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排序方式： 共有8063条查询结果，搜索用时 15 毫秒
71.
Yoav Michowitz Anat Milman Antoine Andorin Georgia Sarquella-Brugada M. Cecilia Gonzalez Corcia Jean-Baptiste Gourraud Giulio Conte Frederic Sacher Jimmy J.M. Juang Sung-Hwan Kim Eran Leshem Philippe Mabo Pieter G. Postema Aviram Hochstadt Yanushi D. Wijeyeratne Isabelle Denjoy Carla Giustetto Yuka Mizusawa Bernard Belhassen 《Journal of the American College of Cardiology》2019,73(14):1756-1765
Background
Information on young patients with Brugada syndrome (BrS) and arrhythmic events (AEs) is limited.Objectives
The purpose of this study was to describe their characteristics and management as well as risk factors for AE recurrence.Methods
A total of 57 patients (age ≤20 years), all with BrS and AEs, were divided into pediatric (age ≤12 years; n = 26) and adolescents (age 13 to 20 years; n = 31).Results
Patients’ median age at time of first AE was 14 years, with a majority of males (74%), Caucasians (70%), and probands (79%) who presented as aborted cardiac arrest (84%). A significant proportion of patients (28%) exhibited fever-related AE. Family history of sudden cardiac death (SCD), prior syncope, spontaneous type 1 Brugada electrocardiogram (ECG), inducible ventricular fibrillation at electrophysiological study, and SCN5A mutations were present in 26%, 49%, 65%, 28%, and 58% of patients, respectively. The pediatric group differed from the adolescents, with a greater proportion of females, Caucasians, fever-related AEs, and spontaneous type-1 ECG. During follow-up, 68% of pediatric and 64% of adolescents had recurrent AE, with median time of 9.9 and 27.0 months, respectively. Approximately one-third of recurrent AEs occurred on quinidine therapy, and among the pediatric group, 60% of recurrent AEs were fever-related. Risk factors for recurrent AE included sinus node dysfunction, atrial arrhythmias, intraventricular conduction delay, or large S-wave on ECG lead I in the pediatric group and the presence of SCN5A mutation among adolescents.Conclusions
Young BrS patients with AE represent a very arrhythmogenic group. Current management after first arrhythmia episode is associated with high recurrence rate. Alternative therapies, besides defibrillator implantation, should be considered. 相似文献72.
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Martin Stroedicke Yacine Bounab Nadine Strempel Konrad Klockmeier Sargon Yigit Ralf P. Friedrich Gautam Chaurasia Shuang Li Franziska Hesse Sean-Patrick Riechers Jenny Russ Cecilia Nicoletti Annett Boeddrich Thomas Wiglenda Christian Haenig Sigrid Schnoegl David Fournier Rona K. Graham Michael R. Hayden Stephan Sigrist Gillian P. Bates Josef Priller Miguel A. Andrade-Navarro Matthias E. Futschik Erich E. Wanker 《Genome research》2015,25(5):701-713
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Mura Eleonora Masnada Silvia Antonello Clara Parazzini Cecilia Izzo Giana Garau Jessica Sproviero Daisy Cereda Cristina Orcesi Simona Veggiotti Pierangelo Zuccotti Gianvincenzo Dilillo Dario Penagini Francesca Tonduti Davide 《Metabolic brain disease》2021,36(5):859-863
Metabolic Brain Disease - Aicardi-Goutières Syndrome (AGS) is a monogenic leukodystrophy with pediatric onset, clinically characterized by a variable degree of neurologic impairment. It... 相似文献
80.
Radio-isotopic techniques may be useful in diagnosis and staging of chronic diffuse liver diseases. Liver angioscintigraphy (LAS) and per-rectal portal scintigraphy (PRPS) are at well discriminating portal hypertension (PHT), very early cirrhosis hemodynamic failure and compensatory arterialisation of liver perfusion. Supplied information is related to PHT, liver morphology and mesenchimal activity in liver, spleen and bone marrow. Correlation of LAS and PRPS may diagnose installing of PHT earlier than any actual morphologic imagistic method. Our experience (after more than 300 PRPS and 500 LAS) suggests that PHT and portal-cave shunts (PCS) may be classified in five functional stages. These five patterns (types) are characteristic for portal dynamics, supporting disease staging and follow-up of evolution to cirrhosis. All five dynamics may be assessed by PRPS and LAS. Scintigraphic techniques also explore portal thromboses, perfusion differences between the lobes of cirrhotic liver, betablockers effect in PHT, earliest stages of PHT, malignant tumours occurring on cirrhosis, the different characteristics of alcoholic liver comparing to viral etiology. 相似文献