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https://www.readbyqxmd.com/read/29779043/glis1-3-transcription-factors-critical-roles-in-the-regulation-of-multiple-physiological-processes-and-diseases
#1
REVIEW
Anton M Jetten
Krüppel-like zinc finger proteins form one of the largest families of transcription factors. They function as key regulators of embryonic development and a wide range of other physiological processes, and are implicated in a variety of pathologies. GLI-similar 1-3 (GLIS1-3) constitute a subfamily of Krüppel-like zinc finger proteins that act either as activators or repressors of gene transcription. GLIS3 plays a critical role in the regulation of multiple biological processes and is a key regulator of pancreatic β cell generation and maturation, insulin gene expression, thyroid hormone biosynthesis, spermatogenesis, and the maintenance of normal kidney functions...
May 19, 2018: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/29778499/congenital-submitral-diverticulum
#2
Tatiana Guimarães, Rui Plácido, Ana G Almeida
No abstract text is available yet for this article.
May 16, 2018: Revista Española de Cardiología
https://www.readbyqxmd.com/read/29778339/lower-risk-of-postoperative-arrhythmias-in-congenital-heart-surgery-following-intraoperative-administration-of-magnesium
#3
Dingchao He, Nimisha Aggarwal, David Zurakowski, Richard A Jonas, Charles I Berul, Sridhar Hanumanthaiah, Jeffrey P Moak
OBJECTIVES: Postoperative arrhythmias are common in children undergoing congenital heart surgery. We evaluated whether intraoperative administration of magnesium was associated with a reduced occurrence of specific postoperative arrhythmias, as has been described previously, or had a broader effect on multiple arrhythmia types, and whether there existed a dose-effect of intraoperative magnesium. METHODS: We used a historical prospective observational cohort study...
April 18, 2018: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/29777899/congenital-dyshormonogenic-hypothyroidism-with-goiter-caused-by-a-sodium-iodide-symporter-slc5a5-mutation-in-a-family-of-shih-tzu-dogs
#4
E A Soler Arias, V A Castillo, J D Garcia, J C Fyfe
An iodide transport defect (ITD) in the thyroid gland was determined to cause congenital dyshormonogenic hypothyroidism with goiter (CDHG) in 2 members of a family of Shih-Tzu dogs. Strikingly, both dogs were also diagnosed with dilated cardiomyopathy at 24 and 1.5 mo of age. The only sign of hypothyroidism was a moderate growth delay in the adult dog. The ITD was recognized by the absence of uptake of technetium-99m in the salivary glands (sg) and goiter observed by scintigraphy. In the same scan, radiopharmaceutical uptake was found in the anterior mediastinum of both dogs and in the right axillary lymph node in the oldest dog...
April 24, 2018: Domestic Animal Endocrinology
https://www.readbyqxmd.com/read/29777892/surgically-treated-de-novo-cervico-medullary-arachnoid-cyst-in-a-symptomatic-adult-patient
#5
William Clifton, Gazanfar Rahmathulla, Kourosh Tavanaiepour, Dunbar Alcindor, George Jakubek, Daryoush Tavanaiepour
Arachnoid cysts are a relatively common finding in adult patients, especially with the advent of advanced imaging techniques. The overall incidence ranges from 1-2%, and the majority are clinically silent1,2 . Arachnoid cysts are postulated to arise by congenital anomalies or trauma1 . De novo formation of arachnoid cysts has been reported, but is exceptionally rare and mostly found in the pediatric population after head trauma3-5 . There have only been two reported cases of symptomatic de novo arachnoid cyst formation in adult patients to date, both with histories of head trauma6,7 ...
May 16, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29777883/atypical-presentation-of-giant-aneurysm-in-a-pediatric-patient-with-duane-syndrome
#6
Chao-Hung Kuo, Lynn B McGrath, Joseph A Carnevale, Neena I Marupudi, Jeffery G Ojemann, Richard G Ellenbogen, Anthony C Wang
BACKGROUND: Duane syndrome is a congenital eye movement disorder characterized by congenital malformation of the abducens nucleus. Thrombogenic conditions during development may lead to vascular anomalies in Duane syndrome, however, the presence of a giant aneurysm in this patient population is a rarely documented phenomenon. CASE DESCRIPTION: We reported a case of a large cerebral aneurysm in a pediatric patient with Duane syndrome, and performed a review of the literatures to identify other potential cases and associations...
