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211.
212.

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   
213.
ABSTRACT

Collaborative goal setting between clinicians and clients/families is considered a fundamental component of the pediatric rehabilitation process. However, truly client-centered goal setting is not without its challenges. The purpose of this paper is to highlight theoretical concepts relevant to rehabilitation goal setting, review clinical studies directly evaluating relationships between goal setting and pediatric rehabilitation outcomes, and provide recommendations to facilitate collaborative goal processes. Four theoretical frameworks were identified that may lie behind and help explain the effectiveness of collaborative goal setting. The four relevant outcome studies found in the review revealed that individualized goal setting is an important component of the intervention, engages families more actively in therapy, and is associated to some extent with positive outcomes. The evidence suggests that the impact of fully collaborative goal setting is sufficiently positive to support investment of organizational and individual time, energy, and resources to make it an integral part of the rehabilitation process.  相似文献   
214.
A wide gathering of scientists, clinicians, pharmacists and nurses specialized in pediatric oncology practice met to celebrate the second anniversary of Children’s Cancer Hospital, Egypt (CCHE). The celebration was in the form of high-brow teaching lectures and reports presented by international experts in the fields of pediatric CNS tumors, solid tumors (neuroblastoma, nephroblastoma, soft tissue and bone tumors, lymphoma, leukemia and pediatric oncology nursing. The conference extends its activities to hospital management, clinical pharmacy and telemedicine. Furthermore, CCHE experts presented the efforts performed to establish a state-of-the-art pediatric oncology hospital equipped with all needed facilities to raise the standard of care to the highest levels.  相似文献   
215.
Complementary and alternative medicine use is increasing in both the general population and in individuals with chronic illness. A significant proportion of adult and pediatric patients with inflammatory bowel disease use or have used complementary and alternative medicine as determined by multiple surveys conducted in North America, Europe and Australia. There was a heterogenous selection of complementary and alternative medicine therapies chosen by patients, with variations due to geography and age. However, in general, their use is sought due to frustration with conventional therapies and the perceived safety of complementary and alternative medicine therapies. One of the dilemmas that arises when assessing the topic of complementary and alternative medicine use is rapidly changing definitions of what is and what is not considered conventional medicine. With our increasing understanding of the intricacies of the gastrointestinal immune system and host–microflora interactions, determining a scientific basis behind many complementary and alternative medicine therapies in vitro or in vivo is leading to evaluation of these therapies in humans. This will require well-designed and rigourously conducted clinical trials with sufficient sample size.  相似文献   
216.
The purpose of this work was to validate a parallel imaging (PI) and compressed sensing (CS) combined reconstruction method for a recently proposed 4D non‐breath‐held, multiphase, steady‐state imaging technique (MUSIC) cardiovascular MRI in a cohort of pediatric congenital heart disease patients. We implemented a graphics processing unit accelerated CS‐PI combined reconstruction method and applied it in 13 pediatric patients who underwent cardiovascular MRI after ferumoxytol administration. Conventional breath‐held contrast‐enhanced magnetic resonance angiography (CE‐MRA) was first performed during the first pass of ferumoxytol injection, followed by the original MUSIC and the proposed CS‐PI MUSIC during the steady‐state distribution phase of ferumoxytol. Qualities of acquired images were then evaluated using a four‐point scale. Left ventricular volumes and ejection fractions calculated from the original MUSIC and the CS‐PI MUSIC were also compared with conventional multi‐slice 2D cardiac cine MRI. The proposed CS‐PI MUSIC reduced the imaging time of the MUSIC acquisition to 4.6 ± 0.4 min from 8.9 ± 1.2 min. Computationally intensive image reconstruction was completed within 5 min without interruption of sequential clinical scans. The proposed method (mean 3.3–4.0) provided image quality comparable to that of the original MUSIC (3.2–4.0) (all P ≥ 0.42), and better than conventional breath‐held first‐pass CE‐MRA (1.1–3.3) for 13 anatomical structures (all P ≤ 0.0014) with good inter‐observer agreement (κ > 0.46). The calculated ventricular volumes and ejection fractions from both original MUSIC (r > 0.90) and CS‐PI MUSIC (r > 0.85) correlated well with 2D cine imaging. In conclusion, PI and CS were successfully incorporated into the 4D MUSIC acquisition to further reduce scan time by approximately 50% while maintaining highly comparable image quality in a clinically practical reconstruction time.  相似文献   
217.
