Diagnostic value of fundus examination in familial adenomatous polyposis

BACKGROUND: Multiple, bilateral lesions of congenital hypertrophy of the retinal pigment epithelium (CHRPE) have been described in patients suffering from familial adenomatous polyposis (FAP) since 1980. This study aimed to determine a reliable diagnostic criterion, based on the size and number of retinal CHRPE lesions, allowing the screening of patient carriers of the gene responsible for FAP. METHODS: 32 control subjects and 144 patients belonging to 85 FAP families were studied, divided into 124 carriers of the genetic alteration and 20 non-carriers. RESULTS: In carriers of the deleted gene, multiple, bilateral retinal lesions were consistently observed. Lesion situation, size, shape, and degree of pigmentation were variable however. A positive criterion for FAP was defined as the presence of at least four lesions whatever their size, or at least two lesions one of which is large. This criterion showed a high sensitivity (0.68) and a maximal specificity (1). Within each family, the retinal phenotypic expression was homogeneous. CHRPE lesions were observed in two thirds of the FAP families and absent from the remaining third. CONCLUSION: By using this new positive diagnostic criterion, fundus examination allows early detection of those children carrying the gene responsible for FAP in families positive at ocular examination.     

A noncompetitive peptide inhibitor of the nicotinic acetylcholine receptor from Conus purpurascens venom

A paralytic peptide, psi-conotoxin Piiie has been purified and characterized from Conus purpurascens venom. Electrophysiological studies indicate that the peptide inhibits the nicotinic acetylcholine receptor (nAChR). However, the peptide does not block the binding of alpha-bungarotoxin, a competitive nAChR antagonist. Thus, psi-conotoxin Piiie appears to inhibit the receptor at a site other than the acetylcholine-binding site. As ascertained by sequence analysis, mass spectrometry, and chemical synthesis, the peptide has the following covalent structure: HOOCCLYGKCRRYOGCSSASCCQR* (O = 4-trans hydroxyproline; * indicates an amidated C-terminus). The disulfide connectivity of the toxin is unrelated to the alpha- or the alphaA-conotoxins, the Conus peptide families that are competitive inhibitors of the nAChR, but shows homology to the mu-conotoxins (which are Na+ channel blockers).     

Positive temperature dependence of the electron impact ionizationcoefficient in In0.53Ga0.47As/InP HBTs

Impact ionization is a major limiting factor to the maximum operating voltage of InGaAs-based, high-speed transistors. In this work, data on the positive temperature dependence of the electron impact ionization coefficient α_n in In_0.53Ga_0.47As at medium-low electric fields are reported for the first time. The increase of α_n with temperature is opposite to the behavior normally observed in most semiconductors. This anomalous behavior implies the onset of a positive feedback between power dissipation and avalanche generation which may adversely affect the power handling capability of In_0.53Ga _0.47As-based devices, and which should be taken into account in device thermal modeling. In the experimental procedure, based on the measurement of the multiplication factor M-1 in npn In_0.53Ga _0.47As/InP Heterojunction Bipolar Transistors (HBT), particular care has been taken in order to rule out possible spurious, temperature-dependent contributions to the measured multiplication current     

A fully soft-switched extended-period quasi-resonantpower-factor-correction circuit

This paper presents the design criteria, procedure, and implementation of a soft-switched power-factor-correction (PFC) circuit based on the extended-period quasi-resonant (EPQR) principles. All power electronic devices including switches and diodes in the circuit are fully soft switched. The design method is demonstrated in a prototype circuit. The operating principles are confirmed with computer simulation and experimental results. A comparison of the EP-QR operation and zero-voltage-transition (ZVT) pulse-width modulation (PWM) method     

Automatic fault detection, isolation, and recovery in transparentall-optical networks

Network fault identification is an important network management function, which is closely related to fault management and has an impact on other network management functions such as configuration management, and performance management. This paper investigates fault surveillance and fault identification mechanisms for a transparent optical network in which data travels optically from the source node to the destination node without going through any optical-to-electrical (O/E) or electrical-to-optical (E/O) conversion. Mechanisms and algorithms are proposed to detect and isolate faults such as fiber cuts, laser, receiver, or router failures. These mechanisms allow nonintrusive device monitoring without requiring any prior knowledge of the actual protocols being used in the data transmission     

