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21.
Von Hippel-Lindau disease (VHL) is an autosomal dominant tumour syndrome caused by germline mutations of the VHL tumour suppressor gene located on chromosome 3p25-26. In VHL tumours may occur in 14 different target organs, including the eye. Retinal angiomas are considered the first manifestation of VHL disease in 43% of cases, and the cumulative probability of developing a retinal angioma in one or both eyes rises during each decade of life, reaching 80% for patients over 80 years old. Since 1976 patients with VHL at the University Hospital of Utrecht and their at-risk relatives have been screened periodically by a multidisciplinary team. Long-term follow-up ophthalmological data were analysed with special attention to natural course and results of treatment. In addition, we looked for a genotype-phenotype correlation. Retinal angiomas were found in all families. In one large family with a missense mutation (V170D) of the VHL gene, in which the complete spectrum of visceral- and central nervous system (CNS) features of VHL is present, macular, parapapillary, optic disc and ora serrata angiomas were also found. In general, however, a clear-cut genotype-phenotype correlation could not be found. Only early detection and treatment of peripheral retinal angiomas can be expected to decrease the percentage of patients with decreased visual acuity. Therefore, early detection and treatment of these tumours is of paramount importance. Ophthalmological screening of patients and persons at risk should start as early as possible. In patients with apparently sporadic retinal angiomas it is advisable to perform germline DNA analysis, since the risk of developing VHL is high, especially if the angiomas are bilateral, or unilateral and multifocal, if the patient is young, or if there is a family history suggestive of VHL.  相似文献   
22.
Intravenous injection of stearoyl vanillylamide (C18-VA), a nonpungent capsaicin (CAP) analog, enhances adrenaline secretion significantly and as effectively as CAP in rats. Because swimming capacity was enhanced by CAP in mice due to CAP-induced adrenal catecholamine secretion, we investigated the effects of oral administration of C18-VA on swimming capacity using an adjustable-current water pool. Male Std ddY 6-wk-old mice were fed a commercial diet for this study and one group was orally administered C18-VA via a stomach tube. Treated mice were able to swim longer before exhaustion than the control mice (62.9 +/- 5.6 vs. 49.6 +/- 7. 0 min, P < 0.05). The swimming capacity of two groups administered C18-VA (0.02 and 0.033 mmol/kg) was significantly greater than that of those administered vehicle alone, (P < 0.05). Substance P concentration in cerebrospinal fluid, which is involved in pain transmission and is the first direct measure of pungency, was not affected by C18-VA administration. In an experiment examining the effects of C18-VA on serum adrenaline concentration, adrenaline was significantly greater in C18-VA treated mice than in controls at 2-h post-dose (C18-VA group, 26.09 +/- 2.82; control group 13.29 +/- 0. 96 microg/L, P < 0.01). In a separate study free fatty acids in serum were elevated in treated mice at 2-h post-dose (P < 0.01). While serum glucose concentration was not affected. These results suggest that C18-VA increased swimming capacity of mice via adrenaline release, independent of pungency. In addition, the present study suggests the usefulness of its application to humans.  相似文献   
23.
24.
OBJECTIVE: The aim of this study was to investigate the clinical and epidemiological factors associated with the appearance of peptic ulcer in patients with cirrhosis and, in particular, the role of Helicobacter pylori infection. METHODS: A total of 201 of 220 consecutive patients included in a prospective study that aimed to evaluate the effect of dietary intervention on cirrhotic complications and survival underwent upper gastrointestinal endoscopy. At entry, an epidemiological and clinical questionnaire was completed and the presence of peptic ulcer disease or esophageal varices at endoscopy was prospectively collected. Sera were obtained and stored at -70 degrees C until analyzed, being tested afterward for Helicobacter pylori antibodies using a commercial ELISA kit. RESULTS: Eleven of 201 patients had borderline anti-Helicobacter pylori IgG titers and were excluded from further analysis. In the remaining 190 patients, point prevalence of peptic ulcer was 10.5% and lifetime prevalence 24.7%. Multivariate analysis selected male sex (OR 2.3; 95%CI 1.09-4.89) and Helicobacter pylori seropositivity (OR: 1.7, 95%CI 1.02-2.81) as the variables independently related to peptic ulcer disease. CONCLUSIONS: Male sex and seropositivity for Helicobacter pylori are the major risk factors for peptic ulcer in cirrhosis.  相似文献   
25.