May 16, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29777671/surgical-management-and-outcomes-of-ebstein-anomaly-in-neonates-and-infants-a-society-of-thoracic-surgeons-congenital-heart-surgery-database-analysis
#7
Kimberly A Holst, Joseph A Dearani, Sameh M Said, Ryan R Davies, Christian Pizarro, Christopher Knott-Craig, T K Susheel Kumar, Vaughn Starnes, S Ram Kumar, Sara K Pasquali, Dylan P Thibault, James M Meza, Kevin D Hill, Karen Chiswell, Jeffrey P Jacobs, Marshall L Jacobs
BACKGROUND: Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multi-centric practice patterns and outcomes. We analyzed multi-institutional data concerning operations and outcomes in neonates and infants with EA. METHODS: Index operations reported in the STS Congenital Heart Surgery Database (2010-2016) were potentially eligible for inclusion...
May 16, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29777670/the-impact-of-mitral-disease-etiology-on-operative-mortality-following-mitral-valve-operations
#8
J Scott Rankin, Maria Grau-Sepulveda, David M Shahian, A Marc Gillinov, Rakesh Suri, James S Gammie, Steven F Bolling, Patrick M McCarthy, Vinod H Thourani, Niv Ad, Sean M O'Brien, Jeffrey P Jacobs, Vinay Badhwar
BACKGROUND: The patho-etiology of mitral regurgitation (MR) has been suggested as a mediator of outcomes following mitral valve (MV) operations, particularly in ischemic functional mitral regurgitation (IMR). This study examined the independent association of MV etiology with mortality. METHODS: Using the Society of Thoracic Surgeons Database, all patients undergoing mitral valve replacement or repair from 2011-2014 were assessed. Patients who underwent concomitant surgical ablation, septal defect closure, tricuspid valve repair, or coronary artery bypass grafting were included...
May 16, 2018: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/29777565/unanticipated-hospital-admission-in-pediatric-patients-with-congenital-heart-disease-undergoing-ambulatory-noncardiac-surgical-procedures
#9
Koichi Yuki, Sophia Koutsogiannaki, Sandra Lee, James A DiNardo
BACKGROUND: An increasing number of surgical and nonsurgical procedures are being performed on an ambulatory basis in children. Analysis of a large group of pediatric patients with congenital heart disease undergoing ambulatory procedures has not been undertaken. AIMS: The objective of this study was to characterize the profile of children with congenital heart disease who underwent noncardiac procedures on an ambulatory basis at our institution, to determine the incidence of adverse cardiovascular and respiratory adverse events, and to determine the risk factors for unscheduled hospital admission...
May 18, 2018: Paediatric Anaesthesia
https://www.readbyqxmd.com/read/29777556/electrophysiology-and-structural-interventions-in-adults-with-congenital-heart-disease-comparison-of-combined-versus-separate-procedures
#10
Ian Lindsay, Farnoosh Nik-Ahd, Jamil A Aboulhosn, Jeremy P Moore
BACKGROUND: Electrophysiologic (EP) and structural interventions in adult congenital heart disease (ACHD) are typically completed during separate hospital encounters. With planning/coordination, these cases can be combined. OBJECTIVES: We hypothesized that this integrated approach would yield patient and health system benefits. METHODS: Consecutive ACHD patients undergoing combined interventions were matched to controls with identical but separate procedures...
