keyword
MENU ▼
Read by QxMD icon Read
search

ebstein

keyword
https://www.readbyqxmd.com/read/28434546/surgical-strategy-toward-biventricular-repair-for-severe-ebstein-anomaly-in-neonates-and-infancy
#1
Shu-Chien Huang, En-Ting Wu, Shyh-Jye Chen, Chi-Hsiang Huang, Jin-Chung Shih, Hen-Wen Chou, Chung-I Chang, Ing-Sh Chiu, Yih-Sharng Chen
BACKGROUND: Neonates with severe forms of Ebstein anomaly present a surgical challenge, and the Starnes operation as single ventricle palliation is highly advocated. Cone reconstruction for tricuspid valvuloplasty (TVP) has become a widely accepted technique, although very few cases of TVP have been reported in neonates. This report describes a surgical strategy for neonatal Ebstein anomaly, with an aim toward biventricular repair. METHODS: Since 2007, 7 neonates or young infants with severe Ebstein anomalies have received TVP at the National Taiwan University Hospital, Taipei, Taiwan...
April 20, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28426384/a-2-year-old-boy-with-circulatory-failure-owing-to-streptococcal-toxic-shock-syndrome-case-report
#2
Werner Keenswijk, Johan Vande Walle
A 2-year-old boy presented with severe hypotension and acute kidney injury after a prodrome of non-bloody diarrhoea and fever in the preceding 3 days. He had a mild Ebstein cardiac anomaly but otherwise a normal past history and growth. On examination, he looked ill, his temperature was 37.5 °C, circulation was poor, and there were several purpuric lesions on the face, hands and scrotum. Haemoglobin was 7.8 g/dL (11-14), total white cell count 27 × 10(9)/L, platelets 62 × 10(9)/L, blood urea nitrogen 20...
April 20, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28410946/right-atrial-dysfunction-in-the-fetus-with-severely-regurgitant-tricuspid-valve-disease-a-potential-source-of-cardiovascular-compromise
#3
Lisa W Howley, Nee Scze Khoo, Anita J Moon-Grady, Sonali S Patel, Fayeza Alrais, Wayne Tworetzky, Timothy Colen, Paul Brooks, Jean Trines, Tiina Ojala, Lisa K Hornberger
BACKGROUND: In severe right heart obstruction (RHO), redistribution of cardiac output to the left ventricle (LV) is well tolerated by the fetal circulation. Although the same should be true of severely regurgitant tricuspid valve disease (rTVD) with reduced or no output from the right ventricle, affected fetuses more frequently develop hydrops or suffer intrauterine demise. We hypothesized that right atrium (RA) function is altered in rTVD but not in RHO, which could contribute to differences in outcomes...
April 11, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/28405996/ebstein-s-anomaly-with-paradoxical-embolism-to-lower-limb
#4
Gouranga Santra, Shinjan Patra
Ebstein's anomaly (EA) is a rare congenital heart disease. Paradoxical embolism (PDE) may occur in EA due to associated atrial septal defect (ASD). An 18 year old male with EA presented with left lower limb ischemia due to PDE to the limb. No features of cerebral embolism was present. PDE is a potential complication of EA. High level of awareness is needed for early diagnosis of PDE in EA to save a limb in case of embolism to the extremity.
December 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28388435/de-novo-disruption-of-the-proteasome-regulatory-subunit-psmd12-causes-a-syndromic-neurodevelopmental-disorder
#5
Sébastien Küry, Thomas Besnard, Frédéric Ebstein, Tahir N Khan, Tomasz Gambin, Jessica Douglas, Carlos A Bacino, William J Craigen, Stephan J Sanders, Andrea Lehmann, Xénia Latypova, Kamal Khan, Mathilde Pacault, Stephanie Sacharow, Kimberly Glaser, Eric Bieth, Laurence Perrin-Sabourin, Marie-Line Jacquemont, Megan T Cho, Elizabeth Roeder, Anne-Sophie Denommé-Pichon, Kristin G Monaghan, Bo Yuan, Fan Xia, Sylvain Simon, Dominique Bonneau, Philippe Parent, Brigitte Gilbert-Dussardier, Sylvie Odent, Annick Toutain, Laurent Pasquier, Deborah Barbouth, Chad A Shaw, Ankita Patel, Janice L Smith, Weimin Bi, Sébastien Schmitt, Wallid Deb, Mathilde Nizon, Sandra Mercier, Marie Vincent, Caroline Rooryck, Valérie Malan, Ignacio Briceño, Alberto Gómez, Kimberly M Nugent, James B Gibson, Benjamin Cogné, James R Lupski, Holly A F Stessman, Evan E Eichler, Kyle Retterer, Yaping Yang, Richard Redon, Nicholas Katsanis, Jill A Rosenfeld, Peter-Michael Kloetzel, Christelle Golzio, Stéphane Bézieau, Paweł Stankiewicz, Bertrand Isidor
No abstract text is available yet for this article.
