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41.
Alagille syndrome (AGS) is one of the major forms of chronic liver disease in childhood with severe morbidity and a mortality of 10 to 20%. It is characterised by cholestasis of variable severity with paucity of interlobular bile ducts and anomalies of the cardiovascular system, skeleton, eyes, and face. Previous studies suggest a wide variation in the expression of the disease and a high incidence of new mutations. To determine more accurately the rate of new mutations and to develop criteria for detecting the disorder in parents we systematically investigated parents in 14 families with an affected child. Clinical examination was supplemented by liver function tests, echocardiography, radiographic examination of the spine and forearm, ophthalmological assessment, and chromosome analysis. Six parents had typical anomalies in two or more systems pointing to the presence of autosomal dominant inheritance. Systematic screening of parents for the features defined in this study should improve the accuracy of genetic counselling.  相似文献   
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This work demonstrates that for constant oxide reliability stresses in the Fowler-Nordheim regime a low initial rate of charge trapping/detrapping results in long times to breakdown. It was found for MOS gate oxides that when the initial trapping has been completed at low fields times to breakdown enhance. Depending on the stress sequence measurement results can vary significantly which is of great relevance for correct oxide lifetime predictions.  相似文献   
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OBJECTIVES: Antral gastritis is frequent in alcoholics. The role of H. pylori in the pathogenesis of gastritis in these patients is not well known. The aim of our study was to study the role of H. pylori and cirrhosis in the pathogenesis of antral gastritis in alcoholic patients. METHODS: Seventy-nine patients were included in the study. All underwent upper gastrointestinal tract endoscopy with antral biopsies, independently of the presence of abdominal pain, and had serological examination for H. pylori antibodies. RESULTS: Cirrhosis and gastritis were present in 50 and 40 patients respectively, H. pylori serological assay and histological identification of the bacterium were positive in 35 (44%) and 19 (24%) patients respectively. Discrepancy between the 2 tests were observed more frequently in cirrhotic patients. A positive serology with a negative histologic examination for H. pylori was present for 18 cirrhotic and 4 noncirrhotic patients (p < 0.05). A gastritis without evidence of H. pylori was more frequent in cirrhotic than in noncirrhotic patients. H. pylori was histologically present in 11 of 29 cirrhotic patients and in 8 of the 11 noncirrhotic patients with a gastritis (p < 0.05). CONCLUSIONS: Discrepancies between histological examination and H. pylori serology in patients with cirrhosis might be due to the inhospitable environment for H. pylori in case of portal hypertension; the positive serology could be in relation with a past infection.  相似文献   
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Memorializes Richard Solomon, one of the major learning theorists of the 2nd half of the 20th century. Solomon was named the 1st James M. Skinner University Professor of Science in 1975, was elected to the National Academy of Sciences, and was awarded, among other honors, the Warren Medal for research by the Society of Experimental Psychologists, the American Psychological Association Distinguished Scientific Contribution Award, and the American Psychological Foundation's Award for Distinguished Teaching in Experimental Psychology. He conducted research on a broad array of topics such as word frequency and perceptual defense, children's estimation of the size of tokens, hoarding behaviors in rats, and personality ratings and sociometric patterns. His work made a major contribution to the exploration of Pavlovian conditioning. Other topics of research focused on in Solomon's laboratories included avoidance learning, learned helplessness, punishment, and the opponent-process theory of acquired motivation. (PsycINFO Database Record (c) 2010 APA, all rights reserved)  相似文献   
47.
BACKGROUND: The most common cause of a sudden onset of painful proptosis with diplopia in an otherwise healthy adult is orbital pseudotumor. However, there are other conditions that mimic this presentation and must be ruled out with laboratory testing and imaging studies. CASE REPORT: A 41-year-old Hawaiian man sought treatment for an acute, progressive, painful, left ophthalmoplegia without exophthalmos. During the next week, a loss of accommodation and associated pupillary reaction, decreased visual acuity, color vision deficits, and a paracentral scotoma developed. CT and MRI revealed a mass in the orbital apex. All systemic findings were negative, and high-dose systemic steroid therapy was initiated. Symptoms resolved within hours of the first dose, and signs were completely absent 1 month later. Follow-up MRI revealed complete absence of the previously noted mass. CONCLUSION: This is an atypical case of orbital pseudotumor, since there was no exophthalmos. It was diagnosed by clinical presentation, laboratory and imaging studies, and response to therapy.  相似文献   
48.
It is experimentally observed that the peritectic reaction, 211 + liquid → 123, can be driven essentially to completion in 1 h at an undercooling of only ∽30°C. The kinetic data, together with the observed microstructures, are inconsistent with the normal mechanism of the peritectic reaction. It is proposed that the mechanism of the reaction involves dissolution of 211 particles into the liquid and precipitation of solid 123. The aligned grain structure is explained through sympathetic nucleation of new 123 grains on existing grains.  相似文献   
49.
Models of the relations between contact with pets and better health are examined in an archival prospective study using data derived from the longitudinal study initiated by Terman in 1921 (current N?=?343 men, 300 women) . In survival analyses of documented longevity, playing with pets in 1977 (M age?=?67 years) was not associated with mortality risk through 1991 for the total sample nor for those who were unmarried or those who were less satisfied with their human relationships. Playing with pets was not associated with health-prone attributes or healthy behaviors such as personality, social ties, education, and smoking. (PsycINFO Database Record (c) 2011 APA, all rights reserved)  相似文献   
50.
Multiresolution decompositions for video coding are reviewed. Both nonrecursive and recursive coding schemes are considered. In nonrecursive schemes, it is shown that pyramid structures have certain advantages over subband or wavelet techniques, and a specific spatiotemporal pyramid coding of HDTV is discussed in some detail. It is shown that recursive, DPCM like schemes will incur a slight loss of optimality due to a restricted form of prediction if multiresolution decomposition with compatible decoding is required. Compatibility and transmission issues are also discussed. Multiresolution transmission for digital broadcast TV is introduced. This, when combined with multiresolution source coding, achieves spectrum efficiency, robustness and graceful degradation under channel impairments.Invited PaperWork supported in part by the National Science Foundation under grants ECD-88-11111, MIP-90-14189 and Bell Communications Research.Work supported by the National Science Foundation under grants ECD-88-11111. K.M. Uz is now with David Sarnoff Research Center in Princeton, NJ 08543.  相似文献   
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