivery of regular benzathine penicillin G (BPG) injections prescribed as secondary prophylaxis for acute rheumatic fever (ARF) and rheumatic heart disease (RHD) is vital to preventing disease morbidity and cardiac sequelae in affected pediatric and young adult populations. However, poor uptake of secondary prophylaxis remains a significant challenge to ARF/RHD control programs. OBJECTIVE: In order to facilitate better understanding of this challenge and thereby identify means to improve service delivery, this systematic literature review explored rates of adherence and factors associated with adherence to secondary prophylaxis for ARF and RHD worldwide...
January 16, 2017: Current Cardiology Reviews
https://www.readbyqxmd.com/read/28092429/residents-understanding-of-adult-congenital-heart-disease
#10
Paul Cooper, Morgan Hindes, Timothy M Maul, Stephen C Cook
OBJECTIVE: Medical residents are exposed to increasing numbers of adults with congenital heart disease (ACHD). While inadequate ACHD knowledge may lead to inappropriate practice, this educational deficit has not been investigated. Our aim was to analyze residents' attitudes, perceived ability, and knowledge of ACHD medicine. DESIGN, METHODS, OUTCOME MEASURES: A single center, multiprogram cross-sectional study was conducted in 2015 using an electronic survey to assess 472 medical residents' perceived knowledge and self-assessed skills related to ACHD medicine...
January 16, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28092064/critical-congenital-heart-disease-newborn-screening-implementation-lessons-learned
#11
Monica R McClain, John S Hokanson, Regina Grazel, Kim Naarden Van Braun, Lorraine F Garg, Michelle R Morris, Kathleen Moline, Keri Urquhart, Amy Nance, Harper Randall, Marci K Sontag
Introduction The purpose of this article is to present the collective experiences of six federally-funded critical congenital heart disease (CCHD) newborn screening implementation projects to assist federal and state policy makers and public health to implement CCHD screening. Methods A qualitative assessment and summary from six demonstration project grantees and other state representatives involved in the implementation of CCHD screening programs are presented in the following areas: legislation, provider and family education, screening algorithms and interpretation, data collection and quality improvement, telemedicine, home and rural births, and neonatal intensive care unit populations...
January 16, 2017: Maternal and Child Health Journal
https://www.readbyqxmd.com/read/28091832/utility-and-safety-of-the-safesept%C3%A2-transseptal-guidewire-for-electrophysiology-studies-with-catheter-ablation-in-pediatric-and-congenital-heart-disease
#12
Joseph J Knadler, Jeffrey B Anderson, Ahmad S Chaouki, Richard J Czosek, Chad Connor, Timothy K Knilans, David S Spar
PURPOSE: The atrial transseptal procedure is used in catheter ablation of left-sided arrhythmias. Studies in adult patients have shown the SafeSept™ transseptal guidewire (SSTG) to be effective in atrial transseptal procedures. We analyzed our 5-year experience with SSTG use in pediatric and congenital heart disease patients undergoing catheter ablation. METHODS: This is a single-center retrospective analysis of patients undergoing catheter ablation from 2009 to 2014...
January 14, 2017: Journal of Interventional Cardiac Electrophysiology: An International Journal of Arrhythmias and Pacing
https://www.readbyqxmd.com/read/28087255/unknown-face-of-known-drugs-what-else-can-we-expect-from-angiotensin-converting-enzyme-inhibitors
#13
REVIEW
Anna Wzgarda, Robert Kleszcz, Monika Prokop, Katarzyna Regulska, Milosz Regulski, Jaroslaw Paluszczak, Beata J Stanisz
The renin-angiotensin system (RAS) is one of important systems among homeostatic mechanisms that controls the function of cardiovascular, renal and adrenal systems. As RAS has a very complex nature, it has been also found as related to the control of cell migration and apoptosis. Angiotensin-converting enzyme inhibitors (ACEI) are drugs most commonly used in the modulation of RAS activity. ACEI have been extensively described as effective in the treatment of hypertension among adults, but also as drugs delaying progression in diabetic nephropathy and reducing mortality in left ventricular dysfunction and congestive heart failure...
January 10, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28079836/symptomatic-cytomegalovirus-infections-in-the-first-year-of-life-when-is-antiviral-therapy-conceived-to-be-justified
#14
Katharina Schuster, Rangmar Goelz, Carsten Speckmann, Philipp Henneke
All infants treated with antiviral medication for symptomatic congenital (diagnosis <3 weeks) and probably postnatal (>3 weeks) cytomegalovirus infection were characterized with the help of a survey covering all German Pediatric hospitals between 2012 and 2013. We found that >50% of infants treated for cytomegalovirus were classified as probably postnatal cytomegalovirus infection, which was associated with preterm birth and underlying diseases of the immune system, heart or lung.