April 6, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28361074/neonatal-ebstein-repair-where-are-we-now
#6
EDITORIAL
Philip L Wackel, Joseph A Dearani, Frank Cetta
No abstract text is available yet for this article.
March 2017: Annals of Translational Medicine
https://www.readbyqxmd.com/read/28343629/biallelic-variants-in-otud6b-cause-an-intellectual-disability-syndrome-associated-with-seizures-and-dysmorphic-features
#7
Teresa Santiago-Sim, Lindsay C Burrage, Frédéric Ebstein, Mari J Tokita, Marcus Miller, Weimin Bi, Alicia A Braxton, Jill A Rosenfeld, Maher Shahrour, Andrea Lehmann, Benjamin Cogné, Sébastien Küry, Thomas Besnard, Bertrand Isidor, Stéphane Bézieau, Isabelle Hazart, Honey Nagakura, LaDonna L Immken, Rebecca O Littlejohn, Elizabeth Roeder, Bulent Kara, Katia Hardies, Sarah Weckhuysen, Patrick May, Johannes R Lemke, Orly Elpeleg, Bassam Abu-Libdeh, Kiely N James, Jennifer L Silhavy, Mahmoud Y Issa, Maha S Zaki, Joseph G Gleeson, John R Seavitt, Mary E Dickinson, M Cecilia Ljungberg, Sara Wells, Sara J Johnson, Lydia Teboul, Christine M Eng, Yaping Yang, Peter-Michael Kloetzel, Jason D Heaney, Magdalena A Walkiewicz
Ubiquitination is a posttranslational modification that regulates many cellular processes including protein degradation, intracellular trafficking, cell signaling, and protein-protein interactions. Deubiquitinating enzymes (DUBs), which reverse the process of ubiquitination, are important regulators of the ubiquitin system. OTUD6B encodes a member of the ovarian tumor domain (OTU)-containing subfamily of deubiquitinating enzymes. Herein, we report biallelic pathogenic variants in OTUD6B in 12 individuals from 6 independent families with an intellectual disability syndrome associated with seizures and dysmorphic features...
April 6, 2017: American Journal of Human Genetics
https://www.readbyqxmd.com/read/28340054/outcomes-of-atrial-arrhythmia-radiofrequency-catheter-ablation-in-patients-with-ebstein-s-anomaly
#8
Abdallah Hassan, Nicholas Y Tan, Htin Aung, Heidi M Connolly, David O Hodge, Emily R Vargas, Bryan C Cannon, Douglas L Packer, Samuel J Asirvatham, Christopher J McLeod
Aims: Atrial arrhythmias are common in patients with Ebstein's anomaly (EA) despite cardiac surgical repair and concomitant Maze procedures. We aimed to evaluate the outcome of radiofrequency catheter ablation in this group of patients. Methods and results: All patients with EA and atrial arrhythmias who underwent catheter ablation for atrial arrhythmias between 1/1999 and 1/2016 were included. Atrial arrhythmia recurrence was identified as the primary outcome; secondary outcomes included repeat ablation, need for antiarrhythmic medications after ablation, and death...
February 27, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28316109/airway-inflammation-and-symptoms-in-children-following-liver-and-heart-transplantation
#9
Andrew J Redmann, John C Bucuvalas, Robert E Wood, Clifford Chin, Catherine K Hart
OBJECTIVES: To describe the upper airway endoscopic findings of children with upper airway symptoms after liver transplantation (LT) or heart transplantation (HT). METHODS: Review of children undergoing airway endoscopy after LT or HT from 2011 to 2015 at a tertiary care pediatric hospital. Airway findings, biopsy results, immunosuppression, and Epstein-Barr virus (EBV) levels were recorded. RESULTS: Twenty-three of 158 LT (111) and HT (47) recipients underwent endoscopy...
