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31.
The idiopathic long QT syndrome (LQTS) is an unusual clinical disorder characterized by a prolongation of the QT interval and by syncopal episodes occurring among young subjects, most often during exercise, stress, or other conditions of increased sympathetic activity. Both an imbalance in sympathetic innervation and an intracar-diac defect in membrane currents have been proposed as pathogenetic. mechanisms. The latter appears substantiated by recent advances in molecular genetics showing a linkage on chromosomes 11,3,7, and 4, with identification of the genes for chromosomes 3 and 7. For symptomatic patients with the long QT syndrome, β-adrenergic blockade, with efficacy in approximately 80% of patients, currently remains the therapy of first choice. For the patients who continue to suffer syncope or cardiac arrest despite β blockade, evidence has been provided that left cardiac sympathetic denervation represents a very effective treatment. The improvement in the understanding of the molecular mechanisms involved may soon lead to gene specific therapy in most long QT patients.  相似文献   
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The ideal prosthetic conduit for surgical repair of complex congenital heart disease has yet to be found. Twenty conduits were implanted between the right ventricle and pulmonary artery in growing sheep as follows: four Dacron porcine valve conduits (mean time in place, 142 days); four avalved glutaraldehyde-fixed bovine iliac veins (mean 132 days); and 12 glutaraldehyde-fixed bovine iliac veins containing a porcine valve (mean 180 days). Fifteen conduits were left in place from 167 to 244 days (mean 204 days), and five were explanted earlier (mean 54 days). Pathological study included gross, x-ray, histological, and ultrastructural investigation. Five conduits failed because of infective endocarditis. The valved Dacron conduits showed significant tissue ingrowth and calcification of the valve graft. The valved bovine iliac veins presented calcification at the valve level and vein wall, as well as a valvelike calcific fibrous ridge at the proximal anastomosis with the right ventricle. The avalved bovine iliac veins also presented calcific deposits along the wall and a valvelike calcific ridge at the ventricular anastomosis. Histological and ultrastructural studies of the vein tunica media revealed the phenomena of inflammatory rejection and foreign body reaction with loss of smooth muscle cells (medionecrosis) and fibrotic replacement. In conclusion, bovine iliac veins undergo inflammation with medionecrosis indicating that smooth muscle cell antigenicity is not attenuated by glutaraldehyde fixation.  相似文献   
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When performing epicardial ablation of ventricular tachycardia (VT), caution must be taken not to damage the coronary arteries. We report a case in which a new, nonfluoroscopic technique for incorporating an accurate, real‐time reconstruction of the main coronary vessels into a three‐dimensional electroanatomic map was used for epicardial VT ablation.  相似文献   
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COMMENTS   总被引:1,自引:0,他引:1  
Within the human testis, three entities of germ cell tumours are distinguished: the teratomas and yolk sac tumors of newborn and infants, the seminomas and nonseminomas of adolescents and young adults, referred to as testicular germ cell tumours (TGCT), and the spermatocytic seminomas. Characteristic chromosomal anomalies have been reported for each group, supporting their distinct pathogenesis. TGCT are the most common cancer in young adult men. The initiating pathogenetic event of these tumours occurs during embryonal development, affecting a primordial germ cell or gonocyte. Despite this intra-uterine initiation, the tumour will only be clinically manifest after puberty, with carcinoma in situ (IS) as the precursor. All invasive TGCT, both seminomas and nonseminomas, as well as CIS cells are aneuploid. The only consistent (structural) chromosomal abnormalities in invasive TGCT are gains of the short arm of chromosome 12, mostly due to isochromosome (i(12p)) formation. This suggests that an increase in copy number of a gene(s) on 12p is associated with the development of a clinically manifest TGCT. Despite the numerous (positional) candidate gene approaches that have been undertaken thus far, identification of a causative gene(s) has been hampered by the fact that most 12p gains involve rather large genomic intervals, containing unmanageable numbers of candidate genes. Several years ago, we initiated a search for 12p candidate genes using TGCT with a restricted 12p-amplification, cytogenetically identified as 12p11.2-p12.1. This approach is mainly based on identification of candidate genes mapped within the shortest region of overlap of amplification (SROA). In this review, data will be presented, which support the model that gain of 12p-sequences is associated with suppression of apoptosis and Sertoli cell-independence of CIS cells. So far, DAD-R is one of the most likely candidate genes involved in this process, possibly via N-glycosylation. Preliminary results on high through-put DNA- and cDNA array analyses of 12p-sequences will be presented.  相似文献   