Series equivalence/operation of current-controlled boost-typesingle-phase voltage source converters for bidirectional power flow

Single-phase voltage source power converters (VSCs) under consideration are AC-DC current-controlled boost-type power converters with bidirectional power-handling capability. Equivalence between two series-connected two-level power converters and a single three-level power converter is considered here. Further considered is the series operation of three-level power converters. Simulation results and experimental verification for both are provided. Economical configurations of three-level power converters leading to multilevel waveforms are presented thereafter     

Modulation and Equalisation Considerations for High Performance Radio LANs

The European Telecommunications Standards Institute (ETSI) has recently defined a European standard for a high performance radio LAN (known as HIPERLAN). To operate as wired LAN replacements, these systems will operate at 5.2 GHz and support instantaneous bit rates of just under 24 Mb/s. To counteract the time dispersive nature of the indoor radio channel, the use of adaptive equalisation is suggested. In this paper a number of possible modulation and equalisation techniques are presented and, in particular, the Bit Error Rate (BER) performance of quasi-coherent GMSK combined with Decision Feedback Equalisation is explored through computer simulation. The trade off between symbol spaced and fractionally spaced equalisation is considered together with the importance of feedfoward and feedback synchronisation to the channel's power delay profile. The paper also includes a comparison of the RLS and LMS based training algorithms and compares the modem developed under the ESPRIT III LAURA project with that specified in HIPERLAN.The application of dual antenna diversity is investigated and its impact on the number of received error free data packets obtained as a function of signal leval and rms delay spread. The use of such diversity is shown to greatly improve the BER performance of a HIPERLAN modem. The problem of frequency offset is considered and modifications are proposed to the HIPERLAN frame structure to improve the receiver's tolerance to such errors. Important practical issues such as frame and symbol synchronisation, frequency offset correction and hardware implementation are discussed from both the LAURA and HIPERLAN viewpoint.     

Phase-only adaptive nulling with a genetic algorithm

This paper describes a new approach to adaptive phase-only nulling with phased arrays. A genetic algorithm adjusts some of the least significant bits of the beam steering phase shifters to minimize the total output power. Using small adaptive phase values results in minor deviations in the beam steering direction and small perturbations in the sidelobe level in addition to constraining the search space of the genetic algorithm. Various results are presented to show the advantages and limitations of this approach, in general, the genetic algorithm proves to be better than previous phase-only adaptive algorithms     

Wave-oriented signal processing of dispersive time-domainscattering data

Phase-space data processing is receiving increased attention because or its potential for furnishing new discriminants relating to classification and identification of targets and other scattering environments. Primary emphasis has been on time-frequency processing because of its impact on transient, especially wideband, short-pulse excitations. Here, we investigate the windowed Fourier transform, the wavelet transform, and model based superresolution algorithms within the context of a fully quantified and calibrated test problem investigated by us previously: two-dimensional (2-D) short-pulse plane wave scattering by a finite periodic array of perfectly conducting coplanar flat strips. Because the forward problem has been fully calibrated and parametrized, some quantitative measures can be assigned with respect to the tradeoffs of these time-frequency algorithms, yielding tentative performance assessments of the tested processing algorithms     

Quick estimation of rare events in stochastic networks

This paper presents a method for fast estimation of probabilities of rare events in stochastic networks, with a particular emphasis on coherent reliability systems. The method is based on the concepts of likelihood-ratios (LR), change of probability measure and the bottleneck-cut in the network. Both polynomial and exponential-time Monte Carlo estimators are defined, and conditions under which the time complexity of the proposed LR estimators is bounded by a polynomial are discussed. The accuracy of the method depends only on the size (cardinality) of the bottleneck-cut, not on the topology and actual size of the network. Supporting numerical results are presented, with the cardinality of the bottleneck-cut ⩽20