Mitochondrial DNA deletions in oculopharyngeal muscular dystrophy.

The deletions in the mitochondrial DNA from skeletal muscle samples of two oculopharyngeal muscular dystrophy cases were studied using polymerase chain reaction techniques. The 4977 bp 'common deletion' was present in both specimens, exceeding the corresponding values of similarly aged, healthy controls. In the two samples multiple different mitochondrial DNA deletions, some case-specific and present at quite high, although not pathogenetic levels, were observed. The results suggest that mitochondrial DNA deletions, and the 'common deletion' in particular, might be a sensitive and early marker of a generalized mitochondrial suffering, due to a variety of pathological and physiological causes.

Exercise capability and myocardial perfusion in chronic angina patients treated with enhanced external counterpulsation.

BACKGROUND: Enhanced external counterpulsation (EECP) has been shown to improve treadmill times and myocardial perfusion. However, improvement in perfusion defects has been demonstrated only in patients exercised to the same cardiac workload on the post-EECP as the pre-EECP stress test. HYPOTHESIS: This study was to determine the effect of EECP on exercise capacity and myocardial perfusion by comparing results of maximal exercise radionuclide testing pre- and post-EECP treatment. METHODS: This prospective study included 25 patients with angina who had performed maximal symptom-limited exercise tolerance tests (ETT) with Bruce protocol and radionuclide perfusion single-photon emission computed tomography (SPECT) study prior to and at completion of EECP treatment. RESULTS: After 35 h of EECP, 23 patients (93%) improved by at least one functional angina class. There is a significant improvement in their total treadmill times (357 +/- 93 to 449 +/- 97 s, p < 0.001). There was a significant change in their peak double products, from 18,891 +/- 3,939 pre-EECP to 20,464 +/- 4,305 post-EECP ETT (p < 0.03). Pre EECP, 16 patients had ST-segment depression on their initial ETT. After EECP, 13 of these patients (80%) either no longer had ST depression or had a significant increase in their time to ST depression (229 +/- 52 to 315 +/- 60 s, p < 0.001). The radionuclide perfusion scores also showed a significant reduction in ischemic segments (16.36 +/- 10.52 to 14 +/- 10.9, p < 0.05). CONCLUSIONS: Patients treated with EECP demonstrated a reduction in angina symptoms, improvement in exercise capacity, increase in time to ST-segment depression, and decrease in perfusion defects despite performing at a higher workload.

Restenosis after carotid surgery.

On the basis of personal experience and reports in the literature, the paper examines the incidence of restenosis after carotid surgery, its potential morbidity and the results of its treatment. The incidence of stenosis after carotid surgery is described in a series of 253 patients given carotid TEAs in 1992-98 and followed up at 1, 3, 6 and 12 months with yearly check-ups thereafter. The group's age range was 64-70 and it included 35% females, 65% males. The initial surgical procedure was a standard TEA with or without patching. Identified by colour Doppler scan, cases of restenoses were examined angiographically in the presence of significant symptoms and haemodynamic disorders. This group constituted 7.5% of the personal series and was classified on the basis of clinical, morphological, topographical and haemodynamic criteria. Only 21% of the restenosis cases required repeat surgery: 2 classic procedures and 2 endovascular operations. No complications ensued and all these patients were symptom-free at 84 months' follow-up. A comparison of these data with reports in the literature confirms an increasing incidence of restenosis after carotid surgery in recent years, which partly reflects more accurate diagnosis. It is concluded that indications to surgery should take greater account of the lesion's histological and clinical characteristics and the increased risk of peripheral nerve damage in repeat surgery. The alternative endovascular approach will need to be examined on the basis of bigger case series and longer, more meaningful follow-ups before any definitive conclusions can be reached though the endovascular approach does seem to represent the future of treatment in this particular sector.

Defibrotide versus heparin in the prevention of coronary reocclusion after thrombolysis in acute myocardial infarction.

A multicenter controlled study versus heparin was conducted to explore the activity of defibrotide, a polydesoxyribonucleotide drug, in preventing reocclusion after urokinase thrombolysis in patients with acute myocardial infarction (AMI). The study involved 137 consecutive patients with AMI and a time from the onset of symptoms < or = 6 hours, treated with urokinase (1,000,000 U intravenous bolus followed by 1,000,000 U slow-drip infusion over 12 hours). Immediately after thrombolysis, patients were allocated to treatment with defibrotide (group D: day 0, 3.6 g by intravenous infusion in 12 hours; days +1 to +6, 800 mg tid intravenously; days +7 to +10/+12, 400 mg tid intramuscularly), or heparin (group H: day 0, 1000 IU/hour infused over 12 hours; days +1 to +10/+12, 5000 IU tid subcutaneously). Coronary angiography was done, whenever possible, at +10/+12 days. The following parameters were assessed: (a) noninvasive estimate of myocardial reperfusion, through the analysis of CPK time-activity curves; (b) incidence of infarct-related artery (IRA) patency (TIMI scores 2-3) at coronary angiography. A total of 125 patients had a complete enzymatic curve (63 in group D and 62 in group H) and 106 had coronary angiography as well. IRA patency (the main end point) was observed in 63% of group D versus 43% of group H patients (p = 0.07). No statistically significant differences were found in the proportion of patients with indirect signs of early reperfusion (63% in group D versus 52% in group H patients).(ABSTRACT TRUNCATED AT 250 WORDS)

[Changes in humoral immunity in ischemic cardiomyopathy]. / Modificazioni dell'immunità umorale in corso di cardiopatia ischemica.

