1例安置埋藏式自动复律除颤器病人的随访

埋藏式自动复律除颤器(AICD)能降低恶性快速室性心律失常患者的猝死发生率,已经得到基本肯定,但其猝死率的降低,在相当程度上取决于能否对置入AICD患者进行认真随访,不断完善其治疗。本文报告1例随访16个月经验,并就随访结果,结合文献,略予讨论。     

A nationwide survey of excessive daytime sleepiness in Parkinson's disease in France

To determine the prevalence of excessive daytime sleepiness (EDS) and that of dozing and sudden onset of sleep episodes (SOS) while driving in ambulatory patients with Parkinson's disease (PD) in France, a national sample of private and public neurologists was asked to recruit the first 10 consecutive nondemented PD patients. Each patient completed a questionnaire including the Epworth Sleepiness Scale (ESS) and the likelihood of dozing off and experiencing SOS episodes behind the wheel. Clinical and demographic data were collected. One thousand six hundred and twenty‐five patients with PD were included in the survey. Twenty‐nine percent of the patients suffered from EDS (ESS score ≥10) but only 0.8% declared a high chance of dozing while driving and 0.5% reported totally unpredictable SOS episodes while driving. Risk factors for EDS were male gender, reduced activity of daily living, and a high daily levodopa equivalent dosage. Risk factors for SOS episodes while driving were an ESS score ≥10, male gender, and low Hoehn and Yahr staging. EDS is common in ambulatory patients with PD and is a major risk factor for dozing and for SOS episodes behind the wheel in patients who drive. © 2007 Movement Disorder Society     

Preexcitation in Hypertrophic Cardiomyopathy: A Case of a Fasciculoventricular Mahaim Fiber

STELLBRINK, C., et al .: Preexcitation in Hypertrophic Cardiomyopathy: A Case of a Fasciculoventricular Mahaim Fiber . A patient with hypertrophic cardiomyopathy (HCM) who presented with preexcitation pattern on the surface ECG suggestive of the Wolff-Parkinson-White (WPW) syndrome is described. Intracardiac electrophysiological study revealed a fixed anomalous QRS complex and a short fixed His-ventricular interval indicating a fasciculoventricular Mahaim fiber. As this specific form of accessory connection does not cause reentrant tachycardias, no treatment was required. It is important to distinguish this entity from atriofascicular or nodoventricular Mahaim fibers or the WPW syndrome in patients with HCM showing a preexcitation pattern in the surface ECG, as these may cause life-threatening arrhythmias in this patient population.     

外伤性截瘫合并耳聋11例临床分析

初步分析了11例外伤性截瘫合并耳聋患者,指出外伤性截瘫多有慢性肾功能不全、肾性贫血等并发症。这些并发症对耳聋的发生有一定的影响,但长时间或反复使用耳毒性药物是一个不可忽视的重要因素。作者对此提出了预防耳聋的一些具体措施,可供临床参考或借鉴。     

Post-mortem analysis for two prevalent β-oxidation mutations in sudden infant death

BACKGROUND: Fatty acid oxidation disorders may cause sudden and unexpected infant death and are associated with the histological hallmark of hepatic steatosis. The goal of the present study was to assess the value of post-mortem molecular analysis for medium-chain acyl-coenzyme A dehydrogenase (MCAD) and mitochondrial trifunctional protein (MTP) defects in unexplained sudden infant death (SID) associated with fatty infiltration of the liver. MCAD catalyzes the first step of medium-chain fatty acid oxidation while MTP catalyzes the last three steps of long-chain fatty acid oxidation. METHODS: In a retrospective study, 220 consecutive cases of sudden and unexplained infant death certified by medical examiners at Wake Forest University Medical Center were assessed for hepatic steatosis. Subjects with evidence of hepatic steatosis were screened for mutations in MCAD and MTPalpha-subunit using DNA isolated from paraffin-embedded liver tissue, single-strand conformation variance, and nucleotide sequence analyses. RESULTS: Sixteen cases (7.3%) were associated with diffuse micro-vesicular or mixed micro- and macro-vesicular hepatic steatosis. Two of these 16 cases (12.5%) had disease-causing mutations. One was homozygous for the prevalent MCAD A985G mutation. The second was a compound heterozygous for the prevalent MTP G1528C mutation and a novel 1 bp deletion in exon 18 of the MTPalpha-subunit gene. CONCLUSIONS: A significant proportion (7.3%) of SID is associated with hepatic steatosis. The present data support post-mortem molecular analysis for the MCAD A985G and MTP G1528C prevalent mutations in cases of sudden and unexplained infant death associated with hepatic steatosis.     

