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101.

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   
102.
103.
The aim of the current study was to determine if induction of metallothionein (MT) via acute or chronic dietary zinc supplementation attenuates intestinal inflammation, and to investigate the relationship with site-specific intestinal MT determined by immunolocalization. Growing rats were assigned to zinc-deficient (ZD), acute zinc-treated (ZT), pair-fed, control or chronic Zn-supplemented (ZS) groups. Half the rats in each dietary group received 5% dextran sulphate sodium (DSS) in their drinking water for 4 days. DSS treatment produced acute intestinal inflammation in the colon only, however, dietary zinc deficiency, acute zinc treatment or chronic zinc supplementation did not alter the severity of ulceration. Serum zinc concentrations were attenuated in the DSS-challenged ZT and ZS groups suggesting that zinc was being utilized in some capacity in response to inflammation. DSS-challenge induced MT immunostaining in the colonic submucosa, however, MT was not associated with histological improvements in the present study. The site-specific MT induction in colonic submucosa during intestinal inflammation requires further clarification as a component of the host defense.  相似文献   
104.
105.
BACKGROUND: Little is known about the morphological and functional evolution of ventricular abnormalities in Loeffler endocarditis. METHODS AND RESULTS: We describe 5 patients, including 3 with long-term echocardiographic follow-up, in whom apical obliteration due to fibro-thrombotic thickening of the endocardium showed favorable patterns of evolution. In one patient there was almost complete resolution of the obliterative process with consequently increased effective ventricular volume. In two patients formation of a flow-passage in the fibrocalcific apical 'floor' between the main medioventricular cavity and the apical chamber, leading to a 'double-chambered' left ventricle was observed. CONCLUSIONS: Medical therapy and appropriate anticoagulation, can induce favorable long-term ventricular remodeling in Loeffler endocarditis.  相似文献   
106.
107.
108.
The aim of our study is to investigate whether single-nucleotide dystrophin gene (DMD) variants associate with variability in cognitive functions in healthy populations. The study included 1240 participants from the Erasmus Rucphen family (ERF) study and 1464 individuals from the Rotterdam Study (RS). The participants whose exomes were sequenced and who were assessed for various cognitive traits were included in the analysis. To determine the association between DMD variants and cognitive ability, linear (mixed) modeling with adjustment for age, sex and education was used. Moreover, Sequence Kernel Association Test (SKAT) was used to test the overall association of the rare genetic variants present in the DMD with cognitive traits. Although no DMD variant surpassed the prespecified significance threshold (P<1 × 10−4), rs147546024:A>G showed strong association (β=1.786, P-value=2.56 × 10−4) with block-design test in the ERF study, while another variant rs1800273:G>A showed suggestive association (β=−0.465, P-value=0.002) with Mini-Mental State Examination test in the RS. Both variants are highly conserved, although rs147546024:A>G is an intronic variant, whereas rs1800273:G>A is a missense variant in the DMD which has a predicted damaging effect on the protein. Further gene-based analysis of DMD revealed suggestive association (P-values=0.087 and 0.074) with general cognitive ability in both cohorts. In conclusion, both single variant and gene-based analyses suggest the existence of variants in the DMD which may affect cognitive functioning in the general populations.  相似文献   
109.
110.
Background: Ketamine is increasingly utilized for a variety of pain management challenges. Audience comments from a ketamine presentation at the 2015 American Society of Pain Management Nursing (ASPMN) Conference reflected wide variation in ketamine practices as well as barriers to use. Aim: The goal was to gain a greater understanding of ASPMN member practice patterns and barriers related to ketamine as adjunctive therapy for pain management. Design: A questionnaire survey design was used. Settings: Respondents represented 35 states and 2 countries. Participants: The participants were 146 respondents from ASPMN membership (1,485 members). Methods: The survey was distributed by ASPMN on SurveyMonkey. Practice setting and ketamine administration practices were assessed with areas for comments. Results were reviewed using frequencies to describe responses and formatted into tables. Comments were individually reviewed and grouped into common themes. Results: Administration of ketamine as an analgesic was reported by 63% of respondents. Continuous intravenous ketamine infusions were the most common route of administration (65%); however, wide variability in dosing and length of therapy was reported. A wide variety of practices and challenges related to ketamine utilization were noted. Conclusions: Numerous studies have indicated the analgesic benefits of ketamine in pain management. The lack of practice standardization has created challenges to its consistent use and outcome measurement. Additionally, the off-label use of ketamine for pain management creates its own unique challenges. However, given the current national climate with intense focus on pain management, interdisciplinary practitioners have an ideal opportunity to evaluate ketamine's use in a comprehensive approach to pain management.  相似文献   
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