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471.
Factor I deficiency causes a permanent, uncontrolled activation of the alternative pathway resulting in an increased turnover of C3 and consumption of factor B, factor H and properdin. Factor I deficiency is clinically associated with recurrent bacterial infections already in early infancy, mainly affecting the upper and lower respiratory tract, or presenting as meningitis or septicemia. We here report on a Brazilian family (n = 9) with known consanguinity, where in 3/7 children, suffering from chronic otitis, meningitis, and respiratory infections, a complete factor I deficiency was recognized. One of the patients died after fulminant sepsis. Hemolytic activity of the alternative pathway was not detectable in the patients' sera due to decreased plasma concentrations of C3, factor B and properdin. As a consequence of factor I deficiency, C3b could not be metabolized with the result that no C3-derived split products (C3dg/C3d) were detectable in the patients' sera. In vitro reconstitution with purified factor I restored the regulatory function in the patients' sera with the subsequent cleavage of C3b to C3c and C3dg. Factor H levels were decreased in all patients' sera and found to be tightly complexed with C3b resulting in a modified electrophoretic mobility. Upon factor I reconstitution, factor H was released from C3b regaining its beta 1 electrophoretic mobility. Complement-mediated biological functions like opsonization of bacteria, chemotactic activity and phagocytosis in these patients were impaired. The parents (cousins, 2nd degree) and 3/4 siblings had significantly reduced factor I plasma levels without further alteration in their complement profile. 3 of these obviously heterozygously deficient family members suffered from recurrent bacterial infections of different frequency and severity.  相似文献   
472.
AIMS: To determine the direction of differentiation of the mucin secreting components in a rare group of oesophageal tumours--oesophageal squamous cell carcinomas with prominent mucin secreting components (mucoepidermoid carcinomas and adenosquamous carcinomas). METHODS: In a review of 617 cases of primary carcinoma of the oesophagus, 16 cases of squamous cell carcinoma with prominent mucin secreting components were studied using a battery of histochemical techniques. RESULTS: The mucin produced by these tumours was mixed and included a variable content of enzyme labile sialomucin (positive for mucicarmine, periodic acid Schiff, and alcian blue, and sensitive to sialidase digestion and negative for high iron diamine-alcian blue). Retrospective analysis of endoscopic biopsy specimens taken from these tumours showed that mucin was present in five (42%) cases. CONCLUSIONS: The glandular component of this group of tumours histochemically differentiated in the direction of oesophageal glands: examination of the mucin secreting component in squamous cell carcinoma in resected specimens is therefore required for recording the true incidence of this type of tumour.  相似文献   
473.
Immunoscintigraphy with radiolabelled monoclonal antibodies has been widely used to detect solid tumours. The purpose of this study was to investigate its potential for the specific localization of syphilitic lesions. F(ab')2 fragments were prepared from murine monoclonal antibodies against Treponema pallidum produced in our laboratory and labelled with 131I. Bilateral testicular infections were created in rabbits by inoculation with T. pallidum or Staphylococcus aureus. Seven to 10 days after inoculation, radiolabelled antibodies were injected intravenously. Serial gamma images were then taken 2 h after the injection and at 24 h intervals thereafter. Beginning as early as 2 h post-injection, the testicles could be visualized with either specific or non-specific antibodies. Gamma images in the monoclonal antibody-treated, T. pallidum-infected group persisted up to 48 h post-injection. Lesions were not discernible from background in the S. aureus-infected group injected with the monoclonal antibody and the S. aureus-infected and T. pallidum-infected groups injected with the polyclonal antibody at 24 h post-injection or later. Therefore, due to its ability to differentiate between specific and non-specific antibody-generated images from 24 h post-injection, immunoscintigraphy using monoclonal antibodies specific for T. pallidum may be employed as one of the methods to diagnose difficult cases of syphilitic internal organ involvement as well as syphilis infection in seronegative HIV-infected patients.  相似文献   
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Transmitted-Reference (TR) signaling, in conjunction with an autocorrelation receiver (AcR), offers a low- complexity alternative to Rake reception in ultrawide bandwidth systems. This paper provides a unified performance analysis of various TR schemes by developing an analytical framework based on the sampling expansion approach. Specifically, we derive the uncoded bit error probability (BEP) of different TR signaling schemes, including TR and differential TR (DTR) signaling valid for a broad class of fading channels. We consider both AcRs and modified AcRs with noise averaging. We further develop a quasi-analytical method as well as an approximate analytical method to extend the BEP analysis to include the effect of narrowband interference (NBI). We show that the approximate analytical method is particularly useful in obtaining BEP expressions that provide insight into the effect of NBI. We quantify the effects of NBI and channel power dispersion profile on the optimum integration interval of an AcR. Finally, we compare TR and DTR signaling in terms of their sensitivity to NBI.  相似文献   
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In addition to their use as an additive to improve physical properties of solvent polymeric membranes, plasticizers have a considerable impact on the specificity and sensitivity of membrane-modified electrochemical sensors. In this work, we aim at the hybridization of two different plasticizers using the electropolymerization technique in the development of a cadmium(II)-selective electrochemical sensor based on screen-printed gold electrode along with cyclic voltammetric measurement. At this point, we first screen for the primary plasticizer yielding the highest signal using cyclic voltammetry followed by pairing it with the secondary plasticizers giving rise to the most sensitive current response. The results show that the hybridization of DOS and TOTM with 3:1 weight ratio (~137.7-μm-thick membrane) renders a signal that is >26% higher than that from the sensor plasticized by DOS per se in water. The solution of 0.1 mM hydrochloric acid (pH 4) is the optimal supporting electrolyte. In addition, hybrid plasticizers have adequate redox capacity to induce cadmium(II) transfer from bulk solution to the membrane/water interfaces. Conversion of voltammetric signals to semi-integral currents results in linearity with cadmium(II) concentration, indicating the irreversible cadmium(II) transfer to the membrane. The DOS:TOTM hybrid sensor also exhibits high sensitivity, with a limit of detection (LOD) and limit of quantitation (LOQ) of 95 ppb and 288 ppb, respectively, as well as greater specificity towards cadmium(II) than that obtained from the single plasticizer sensor. Furthermore, recovery rates of spiked cadmium(II) in water samples were higher than 97% using the hybrid plasticizer sensor. Unprecedentedly, our work reports that the hybridization of plasticizers serves as ion-to-electron transducer that can improve the sensor performance in cadmium(II) detection.  相似文献   
479.
Artificial Life and Robotics - Pig weights are important indicator for the healthcare and the economic operation of pig farms, and the development of a system to easily estimate these weights is...  相似文献   
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