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Aim: Hypertension is common in haemodialysis (HD) patients. Determining the most appropriate method of blood pressure (BP) measurement, representative of target organ damage, is still an issue. BP variations between pre‐ and post‐HD treatment, or between on‐dialysis day and off‐dialysis day, are common. The aim of this study was to examine the possible differences between pre‐HD office BP (OBP) levels, inter‐HD (iHD) or HD day 24 h ambulatory BP measurement (ABPM) with 48 h ABPM, where the latter was considered the gold standard. Methods: 163 HD patients were studied. BP was monitored consecutively for 48 h with a Takeda TM2421 device, then sub‐analysed into two periods of 24 h: HD and iHD day. An average of 12 sessions pre‐HD OBP measurements was determined. Results: OBP significantly overestimates systolic (SBP) and diastolic BP (DBP) when compared with 48 h ABPM. SBP and DBP are significantly higher on iHD day than on HD day: 141.2 ± 20.8 versus 137.9 ± 20.9, and 77.1 ± 11.1 versus 76.1 ± 10.9 (P < 0.01). No differences of SBP night/day ratio were reported between 48 h ABPM and iHD 24 h ABPM or HD 24 h ABPM. The highest correlations were reported between 48 h SBP/DBP with iHD or HD 24 h ABPM (r2 = 0.95, P < 0.001), while the lowest between 48 h SBP/DBP and OBP (r2 = 0.40, P < 0.01, r2 = 0.12, P < 0.01). The narrowest limits of agreement using the Bland and Altman test were reported between 48 h SBP or DBP and 24 h iHD or HD day ABPM. Considering 48 h ABPM, 80.5% of patients had BP higher than the norm, compared with 61.7% of patients in the case of OBP (χ2 = 13.28, P < 0.001). The sensibility for detecting hypertension for iHD day 24 h ABPM was 98.4%, with specificity of 90%. The sensibility of 24 h HD day ABPM was 90.3%, with specificity 96.6%. In the case of OBP, sensibility and specificity were considerably lower, that is, 72.6% and 83.3% respectively. Conclusion: Significant differences are shown between OBP and 48 h ABPM in the recognition of a hypertensive state. OBP measurement has a lower sensibility and specificity than 24 h ABPM, which remains a valid alternative approach to 48 h ABPM in HD patients. Errors of OBP estimation should be taken into account, with possible negative impact on treatment strategies and epidemiology studies.  相似文献   
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We present the case of a 49‐year‐old woman with atrioventricular nodal re‐entrant tachycardia and a severe pectus excavatum. The patient underwent an electrophysiological study and fast pathway ablation. Fast pathway ablation was not done on purpose but accidentally, likely due to the abnormal position of the heart in the chest cavity in this patient suffering from severe pectus excavatum. Some hours after the ablation, the patient developed inappropriate sinus tachycardia (IST), complaining of dyspnea and fatigue. IST has been described as a complication of fast pathway ablation in 10% of the cases. In our case it was not possible to treat IST with β‐blockers due to an important lowering of the blood pressure. Digitalis, given as second choice, was not successful. Ivabradine—the specific sinus node If current inhibitors—was used to successfully lower the heart rate with immediate relief of symptoms. A 24‐hour Holter, 10 days later, showed a complete control of the heart rate without any episode of IST. The patient was completely symptom free and able to undertake her normal daily activities without any discomfort. Our case confirms the potential use of ivabradine for indications other than coronary artery disease. (PACE 2010; 33:e32–e35)  相似文献   
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STABILE, G., et al.: Feasibility of Pulmonary Vein Ostia Radiofrequency Ablation in Patients with Atrial Fibrillation: A Multicenter Study (CACAF Pilot Study) Radiofrequency (RF) catheter ablation has been proposed as a treatment of atrial fibrillation (AF). Several approaches have been reported and success rates have been dependent on procedural volume and operator's experience. This is the first report of a multicenter study of RF ablation of AF. We treated 44 men and 25 women with paroxysmal   (n = 40)   or persistent   (n = 29)   , drug refractory AF. Circular pulmonary vein (PV) ostial lesions were deployed transseptally, during sinus rhythm   (n = 42)   or AF  ( n = 26)   , under three-dimensional electroanatomic guidance. Cavo-tricuspid isthmus ablation was performed in 27 (40%) patients. The mean procedure time was   215 ± 76   minutes (93–530), mean fluoroscopic exposure   32 ± 14   minutes (12–79), and mean number of RF pulses per patient   56 ± 29   (18–166). The mean numbers of separate PV ostia mapped and isolated per patient were   3.9 ± 0.5   , and   3.8 ± 0.7   , respectively. Major complications were observed in 3 (4%) patients, including pericardial effusion, transient ischemic attack, and tamponade. At 1-month follow-up, 21 of 68 (31%) patients had had AF recurrences, of whom 8 required electrical cardioversion. After the first month, over a mean period of   9 ± 3   (5–14) months, 57 (84%) patients remained free of atrial arrhythmias. RF ablation of AF by circumferential PV ostial ablation is feasible with a high short-term success rate. While the procedure and fluoroscopic exposure duration were short, the incidence of major cardiac complications was not negligible. (PACE 2003; 26[Pt. II]:284–287)  相似文献   
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