急性心肌梗塞猝死的诱因及抢救(附13例报告)

本文报告了急性心肌梗塞猝死13例。分析猝死的诱因主要有血清钾浓度降低,严重心律失常,包括室内传导阻滞、室性并行心律、室性早搏等。近年来急性心肌梗塞的发病率日趋增高,心源性猝死也随之增多,有必要对猝死的诱因进行探讨,以采取有效的抢救措施。     

突发性耳聋甲皱微循环改变及针刺的影响

目的：观察突发性耳聋患甲皱微循环改变及针刺的影响。方法：采用自身前后对照的方法。对突发性耳聋患针刺前后甲皱微循环进行记录。结果：突聋患甲皱微循环明显异常，针刺后患甲皱微循环加权积分值明显改善（P＜0．05）。结论：针刺可改善突聋甲皱微循环状态，提示针刺治疗突聋的机制可能与患外周血液循环得到改善有关。     

Vietnamese refugees in Victoria, B.C.: An overview of immigrant and refugee health care in a medium-sized Canadian urban centre

This paper examines the manner in which Vietnamese refugees acces the healthcare system in Victoria, British Columbia. A major theme of this study was the identification of barriers to health care access and provision as perceived by refugees and health care providers, as well as areas of overlap between the two sets of perceptions. The study was based on interview protocols developed with key informants followed by structured samples of 20 Vietnamese and 20 health care workers. The major issue identified by both groups was problematic interpretation of patient symptoms and health care provider recommendations. Lack of health care worker understanding of traditional remedies for common ailments was also identified as a barrier to health care access and utilization. The special problems of unemployment, depression, surviving torture and getting assistance are all made more difficult for refugees living in a smaller urban centre which lacks sufficiently large ethnic populations to assist in service provision. A number of suggestions are made which might ameliorate the difficulties of refugees living in smaller communities. These include municipally based client advocates and special translation training for existing hospital staff.     

Novel pore mutation in SCN5A manifests as a spectrum of phenotypes ranging from atrial flutter, conduction disease, and Brugada syndrome to sudden cardiac death

OBJECTIVES: The purpose of this study was to determine the clinical and biophysical characteristics of a novel SCN5A mutation. BACKGROUND: Brugada syndrome and isolated cardiac conduction defect have been linked to SCN5A mutations. METHODS: Eleven members of a western European family underwent electrophysiologic investigations and mutation analysis of the SCN5A gene. Wild-type and mutant SCN5A channels were expressed in HEK293 cells, and whole cell currents were studied using patch clamp procedures. RESULTS: A novel mutation, R376H, in the first pore segment of SCN5A variably causes Brugada syndrome and/or conduction disease in a single family. Biophysical analysis demonstrated a significant current reduction for the mutant, a pathophysiologic profile consistent with Brugada syndrome and isolated cardiac conduction defect. Among 11 family members, 9 were carriers of the mutation. The proband's initial presentation was a saddleback Brugada ECG, atrial flutter, and diffuse conduction disturbances. He had no inducible ventricular arrhythmias but experienced sudden cardiac death. His brother was affected by atrial flutter and had a clear conduction disorder, but he did not display baseline or evocable ECG signs of Brugada syndrome. He received an implantable cardioverter-defibrillator that delivered one appropriate shock after 1 year of follow-up. The phenotype in the family members was highly variable and ranged from noninducible and inducible asymptomatic carriers of the mutations to isolated conduction disease and to symptomatic Brugada syndrome. CONCLUSIONS: We describe the functional characterization of a novel SCN5A pore mutation, R376H, with variable clinical expression in the same family. Differentiating between electrophysiologic entities (Brugada syndrome-isolated cardiac conduction defect) is more challenging. Recognition of factors modifying the clinical presentation may be important for clinical decision making.