The present study has been performed with the aim of assessing the incidence and the possible implications of the changes in humoral immunity in patients with coronary heart disease. Serial determinations of the immunoglobulins (Ig) G, A and M, of specific anti-heart antibodies and of some non-organ-specific antibodies have been carried out in the venous blood of 15 patients with acute myocardial infarction (AMI), of 30 subjects with angina pectoris (AP) and of 30 controls. The occurrence of anti-smooth muscle and anti-nuclear antibodies resulted negligible in all subjects, while anti-mitochondrial antibodies were found in a relatively high percentage of cases, which is probably due to chance. Only in 13.3% of AMI patients, and in 16.7% of AP subject, were anti-heart antibodies detectable, and their presence was not related to the occurrence of Dressler's syndrome, nor to any clinical finding. The mean IgG curve in the AMI patients showed a triphasic time-course in the first 20 days of disease. In the AP patients an inverse correlation has been found between monthly frequencies of anginal attacks and serum concentrations of IgG (r = 0.382; p less than 0.05). In the control group serum IgA were directly correlated to age (r = 0.493; p less than 0.01); furthermore, in patients with exertional or mixed angina serum IgA were often higher than those of patients with only rest angina (x2 = 3.906; p less than 0.05). These data suggest the working hypothesis that a possible link (of secondary or primary type) between serum concentrations of IgA and severity of atherosclerosis may exist.

Uso de atracúrio para o bloqueio da musculatura extrínseca do bulbo ocular em cães submetidos à anestesia inalatória sob ventilação espontânea / Blocking of ocular bulb extrinsic musculature by atracurium in inhalant anesthetized dogs under spontaneous breathing

Comparou-se o bloqueio da musculatura extrínseca do bulbo ocular com três doses de atracúrio em cães submetidos à anestesia inalatória sob ventilação espontânea. Em estudo cego, foram utilizados seis cães, pré-medicados com 0,1mg/kg de acepromazina intravenoso (IV), anestesiados com 5mg/kg de propofol, entubados e mantidos sob anestesia inalatória com 1,5 por cento de isofluorano e submetidos a quatro tratamentos: não tratados (controle), tratados com 25µg/kg (G25) de atracúrio IV, com 50µg/kg (G50) de atracúrio IV e com 75µg/kg (G75) de atracúrio IV. Mensuraram-se: pressão parcial de CO2 expirado (ETCO2), freqüência cardíaca (FC), freqüência respiratória (ƒ), saturação arterial de oxiemoglobina (SatO2) e tempo de centralização do bulbo ocular. Nos grupos tratados com atracúrio, o ETCO2 aumentou aos 5min, e permaneceu aumentado até 10min em G50 e até 20min em G75, sendo este o único tratamento cuja concentração de ETCO2 apresentou-se acima de 50mmHg. Não houve reinalação de CO2 em nenhum grupo. Em G75, observou-se aumento crescente da ƒ até os 40min e considerável bradicardia após 10min; ambos retornaram aos valores basais após esse período. A centralização do bulbo ocular foi crescente de acordo com a dose: G25, 38±13min; G50, 65±16,4 min; G75, 78±27min, mas não houve diferença estatística entre G50 e G75. Conclui-se que G50 apresentou bloqueio satisfatório sem promover intensa e prolongada hipercapnia nos animais.

The ocular bulb extrinsic musculature blocking by the administration of three atracurium doses in isoflurane anesthetized dogs under spontaneous breathing was compared. In a blind study, six dogs were premedicated with 0.1mg/kg of acepromazine, anesthetized with 5mg/kg of propofol, intubated and maintained in inhalation anesthesia with 1.5 percent of isoflurane in 100 percent of oxygen. Afterwards, they were submitted to four treatments (control, G25:25µg/kg of atracurium IV, G50:50µg/kg IV and G75:75µg/kg IV). Heart rate (HR), breathing rate (BR), CO2 extrated (ETCO2), arterial saturation of oxyhaemoglobin (SatO2) and ocular bulb centralization time were measured. ETCO2 in all animals were increased at 5min keeping high until 10min in G50 and until 20min in G75 dogs; this was the unique animal group that showed results above 50mmHg of ETCO2. CO2 was not reinhaled by any animal. It was observed an increase in BR until 40min and a considerable bradycardia after 10min in G75 animals; both returned to basal levels thereon. The centralization time was crescent, according the doses (G25: 38±13min; G50: 65±16.4min; G75: 78±27min), but no difference between G50 and G75. It was showed that G50 dogs took a content ocular centralization without intense and prolonged hypercapnia.