March 18, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28267539/assessment-of-left-ventricular-deformation-in-patients-with-ebstein-s-anomaly-by-cardiac-magnetic-resonance-tissue-tracking
#10
Xi Liu, Qin Zhang, Zhi-Gang Yang, Ke Shi, Hua-Yan Xu, Lin-Jun Xie, Li Jiang, Kai-Yue Diao, Ying-Kun Guo
PURPOSE: The aim of this study was to clarify the feasibility of myocardial strain using cardiovascular magnetic resonance (CMR) for the evaluation of left ventricular (LV) deformation in patients with Ebstein's anomaly (EA). MATERIALS AND METHODS: We recruited 32 patients with EA and 30 controls for CMR examination and measured LV function, dimension and tissue tracking parameters (the global and regional radial, circumferential and longitudinal peak strain), together with the right ventricle (RV) dimension...
April 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28265211/modified-cone-reconstruction-of-the-tricuspid-valve-for-ebstein-anomaly-as-performed-in-siberia
#11
Evgeny V Krivoshchekov, Jaeger P Ackerman, Olga S Yanulevich, Alexander A Sokolov, Nadezhda V Ershova, Joseph A Dearani, Frank Cetta
The cone reconstruction technique, first described by da Silva and modified by Dearani and by others, has become the repair method of choice in patients with Ebstein anomaly of the tricuspid valve. This report details the outcome of the modified cone reconstruction technique in 6 children who underwent surgical correction of Ebstein anomaly at the Tomsk Institute of Cardiology in Siberia. From 2012 through 2015, 4 boys and 2 girls (age range, 11 mo-12 yr) underwent surgery to correct Ebstein anomaly. All had presented with cyanosis, exertional dyspnea, fatigue, or new-onset atrial arrhythmia, and none had undergone previous cardiac surgery...
February 2017: Texas Heart Institute Journal
https://www.readbyqxmd.com/read/28258242/successful-cardiac-transplantation-outcomes-in-patients-with-adult-congenital-heart-disease
#12
Jonathan N Menachem, Jessica R Golbus, Maria Molina, Jeremy A Mazurek, Nicole Hornsby, Pavan Atluri, Stephanie Fuller, Edo Y Birati, Yuli Y Kim, Lee R Goldberg, Joyce W Wald
OBJECTIVES: The purpose of our study is (1) to characterise patients with congenital heart disease undergoing heart transplantation by adult cardiac surgeons in a large academic medical centre and (2) to describe successful outcomes associated with our multidisciplinary approach to the evaluation and treatment of adults with congenital heart disease (ACHD) undergoing orthotopic heart transplantation (OHT). BACKGROUND: Heart failure is the leading cause of death in patients with ACHD leading to increasing referrals for OHT...
March 3, 2017: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/28253361/respiratory-syncytial-virus-hospitalization-risk-in-the-second-year-of-life-by-specific-congenital-heart-disease-diagnoses
#13
Deborah Friedman, Jon Fryzek, Xiaohui Jiang, Adam Bloomfield, Christopher S Ambrose, Pierre C Wong
Children with hemodynamically significant congenital heart disease (CHD) are at elevated risk of morbidity and mortality due to respiratory syncytial virus (RSV) disease compared to their healthy peers. Previous studies have demonstrated lower RSV hospitalization risk among all children with CHD at 12-23 months of age versus 0-11 months of age. However, RSV hospitalization risk at 12-23 months of age by specific CHD diagnosis has not been characterized. Both case-control and cohort studies were conducted using data from the US National Inpatient Sample from 1997 to 2013 to characterize relative risk of RSV hospitalization among children 12-23 months of age with CHD...
2017: PloS One
https://www.readbyqxmd.com/read/28212520/adp-ribosyl-cyclases-cd38-cd157-social-skills-and-friendship
#14
Anne Chong, Fabio Malavasi, Salomon Israel, Chiea Chuen Khor, Von Bing Yap, Mikhail Monakhov, Soo Hong Chew, Poh San Lai, Richard P Ebstein
Why some individuals seek social engagement while others shy away has profound implications for normal and pathological human behavior. Evidence suggests that oxytocin (OT), the paramount human social hormone, and CD38 that governs OT release, contribute to individual differences in social skills from intense social involvement to extreme avoidance that characterize autism. To explore the neurochemical underpinnings of sociality, CD38 expression of peripheral blood leukocytes (PBL) was measured in Han Chinese undergraduates...