We investigated intermodality agreements of strains from two‐dimensional echocardiography (2DE) and cardiac magnetic resonance (CMR) feature tracking (FT) in the assessment of right (RV) and left ventricular (LV) mechanics in tetralogy of Fallot (TOF). Patients were prospectively studied with 2DE and CMR performed contiguously. LV and RV strains were computed separately using 2DE and CMR‐FT. Segmental and global longitudinal strains (GLS) for the LV and RV were measured from four‐chamber views; LV radial (global radial strain [GRS]) and circumferential strains (GCS) measured from short‐axis views. Intermodality and interobserver agreements were examined. In 40 patients (20 TOF, mean age 23 years and 20 adult controls), LV, GCS showed narrowest intermodality limits of agreement (mean percentage error 9.5%), followed by GLS (16.4%). RV GLS had mean intermodality difference of 25.7%. GLS and GCS had acceptable interobserver agreement for the LV and RV with both 2DE and CMR‐FT, whereas GRS had high interobserver and intermodality variability. In conclusion, myocardial strains for the RV and LV derived using currently available 2DE and CMR‐FT software are subject to considerable intermodality variability. For both modalities, LV GCS, LV GLS, and RV GLS are reproducible enough to warrant further investigation of incremental clinical merit.  相似文献   
218.
This cross‐sectional study aimed to investigate the association of periodontal status with occlusal force and food acceptability. We hypothesised that mastication deteriorated with reduced periodontal support, even when posterior occlusal contacts with natural teeth were maintained and the patients remained clinically asymptomatic. Participants were 482 independently living 69‐71‐year‐olds, classified as Eichner's group A, having no mobile teeth and no periodontal symptoms. The periodontal probing depth (PPD) and restoration status of each tooth were examined. Occlusal force in the intercuspal position was measured with pressure‐sensitive films. Food acceptability was evaluated from the difficulty experienced in chewing apples, grilled beef, and hard rice crackers. Multivariate regression analysis was performed to investigate the association of periodontal status with occlusal force and food acceptability. A P‐value of <0·05 was considered statistically significant. Multiple linear regression analysis showed that occlusal force had significant negative associations with maximal PPD (standardised partial regression coefficient (β) = ?0·121) after controlling for gender, handgrip strength, number of teeth, and percentage of restored teeth. Approximately 15% of participants were included in the compromised food acceptability group. Logistic regression analyses showed that compromised food acceptability was significantly associated with PPD, after controlling for gender, number of teeth, and percentage of restored teeth. Periodontal probing depth (PPD) was significantly correlated with occlusal force and self‐rated food acceptability after controlling for the possible confounding factors in septuagenarians, even those with complete posterior occlusal contacts and no tooth mobility.  相似文献   
219.
Studies have suggested there is a shortage of pediatric dermatologists in the United States, but the workforce has not been well defined. The Society for Pediatric Dermatology (SPD) Workforce Committee sought to characterize the US pediatric dermatology workforce with a nine‐question survey, sent to all 484 US SPD members in December 2016. The response rate was 30%. Most pediatric dermatologists were practicing in major metropolitan markets, seeing an average of 80 patients a week with an average 6‐week wait time. These findings indicate that geographic maldistribution and long wait times for new patient appointments remain substantial hurdles for adequate access to subspecialty pediatric dermatology care.  相似文献   
220.
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