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INTRODUCTION: Cystic fibrosis is a recessive genetic systemic exocrinopathy caused by a variety of mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). The disease is characterized by alterations of the secretions, which become thickened and viscous. Both the paranasal sinuses and the lung parenchyma are involved in all cases. The aim of this study was to assess a correlation between the rhinosinusal and lung parenchyma changes in cystic fibrosis patients. MATERIAL AND METHODS: Eighteen patients (11 men and 7 women, age range: 8 to 22 years) were examined with chest HRCT and sinonasal low dose CT. Lung symptoms were found in all patients; 13 of them, also affected with rhinosinusal symptoms, had been examined with ENT and nasal endoscopy. The other 5 patients, without rhinosinusal symptoms and previously examined with ENT, were evaluated as control group. Chest CT was performed with the high-resolution technique, 2 mm slice thickness and 10 mm table feed. Rhinosinusal CT was performed with the low dose technique, acquiring contiguous 2-4 mm thickness coronal sections. The CT patterns were analyzed by two radiologists and scored as slight, medium and diffuse involvement of both districts. RESULTS: No statistically significant correlation between lung and sinonasal damage was found in our study. Parenchymal lung involvement appeared more severe than sinonasal involvement in 14/18 patients. The retention of secretions in the paranasal sinuses, even if limited, was demonstrated in all symptomatic and asymptomatic patients. CONCLUSIONS: The absence of correlation between pulmonary and sinonasal damage and more generally, the different severity of cystic fibrosis can be caused by different allele mutations of the cystic fibrosis transmembrane conductance regulator gene, the most frequent of which is Delta F-508. 相似文献
2.
JOM - This paper provides some insight into an area that has been neglected, namely the possibility of developing high-strength, niobium-base alloys by improved oxidation resistance via the... 相似文献
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We solve the problem of set-point (respectively, tracking) control of a permanent-magnet synchronous motor via linear time-invariant (respectively, time varying) control. Our control approach is based on the physical properties of the machine: inherent stability and robustness to external disturbances. Our analysis is carried out under mild conditions, using cascaded systems theory. For all cases: constant operating point, trajectory tracking, and with known and unknown load, we show uniform global asymptotic stability of the closed-loop system with a linear controller that uses only velocity measurements. Furthermore, we explore natural extensions of our results to improve robustness with respect to external ldquodisturbancesrdquo and parametric uncertainties. 相似文献
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E Scribano G Loria G Ascenti I Pandolfo A Blandino M Gaeta 《Canadian Metallurgical Quarterly》1995,89(5):658-661
The facial lymph nodes are classified in five groups: mandibular, buccinator, infraorbital, malar and retrozygomatic nodes. This paper reports the CT appearance of neoplastic involvement of these nodes, an unusual and not well documented event. The CT examinations of 62 patients with a history of primary or recurrent cancer of the epidermal structures of the face, oral cavity and sinonasal region were retrospectively reviewed to assess the presence of facial adenopathy. Nine cases of neoplastic involvement of facial nodes were found. Most commonly the buccinator nodes (4 cases) were involved, the infraorbital, mandibular (2 cases) and retrozygomatic nodes (1 case) being less commonly involved. No malar nodes were found. Neoplastic involvement of these nodes was caused by squamous cell carcinoma in 6 cases, by adenocarcinoma in 2 cases and by a lymphoma in 1 case. Normal nodes could not be confidently identified on CT studies. CT diagnosis of neoplastic involvement of facial nodes is based on the presence of a nodular lesion which lies along the lymphatic pathways of the primary neoplasm in an anatomically compatible location. CT diagnosis of facial nodes is very important for treatment planning if the nodes are deep or at a distance from primary cancer. This is especially true for retrozygomatic and buccinator nodes. 相似文献
6.
Provides an answer to the long-standing question of designing asymptotically stable proportional plus integral regulators with only position feedback for robots with uncertain payload. It has previously been shown in Kelly (1993) and Ailon and Ortega (1993) that globally asymptotically stable set-point regulators for robot manipulators without velocity measurement can be obtained replacing the velocity feedback of a proportional plus derivative controller by a filtered position feedback. In these schemes, the only robot prior information required is the evaluation of the gravity forces at the reference (constant) position. This prior knowledge is used to shape the robot potential energy to have a unique minimum at the desired position. A mismatch in the estimation of the gravity forces leads to a position steady-state error. The authors' main contribution in this paper is to obviate the need of this prior information via the inclusion of two integral terms, around the position error and the filtered position, respectively. Semiglobal stability of the resulting control law is established 相似文献
7.
N Carulli M Ponz De Leon F Zironi A Pinetti A Smerieri R Iori P Loria 《Canadian Metallurgical Quarterly》1980,21(1):35-43
The activity of 3-hydroxy-3-methylglutaryl-CoA (HMG-CoA) reductase and 7alpha-hydroxylase, the enzymes controlling the rate of hepatic synthesis, respectively, of cholesterol and bile acids, and the microsomal cholesterol content were evaluated in 25 patients with cholesterol gallstones and 17 subjects without gallstones. The same quantities were estimated in 16 additional patients with gallstones given chenodeoxycholic (CDCA) or ursodeoxycholic acid (UDCA) at a dose of 15 mg/kg per day in order to investigate the comparative effect of a short term (7 days) administration of the two bile acids on the hepatic sterol metabolism. As compared to the controls, subjects with gallstones exhibited a 36% decrease of 7alpha-hydroxylase (26.8 +/- 6.2 versus 41.7 +/- 4.2 pmol/min per mg protein) and a 24% increase of the microsomal cholesterol (78.7 +/- 15.3 versus 63.1 +/- 18.1 nmol/mg protein). Although higher in the gallstone patients, the activity of HMG-CoA reductase did not differ significantly in the two groups of subjects. Administration of CDCA and UDCA changed the bile acid pool composition so that the fed bile acid predominated in the bile (mean CDCA 73% and mean UDCA 54%). Bile lipid composition did not appreciably change. In the eight subjects treated with CDCA the activity of HMG-CoA reductase was reduced to 45% of the value of untreated subjects (27.9 +/- 14.5 versus 63.5 +/- 25.3 pmol/min per mg protein) whereas in the eight subjects treated with UDCA the same enzyme showed a twofold increase (123.5 +/- 20.9). In the treated groups 7alpha-hydroxylase activity was somewhat decreased but the values did not differ significantly from those of the untreated subjects. Microsomal cholesterol content decreased with CDCA (64.8 +/- 11.6 nmol/mg protein) as well as with UDCA (59.1 +/- 10.1) treatment; however in the latter the difference attained statistical significance (P < 0.05). Altogether the results would suggest that in the liver of patients with gallstones the conversion of cholesterol to bile acids is somewhat reduced, and that changing the bile acid pool composition, by exogenous bile acid feeding, has disparate effects on hepatic cholesterol synthesis. The findings could represent the acute changes produced by bile acid feeding, however they could imply that the effects of two bile acids in dissolving cholesterol gallstones might not be related only to the changes in hepatic sterol metabolism.-Carulli, N., M. Ponz De Leon, F. Zironi, A. Pinetti, A. Smerieri, R. Iori, and P. Loria. Hepatic cholesterol and bile acid metabolism in subjects with gallstones: comparative effects of short term feeding of chenodeoxycholic and ursodeoxycholic acid. 相似文献
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