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891.
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. 相似文献
892.
KJ Shon M Grilley R Jacobsen GE Cartier C Hopkins WR Gray M Watkins DR Hillyard J Rivier J Torres D Yoshikami BM Olivera 《Canadian Metallurgical Quarterly》1997,36(31):9581-9587
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). 相似文献
893.
This paper presents a new self-routing packet network called the plane interconnected parallel network (PIPN). In the proposed design, the traffic arriving at the network is shaped and routed through two banyan network based interconnected planes. The interconnections between the planes distribute the incoming load more homogeneously over the network. The throughput of the network under uniform and heterogeneous traffic requirements is studied analytically and by simulation. The results are compared with the results of the baseline network and another banyan network based parallel interconnection network. It is shown that, for the proposed design, a higher degree of heterogeneity results in better performance 相似文献
894.
Reliable multicast protocols suffer from the problem of feedback implosion. To avoid this problem, the number of receivers sending feedback in case of loss must be small. However, losses experienced by different receivers are strongly correlated, since receivers share common resources in the multicast tree. One approach to feedback implosion avoidance relies on delaying feedback at the receivers. We present deterministic timeouts for reliable multicast (DTRM), a distributed algorithm to compute optimal deterministic timeouts for each receiver in a multicast tree as a function of the tree topology and the sender-to-receiver round-trip delays. DTRM has several desirable properties. First, feedback implosion is provably avoided for a single loss anywhere in the tree, provided delay jitter is bounded. Second, the computation of the timeouts can be entirely distributed; receivers and intermediate nodes only rely on local topology information. Third, the timeouts computed by DTRM are optimal with respect to the maximum response time 相似文献
895.
A beam propagation method (BPM) based on the finite element method (FEM) is described for longitudinally varying three-dimensional (3-D) optical waveguides. In order to avoid nonphysical reflections from the computational window edges, the transparent boundary condition is introduced. The present algorithm using the Pade approximation is, to our knowledge, the first wide-angle finite element beam propagation method for 3-D waveguide structures. To show the validity and usefulness of this approach, numerical results are shown for Gaussian-beam excitation of a straight rib waveguide and guided-mode propagation in a Y-branching rib waveguide 相似文献
896.
Neviani A. Meneghesso G. Zanoni E. Hafizi M. Canali C. 《Electron Device Letters, IEEE》1997,18(12):619-621
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 αn in In0.53Ga0.47As at medium-low electric fields are reported for the first time. The increase of αn 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 In0.53Ga 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 In0.53Ga 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 相似文献
897.
The application of a general self-calibration procedure, the so-called TNA (through, network, attenuator) method, to a dual six-port network analyzer is presented for the first time. This innovation results from strictly separating the establishment of the complex measurement ability and the determination of the analyzers system error parameters. As will be seen, the well-known TRL (through, reflect, line) method is a special case of the general TNA technique 相似文献
898.
T. Herrmannsdörfer J. Friebel F. Pobell J. Mrachkov M. Kirov E. Leyarovski 《Journal of Low Temperature Physics》1997,107(1-2):209-224
We have measured the 16 Hz susceptibility of the diluted Van Vleck paramagnets Pr1−x
Y
x
Ni5 withx=0.00, 0.05, 0.10. 0.20 at temperatures 1.5 K≤T≤50 K and magnetic fields up to 15T. Their Van Vleck type magnetic susceptibility χ(T, B, x) strongly depends on the field atB≥6 T. The temperatureT
max(B, x) of the maximum of χ(T, B, x) decreases at increasing the field from zero to 15 T by approximately one order of magnitude for all Pr1−x
Y
x
Ni5 compounds. Changing the dilutionx from zero to 0.20, the field whereT
max(B, x) strongly drops is increased from 9 T to 11 T. Our data agree qualitatively with the predictions of a point charge model
which considers the Zeeman term in addition to the electronic exchange and the dominating crystal field contributions. 相似文献
899.
Douay M. Xie W.X. Taunay T. Bernage P. Niay P. Cordier P. Poumellec B. Dong L. Bayon J.F. Poignant H. Delevaque E. 《Lightwave Technology, Journal of》1997,15(8):1329-1342
A comprehensive survey of photosensitivity in silica glasses and optical fiber is reviewed. Recent work on understanding the mechanisms contributing to germanium or aluminum doped fiber photosensitivity is discussed within the framework of photoelastic densification models 相似文献
900.