The reduction of dissolved oxygen has been studied over a wide conductivity range for use in steady or nonsteady hydrodynamic measurements. Mass transfer fluctuations can be analysed statistically to obtain the power spectra of hydrodynamic fluctuations. This requires a consideration of the proton reduction, which prevents diffusion from limiting the current. The transfer function for deducing the hydrodynamic spectra from mass transfer spectra includes not only transport effects but also kinetic effects which account for the finite rate of the reaction. The experimental study was performed using electrohydrodynamic (EHD) impedance.  相似文献   
26.
Phosphodiesterase 7 (PDE7) is an enzyme responsible for the degradation of cyclic adenosine monophosphate (cAMP), an important cellular messenger. PDE7’s role in neurotransmission, expression profile in the brain and the druggability of other phosphodiesterases have motivated the search for potent inhibitors to treat neurodegenerative and inflammatory diseases. Different heterocyclic compounds have been described over the years; among them, phenyl-2-thioxo-(1H)-quinazolin-4-one, called S14, has shown very promising results in different in vitro and in vivo studies. Recently, polymeric nanoparticles have been used as new formulations to target specific organs and produce controlled release of certain drugs. In this work, we describe poly(lactic-co-glycolic acid) (PLGA)-based polymeric nanoparticles loaded with S14. Their preparation, optimization, characterization and in vivo drug release profile are here presented as an effort to improve pharmacokinetic properties of this interesting PDE7 inhibitor.  相似文献   
27.
The effect of niobium implantation (2×1016 and 1017 ions/cm2) on the oxidation behavior of a -TiAl-based intermetallic alloy Ti–48Al–2Cr in air at 800°C has been examined. Isothermal studies with exposure times up to 200 hr and cyclic-oxidation tests up to 800 hr revealed a negligible effect for the low implantation dose. However, a remarkable decrease in oxidation rates was observed for the material implanted with 1017 Nb ions/cm2. The improvement in the oxidation resistance appeared to be similar to that obtained by alloying of the intermetallic with a few atomic percent niobium. Possible mechanisms for the effect of niobium on the oxidation resistance of TiAl-base intermetallics are discussed in view of the results obtained for the implanted material.  相似文献   
28.
Temperature‐responsive interpenetrating polymer network (IPN) hydrogels constructed with poly(vinyl alcohol) and poly(diallyldimethylammonium chloride) using the sequential IPN method were studied. The characteristics of IPN hydrogels were investigated using the dynamic vapor sorption system. IPN hydrogels exhibited a relatively high sorption ratio, 180–360% at room temperature. The sorption ratio of hydrogels depended on temperature. Diffusion coefficients were calculated according to the Fickian Law at several temperatures. The apparent activation energy was 5.43 kJ mol?1, which corresponds to typical diffusion processes. © 2003 Wiley Periodicals, Inc. J Appl Polym Sci 90: 1389–1392, 2003  相似文献   
29.
Although carbon fiber epoxy composite materials have excellent properties for structures, the joint in composite materials often reduces the efficiency of the composite structure because the joint is often the weakest area in the composite structure.

In this paper, the effects of the adhesive thickness and the adherend surface roughness on the static and fatigue strengths of adhesively-bonded tubular polygonal lap joints have been investigated by experimental methods. The dependencies of the static and fatigue strengths on the stacking sequences of the composite adherends were observed.

From the experimental investigations, it was found that the fatigue strength of the circular adhesively-bounded joints was quite dependent on the surface roughness of the adherends and that polygonal adhesively-bonded joints had better fatigue strength characteristics than circular adhesively-bonded joints.  相似文献   
30.
In the present work, the complete oxidation of acetone has been studied over a series of supported manganese oxide catalysts on the unpillared and the Al- and Zr-pillared forms of two natural clays, a montmorillonite and a saponite. A significant influence of the clay supports characteristics on the catalytic performance has been found. The following order of improving catalytic performance is established with respect to the pillars composition: Al-pillared clays < unpillared clays < Zr-pillared clays.  相似文献   
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