February 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28074279/identification-and-clinical-course-of-166-pediatric-cardiac-tumors
#15
Lin Shi, Lanping Wu, Huijuan Fang, Bo Han, Jialun Yang, Xiaojin Ma, Fang Liu, Yongwei Zhang, Tingting Xiao, Min Huang, Meirong Huang
: The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors...
February 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28073682/study-design-for-control-of-heart-rate-in-infant-and-child-tachyarrhythmia-with-heart-failure-using-landiolol-heartful-a-prospective-multicenter-uncontrolled-clinical-trial
#16
Naokata Sumitomo, Hitoshi Horigome, Masaru Miura, Hiroshi Ono, Hideaki Ueda, Kiyohiro Takigiku, Jun Yoshimoto, Naoki Ohashi, Tsugutoshi Suzuki, Koichi Sagawa, Hiroya Ushinohama, Kazuhiro Takahashi, Aya Miyazaki, Heima Sakaguchi, Mari Iwamoto, Motoki Takamuro, Chiho Tokunaga, Tetsuji Nagano
BACKGROUND: Persistent tachycardia in pediatric patients after congenital heart surgery further deteriorates their hemodynamic condition, and may become fatal. Therefore, immediate control of the tachycardia is mandatory in these patients. For this purpose, quick-acting, short-acting, titratable intravenous agents are required. However, there are no agents with such characteristics among the drugs approved for control of pediatric arrhythmias in Japan, and thus novel and effective medications for these patients are awaited...
January 7, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/28073609/interaction-of-older-donor-age-and-survival-after-weight-matched-pediatric-heart-transplantation
#17
Thomas C Westbrook, David L S Morales, Muhammad S Khan, Roosevelt Bryant, Chesney Castleberry, Clifford Chin, Farhan Zafar
BACKGROUND: Donors are matched for weight in pediatric heart transplantation (PHT), yet age differences are not considered in this decision. In this study we attempt to identify the effect of age differences in weight-matched patients and the effect these differences have on post-transplant survival. METHODS: The United Network of Organ Sharing (UNOS) database was queried for the period from October 1987 to March 2014 for all pediatric heart transplant patients...
December 1, 2016: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28072745/safety-and-efficacy-of-chloral-hydrate-for-conscious-sedation-of-infants-in-the-pediatric-cardiovascular-intensive-care-unit
#18
Mei-Lian Chen, Qiang Chen, Fan Xu, Jia-Xin Zhang, Xiao-Ying Su, Xiao-Zhen Tu
This study evaluates the safety and efficacy of chloral hydrate administration for the conscious sedation of infants in the pediatric cardiovascular intensive care unit (PCICU).We conducted a retrospective review of the charts of 165 infants with congenital heart disease who received chloral hydrate in our PCICU between January 2014 and December 2014. Chloral hydrate was administered orally or rectally to infants using doses of 50 mg/kg. We collected and analyzed relevant clinical parameters.The overall length of time to achieve sedation was ranged from 5 to 35 min (10...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28070903/myocardial-deformation-in-pediatric-patients-with-mucopolysaccharidoses-a-two-dimensional-speckle-tracking-echocardiography-study
#19
Francesco Borgia, Enrica Pezzullo, Vincenzo Schiano Lomoriello, Regina Sorrentino, Francesco Lo Iudice, Sara Cocozza, Roberto Della Casa, Giancarlo Parenti, Pietro Strisciuglio, Bruno Trimarco, Maurizio Galderisi
BACKGROUND: Mucopolysaccharidoses (MPS) are inherited lysosomal storage disorders caused by deficiency of required glycosaminoglycans breakdown enzymes, inducing cardiac involvement. Little is known about myocardial deformation involvement in MPS. Our aim was to assess biventricular structure and function in asymptomatic children with MPS using standard echo Doppler and 2D speckle tracking (STE). METHODS: Fifteen MPS children (one type I, six type II, three type III A, one III B, three IV A, one VI), asymptomatic for cardiac symptoms, and 15 age and sex-matched healthy controls underwent echo Doppler and STE...
January 10, 2017: Echocardiography
https://www.readbyqxmd.com/read/28070248/childhood-cardiomyopathies-a-study-in-tertiary-care-hospital-in-upper-egypt
#20
Mohamed Abd Elaal Bakeet, Montaser Mohamed Mohamed, Ahmed Ahmed Allam, Rania Gamal
INTRODUCTION: Cardiomyopathy (CMP) is defined by the World Health Organization (WHO) as a disease of the myocardium associated with cardiac dysfunction. An understanding of CMP is very important, as it is a common cause of heart failure in children, and the most common indication for heart transplantation in children older than one year, but data on CMP in Egypt are scarce. The aim of this study was to determine the number, risk factors, clinical presentation, complications and outcome of different types of childhood cardiomyopathies in Sohag University Hospital...
November 2016: Electronic Physician
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