January 16, 2017: Psychoneuroendocrinology
https://www.readbyqxmd.com/read/28203575/ebstein-anomaly-with-qrs-fragmentation-on-electrocardiogram
#15
Prakash Acharya, Jonathan Ross Ang, Bernard Gitler
Ebstein anomaly is a rare congenital disorder that involves the tricuspid valve and the right ventricle. It is associated with interatrial communication, which allows for paradoxical embolization causing unilateral blindness. Abnormal conduction through the atrialized right ventricle leads to QRS fragmentation on electrocardiogram. Its presence suggests a more severe abnormality and a higher risk of arrhythmia. The QRS fragmentation disappears after corrective surgery with resection of the atrialized right ventricle...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28183001/social-impairments-among-children-perinatally-exposed-to-oxytocin-or-oxytocin-receptor-antagonist
#16
Edwa Friedlander, Ohad Feldstein, David Mankuta, Maya Yaari, Ayelet Harel-Gadassi, Richard P Ebstein, Nurit Yirmiya
No abstract text is available yet for this article.
March 2017: Early Human Development
https://www.readbyqxmd.com/read/28161232/-correctly-address-the-cause-of-hemophagocytic-lymphohistiocytosis
#17
M Penel-Page, B Ben Said, A Phan, L Hees, C Hartmann-Merlin, S Girard, Y Gillet, A Belot
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe syndrome usually associated with a cytotoxicity deficiency, which leads to an excess of immune response driven by activated macrophages and cytotoxic T cells. In children, HLH can be genetic, as part of a familial lymphohistiocytosis, or secondary: the most frequent causes are systemic-onset juvenile idiopathic arthritis, hematological malignancies, and severe infections, especially with Ebstein-Barr virus or leishmaniosis. We report on the case of a 3-year-old girl with no past medical history, who presented inaugural Pseudomonas aeruginosa maxillary osteitis, with secondary HLH...
February 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28151866/acute-arterial-embolism-of-left-lower-extremity-caused-by-paradoxical-embolism-in-ebstein-s-anomaly-a-case-report
#18
Jun-Sheng Li, Jie Ma, Zi-Xing Yan, Dong-Ming Cheng, Liang Chang, Hai-Chun Zhang, Jiang-Yan Liu
INTRODUCTION: Ebstein's anomaly is a benign and stable congenital heart disease for asymptomatic patients. Despite a low incidence of Ebstein's anomaly (EA), patients' quality of life can be badly affected by EA without positive surgical intervention. Especially EA is associated with other congenital heart disease, such as the atrial septal defect, patent foramen ovale, and arterial embolism exclude other reasons, it is often considered to be the consequence of paradoxical embolism, and surgical intervention must be conducted...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28149483/age-related-ebv-positive-clonal-b-cell-lymphoid-proliferation-ebv-dlbcl
#19
Eleni Georgakopoulou, Marina Doukas-Alexiou, Eleana Stoufi, Christos Kittas, Gerasimos Pangalis, George Laskaris
The Ebstein Barr virus(EBV), herpes virus 5 has been associated with lymphoproliferative disordrers. Age-related EBV+ B-LPD is defined as an EBV+ clonal B-cell lymphoid proliferation or EBV+-DLBCL developing in patients over the age of 40 years in the absence of any known immunodeficiency and without an underlying T-cell lymphoma1. We present a case of EBV+ clonal B-cell lymphoid proliferation. Key words:Oral mucosa ulcer, EBV+-DLBCL, age related.
January 2017: Journal of Clinical and Experimental Dentistry
https://www.readbyqxmd.com/read/28143995/ebstein-anomaly-in-pregnancy
#20
Lusiani Rusdi, Syahrir Azizi, Christopher Suwita, Astrid Karina, Sally A Nasution
A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable. Her ECG showed incomplete RBBB and prolonged PR-interval. Blood tests revealed mild anemia. Observation of two-dimensional echo with color flow Doppler study showed Ebstein's anomaly with PFO as additional defects, EF of 57%, LV and LA dilatation, RV atrialization, severe TR, and moderate PH with RVSP of 44...
October 2016: Acta Medica Indonesiana
keyword
keyword
38157
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"