Ophthalmologic findings in Usher syndrome type 2A

Thirty-seven patients, comprising 24 familial cases and 13 isolated patients with Usher syndrome type II (USH2), underwent ophthalmologic examination. Based on the degree of hearing loss, normal vestibular function, and gene-linkage analysis, familial cases were assumed to have USH2A. An analysis of genetic heterogeneity failed to reveal the presence of a second locus in the Dutch population. Although the patients appear to belong to a genetically homogeneous group, remarkable ophthalmologic variability was found. Corrected visual acuity decreased with age and remarkable differences in visual acuity were found within one family. Fundoscopic findings were classified as type A if attenuated vessels and bone corpuscles in all quadrants were found or as type B if findings other than these were found. The prevalence of type A significantly increased with age.     

Determination of cycle nonproportionality coefficient

We propose a cycle nonproportionality coefficient for a broad class of complex cyclic paths, allowing us to establish a clear relationship between the geometry of the strain path and the maximum hardening level attainable in the material in the steady state for a fixed value of the maximum plastic or total strain range. We discuss the effectiveness of using the proposed cycle nonproportionality coefficient compared with analogous parameters familiar from the literature. Based on a previously developed version of the endochronic theory of plasticity, we have numerically modeled the behavior of a material for cyclic loading along different planar nonproportional paths.Translated from Problemy Prochnosti, Nos. 5–6, pp. 29–38, May–June, 1995.     

Surgical treatment of aneurysms of the left ventricle. Immediate and long-term results. Apropos of a consecutive series of 121 cases

From 1978 to 1992, 121 cases of postinfarction left ventricular aneurysm (99 males, 22 females, mean age 60 years) were operated on. The authors insist on a high rate of clinical arhythmogenicity (31.4%) and associated mechanical complications (21%). 76% of patients were in functional NYHA class III or IV. Resection was performed in 90% of patients, plication in 10%. 58% underwent coronary artery bypass grafting (1.7 graft/patient), 16% encircling ventriculotomy, 8% mitral valve replacement and 13% closure of ventricular septal defect. Operative mortality was 14.9% (10% when other mechanical complications where excluded). 5-year survival is 67.9%. Late cardiac deaths are as follow: left ventricular failure (1.8% A/P), Sudden death (1.4% A/P), Myocardial infarction (0.6% A/P). 82% of survivals are in functional NYHA class I or II. Only functional class NYHA III or IV is predictive of late death. We conclude that postinfarction left ventricular aneurysm remains a high risk complication especially when associated with other mechanical complications. When arhythmogenicity is present we suggest rhythmologic surgery and in all cases, complete revascularization.     

Transcutaneous balloon angioplasty in the treatment of subclavian steal syndrome. Characteristics of vertebral basal flow with transcranial Doppler technique

Subclavian steal syndrome (SSS) appears when the origin of the subclavian artery (SA) is occluded or stenosed. Introduction of transcranial Doppler sonography (TCD) provided an opportunity to evaluate parameters of the blood flow in the vertebral (VA) and basilar artery (BA). Measurements of blood flow velocities performed at rest and after the brachial hyperemia test allow one to classify hemodynamic types of SSS. The aim of the study was to categorize types of steal and to compare the differences of flow patterns before and after percutaneous transluminal SA balloon angioplasty (SA-PTA). Fourty-eight patients with angiographically confirmed SSS (aged from 27 to 68 years, mean 53; 2/1 f/m ratio) were examined with 2 MHz range-gated, pulsed transcranial Doppler device (TC 2-64B EME). Both VA and BA were evaluated by the transoccipital approach at rest and during the brachial hyperemia. In 5 cases (10.4%) permanent reversal blood flow in the BA was observed (complete basilar steal). In flow in the BA blood flow was in the normal direction at rest and altered (reversed or decreased) when induced with brachial hyperemia test (transient basilar steal). In the next 14 patients (29.2%) permanently reversed VA blood flow was observed with only a slight or no alterations of the BA flow after the hyperemia test (complete vertebral steal). In the last 19 cases (39.6%) alterations of the VA blood flow without changes in BA flow were observed (latent vertebral steal). Between 1991 and 1994 twenty seven symptomatic patients with different hemodynamic types of SSS were treated with SA-PTA. TCD evaluation of VA's and BA using the hyperemia test was performed before, 3 to 7 days and 3 months after morphologically and hemodynamically successful subclavian artery balloon PTA. Normal results of vertebrobasilar examinations were obtained in 26 cases after this procedure. In one case the latent vertebral steal was detected. The 28 months mean follow-up revealed no significant changes in TCD flow patterns recorded from VA's and BA. After collecting data of about 60 patients with SSS we examined with TCD we conclude that: in patients with a hemodynamically significant SA stenosis the presence of reversed ipsilateral VA blood flow (a radiologic steal) its not a good determinant of either the presence or type of presenting symptoms and after successful PTA or recanalisation and PTA of SA in almost all cases we examined close to normal TCD recordings in BA and VA.     

Experimental Investigation of the Density of Molten Lead–Bismuth Eutectic

The density of molten Pb–Bi eutectic is measured by the pycnometer method in a temperature range of 410–726 K. The confidence of error of measurement, made up by the systematic and random components, does not exceed 0.1%. The experimental results are compared with the experimental data on the density of a liquid lead–bismuth alloy of eutectic composition available in the literature.     

Hydrogen absorption by Pd-coated ZrNi prepared by using Barrel-Sputtering System

To improve the durability of hydrogen storage materials against surface poisoning by impurity gases, effectiveness of Pd-coating layer prepared by using a Barrel-Sputtering System was examined for ZrNi powder. The effectiveness of Pd-coating was evaluated by activation temperature, at which Pd/ZrNi poisoned by air could be activated to absorb hydrogen. Characterization of Pd-coated ZrNi (denoted as Pd/ZrNi) by scanning electron microscopy, electron probe microanalysis and X-ray diffraction showed that a uniform Pd-coating layer was formed with the barrel-sputtering system. It was found that the poisoned Pd/ZrNi sample could be activated even at 423 K to absorb hydrogen at room temperature. This exhibits remarkable contrast to bare ZrNi, which could be only activated appreciably above 1073 K. It is concluded that the Pd-coating by barrel sputtering is quite effective to avoid the effect of surface poisoning of powdery hydrogen storage materials. However, the activation at excessively high temperature resulted in the loss of high activity to absorb hydrogen. It was concluded that this phenomenon was associated with reactions between Pd and ZrNi to form PdZr and other byproducts.     

Nonholonomic Mobile Manipulators: Kinematics, Velocities and Redundancies

We consider nonholonomic mobile manipulators built from an n _a joint robotic arm and a nonholonomic mobile platform with two independently driven wheels. Actually, there is no efficient kinematic formalism for these systems which are generally characterized by their high number of actuators. So, kinematic modelling is presented with particular emphasis on redundancy. Whereas kinematic redundancy is well known in the holonomic case, it is pointed out that it is necessary to define velocity redundancy in the case of nonholonomic systems. Reduced velocity kinematics based on quasi-velocities are shown to provide an efficient formalism. Two examples of mobile manipulators are presented. Finally, reduced velocity kinematics and velocity redundancy are shown to be adequate tools in order to realize operational task while optimizing criteria such as manipulability.