Electrodiagnostic evidence of phrenic nerve demyelination in Charcot-Marie-Tooth disease 1A

OBJECTIVE: To determine whether neurophysiologic findings correlate to clinical respiratory signs or spirometric abnormalities in patients with hereditary motor and sensory neuropathy type 1 (Charcot-Marie-Tooth disease). DESIGN: A total of 11 patients with hereditary motor and sensory neuropathy type 1A, genetically identified, (age range, 10-58 yr) were included and studied by physical pulmonary examination, chest radiography, respiratory function tests, and bilateral transcutaneous phrenic nerve conduction. RESULTS: No patient complained of respiratory symptoms or revealed abnormal spirometric or maximal respiratory pressure data, despite a phrenic nerve conduction significantly slower (P < 0.0001; median conduction time, 18.6 msec; 95th percentile, 31.97 msec) than that recorded in the control group of healthy subjects (median, 6.05 msec; 95th percentile, 8.82 msec); the amplitudes of compound muscle action potentials were not statistically different from the controls. CONCLUSIONS: Our study confirms a dramatic phrenic nerve involvement in absence of clinical and laboratory evidence of diaphragmatic weakness; further studies and an adequate follow-up are necessary to discover whether the disease progress might encompass respiratory dysfunction at later stages.     

Second cycle of intralesional Collagenase Clostridium histolyticum for Peyronie's disease using the modified shortened protocol: Results from a retrospective analysis

The purpose of this study was to evaluate the effectiveness and safety of the second cycle of Collagenase Clostridium histolyticum injections using the modified shortened protocol. We performed a retrospective analysis on patients who had already undergone the first cycle of injections using the modified shortened protocol and requested more injections to improve the remaining curvature. The International Index of Erectile Function, the Peyronie's Disease Questionnaire and the Global Assessment of Peyronie's Disease questionnaire were self-administered to all patients. All the parameters were recorded at baseline, after the first cycle and after the second cycle of injections. All adverse events were recorded. Seventeen patients completed two cycles of injections. All patients had a reduction of the initial curvature after the first cycle, with a mean improvement of 17.4° (27.4%). After the second cycle, the reduction of the curvature was 7.9° (17.1%), and 29.4% of patients had no further improvement. No severe side effect was recorded. The results of the present study confirm the effectiveness and safety of the modified shortened protocol of Collagenase C. histolyticum injections for Peyronie's disease. However, the second cycle of three injections may be less effective, and patients may not be completely satisfied.     

Imaging and monitoring in minimally invasive valve surgery using an intra-aortic occlusion device: a single center experience

BackgroundMinimally invasive approach through a right mini-thoracotomy is a world-wide used procedure for mitral valve surgery. We performed a retrospective analysis based on our center experience in order to propose an effective, safe and reproducible method using an intra-aortic occlusion device.MethodsThis is a retrospective analysis on 48 consecutive patients undergoing mitral valve surgery through a right anterolateral mini-thoracotomy in our center. An intra-aortic occlusion device was used for aortic clamping and cardioplegia delivery. Simultaneous multi-plane three-dimensional echocardiography imaging was acquired to detect the venous cannulas position, the intra-aortic device location in the ascending aorta, the balloon inflation, the complete occlusion of the aorta, the cardioplegia delivery, the origin and the blood flow in the right coronary artery. Aortic root pressure was measured by the tip of the intra-aortic occlusion device. A bilateral upper extremity invasive arterial pressure monitoring was detected. Neuromonitoring was performed through bilateral cerebral oximetry.ResultsThe analysis has shown no aortic dissection, neurological damage type 1 and myocardial ischemia in the study population. In 3 cases a distal displacement of the intra-aortic occlusion device was promptly detected by the combined use of echocardiographic imaging and by a drop of the right cerebral oximetry saturation and of the right radial artery pressure.ConclusionsThe combined use of transesophageal simultaneous multi-plane three- dimensional echocardiography imaging, bilateral upper extremity invasive arterial pressure monitoring, aortic root pressure and cerebral oximetry is an effective, safe and reproducible method in patients undergoing minimally invasive valve surgery using an intra-aortic occlusion device.     

A comparative study of the in vitro antioxidant activity of statins

Background: Treatment of hypercholesterolemia with statins is remarkably effective in cardiovascular prevention. This has led to the hypothesis that these drugs may act on the atherosclerotic plaque by mechanism(s) independent of the reduction of serum cholesterol levels. The aim of this study was to assess the total antioxidant activity of the most prescribed statins: fluvastatin, atorvastatin, pravastatin and simvastatin. Methods: We measured the in vitro antioxidant activity of statins as their ability to antagonize the oxidation of -keto-γ-methiolbutyric acid by both hydroxyl and peroxyl radicals. The results are expressed as Total Oxyradical Scavenging Capacity (TOSC) units. Uric acid and Trolox were used as the reference antioxidants. Results: The scavenging capacity towards hydroxyl radicals was highest for simvastatin (3375±112 U/mg), a value 270.2% higher (P<0.0001) compared to uric acid (reference antioxidant vs. hydroxyl radicals, 1249±71 U/mg). Among the tested statins, fluvastatin exhibited the highest anti-peroxyl radical antioxidant capacity (8755±187 U/mg) which appeared 50% lower (P<0.0001) compared to Trolox (reference antioxidant vs. peroxyl radicals, 17 460±379 U/mg). Conclusions: All the statins tested have intrinsic antioxidant activity with both anti-hydroxyl and peroxyl radical activity. Simvastatin was the most effective as an anti-hydroxyl radical antioxidant and fluvastatin as an anti-peroxyl radical antioxidant.