May 18, 2018: Journal of Cardiovascular Electrophysiology
https://www.readbyqxmd.com/read/29777282/ambulatory-intravenous-inotropic-support-and-or-levosimendan-in-pediatric-and-congenital-heart-failure-safety-survival-improvement-or-transplantation
#11
Sotiria C Apostolopoulou, George A Vagenakis, Alexandros Tsoutsinos, Felicia Kakava, Spyridon Rammos
End-stage heart failure (HF) frequently needs continuous inotropic support in hospital and has high morbidity and mortality in absence of heart transplantation. This study reports outcome, efficacy, and safety of continuous ambulatory inotropes (AI) and/or periodic levosimendan (LS) infusions in pediatric HF patients. The study included 27 patients, median age 9.4 (0.1-26.1) years, with severe HF (6 myocarditis, 13 dilated cardiomyopathy, 2 restrictive cardiomyopathy, 6 repaired congenital heart disease). Dobutamine and milrinone AI were administered in 21 patients through a permanent central catheter for median duration 1...
May 18, 2018: Pediatric Cardiology
https://www.readbyqxmd.com/read/29776964/arrhythmias-in-adult-patients-with-congenital-heart-disease-and-pulmonary-arterial-hypertension
#12
Maria Drakopoulou, Heba Nashat, Aleksander Kempny, Rafael Alonso-Gonzalez, Lorna Swan, Stephen J Wort, Laura C Price, Colm McCabe, Tom Wong, Michael A Gatzoulis, Sabine Ernst, Konstantinos Dimopoulos
OBJECTIVES: Approximately 5%-10% of adults with congenital heart disease (CHD) develop pulmonary arterial hypertension (PAH), which affects life expectancy and quality of life. Arrhythmias are common among these patients, but their incidence and impact on outcome remains uncertain. METHODS: All adult patients with PAH associated with CHD (PAH-CHD) seen in a tertiary centre between 2007 and 2015 were followed for new-onset atrial or ventricular arrhythmia. Clinical variables associated with arrhythmia and their relation to mortality were assessed using Cox analysis...
May 18, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29776940/successful-treatment-of-fulminant-neonatal-enteroviral-myocarditis-in-monochorionic-diamniotic-twins-with-cardiopulmonary-support-intravenous-immunoglobulin-and-pocapavir
#13
Shahnawaz M Amdani, Hannah S Kim, Anthony Orvedahl, Audrey Odom John, Ahmed Said, Kathleen Simpson
Neonatal cardiogenic shock most commonly occurs due to critical congenital heart disease, sepsis, metabolic disorder or arrhythmias. In particular, enterovirus infections are common in the neonatal period, and patients can present with fulminant myocarditis. Early recognition is imperative due to its high morbidity and mortality without prompt and aggressive treatment. We present the successful treatment of fulminant neonatal enteroviral myocarditis in a pair of monochorionic diamniotic twins with cardiopulmonary support, intravenous immunoglobulin and pocapavir, an enteroviral capsid inhibitor...
May 18, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29776810/congenital-heart-disease-in-adults-assessmentof-functional-capacity-using-cardiopulmonary-exercise-testing
#14
Sílvia Aguiar Rosa, Ana Agapito, Rui M Soares, Lídia Sousa, José Alberto Oliveira, Ana Abreu, Ana Sofia Silva, Sandra Alves, Helena Aidos, Fátima F Pinto, Rui Cruz Ferreira
AIM: The aim of the study was to compare functional capacity in different types of congenital heart disease (CHD), as assessed by cardiopulmonary exercise testing (CPET). METHODS: A retrospective analysis was performed of adult patients with CHD who had undergone CPET in a single tertiary center. Diagnoses were divided into repaired tetralogy of Fallot, transposition of the great arteries (TGA) after Senning or Mustard procedures or congenitally corrected TGA, complex defects, shunts, left heart valve disease and right ventricular outflow tract obstruction...
May 15, 2018: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29776801/determinants-of-a-good-perinatal-outcome-in-588-pregnancies-in-women-with-type-1-diabetes
#15
J Lepercq, C Le Ray, C Godefroy, L Pelage, D Dubois-Laforgue, J Timsit
AIM: This study assessed pregnancy outcomes in women with type 1 diabetes (T1D) over the last 15 years and identified modifiable factors associated with good perinatal outcomes. METHODS: Pregnancy outcomes were prospectively assessed in this cohort study of 588 singleton pregnancies (441 women) managed by standardized care from 2000 to 2014. A good perinatal outcome was defined as the uncomplicated delivery of a normally formed, non-macrosomic, full-term infant with no neonatal morbidity...
May 8, 2018: Diabetes & Metabolism
https://www.readbyqxmd.com/read/29776787/the-value-of-peripheral-perfusion-index-measurements-for-early-detection-of-critical-cardiac-defects
#16
Ozgun Uygur, Ozge Altun Koroglu, Erturk Levent, Merve Tosyali, Mete Akisu, Mehmet Yalaz, Nilgun Kultursay
BACKGROUND: Approximately 25% of congenital heart diseases (CHD) are estimated to be critical and require an intervention. In this study, we aimed to investigate the additional value of peripheral perfusion index (PPI) measurements to pulse oximetry screening for critical CHD (CCHD). METHODS: Infants born at Ege University Hospital between May 2013 and September 2015 were prospectively included in the study. In addition to physical examination, pre- and postductal oxygen saturations and PPI values were measured with a new generation pulse oximeter before discharge from the hospital...
April 12, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29776769/-congenital-kissing-nevus-lid-lesions
#17
Y Hanafi, N El Outassi, A El Omari, F El Asri, K Reda, A Oubaaz
No abstract text is available yet for this article.
May 15, 2018: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29776426/detection-of-zika-virus-infection-among-asymptomatic-pregnant-women-in-the-north-of-peru
#18
Claudia Weilg, Lucinda Troyes, Zoila Villegas, Wilmer Silva-Caso, Fernando Mazulis, Ammy Febres, Mario Troyes, Miguel Angel Aguilar-Luis, Juana Del Valle-Mendoza
OBJECTIVE: To report an outbreak of ZIKV infection among asymptomatic pregnant women during 2016 in the city of Jaen, Cajamarca. RESULTS: Zika virus RNA was detected in 3.2% (n = 36) of cases by RT-PCR. The mean age of patients positive for ZIKV infection was 29.6 years. 7 patients (19.4%) infected with ZIKV were in their first-trimester of gestation, 13 (36.1%) were in their second-trimester, and 16 (44%) were in their third-trimester. All of the infected pregnant women were asymptomatic...
May 18, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29776403/rostral-cranial-fossa-as-a-site-for-cerebrospinal-fluid-drainage-volumetric-studies-in-dog-breeds-of-different-size-and-morphotype
#19
Wojciech Sokołowski, Norbert Czubaj, Michał Skibniewski, Karolina Barszcz, Marta Kupczyńska, Wojciech Kinda, Zdzisław Kiełbowicz
BACKGROUND: Hydrocephalus is a multifactorial condition, whose aetiology is not fully understood. Congenital hydrocephalus frequently occurs in small and brachycephalic dog breeds. Although it is widely accepted that the cribriform plate located in the rostral cranial fossa (RCF) is a site of cerebrospinal fluid (CSF) drainage, the RCF has not been studied extensively. Literature reports indicate that a decreased caudal cranial fossa (CCF) volume in the course of the Chiari-like malformation may obstruct CSF circulation...
May 18, 2018: BMC Veterinary Research
https://www.readbyqxmd.com/read/29776376/hybrid-surgery-in-treatment-of-pulmonary-sequestration-with-abdominal-aorta-feeding-vessel-a-case-report
#20
Haining Zhou, Shoujun Tang, Quanshui Fu, Li Yu, Lunxu Liu
BACKGROUND: Pulmonary sequestration is a rare congenital pulmonary dysplasia, which requires surgical resection (either via open thoracotomy or video-assisted thoracoscopic surgery [VATS] or via endoluminal occlusion of the abnormal feeding vessel). CASE PRESENTATION: We described a 51-year-old female patient with a history of recurrent cough and repeated pneumonia. She was referred to our hospital for further work-up of pulmonary sequestration. We performed a hybrid surgery (i...
May 18, 2018: Journal of Cardiothoracic Surgery
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