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921.
922.
Gaucher disease   总被引:1,自引:0,他引:1  
  相似文献   
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926.
BACKGROUND: Although hypotension commonly accompanies intermittent hemodialysis for acute renal failure (ARF) in the intensive care unit (ICU), little is known about how it may be prevented. Online relative blood volume (RBV) monitoring has been used to reduce hypotension in chronic hemodialysis, but is of unproven benefit in ARF. This study tested the hypothesis that hypotension is predictable using online RBV monitoring in patients dialyzed for ARF in an ICU setting. METHODS: The primary definition of hypotension was mean arterial pressure (MAP) <70 mm Hg; systolic blood pressure (SBP) <100 mm Hg was used as a secondary outcome. Fifty-seven treatments were prospectively studied in twenty consecutive adult patients treated with intermittent hemodialysis. RBV was continuously measured using the Hemoscan device (Gambro). RESULTS: Hypotension occurred in 30% of treatments as defined by MAP, and 18% as defined by SBP. There was no evidence of patient-specific or universal RBV thresholds that were associated with hypotension. Analysis using the kappa statistic showed that concordance of RBV and hypotension (that is, RBV falling prior to hypotensive episodes rather than rising or remaining stable) was no greater than chance. RBV and rate of change in RBV slopes did not predict hypotension as a dichotomous outcome, and were not correlated with MAP or SBP whether autocorrelation was corrected for or neglected. Substituting rate of change in RBV (RBV slopes) for absolute RBV values did not improve predictive power. CONCLUSIONS: These results suggest that strategies based on online RBV monitoring are unlikely to reduce hypotension in intermittent hemodialysis for ARF that is delivered through central venous catheters.  相似文献   
927.
The cloning of the dystrophin gene has led to major advances in the understanding of the molecular genetic basis of Duchenne, Becker, and other muscular dystrophies associated with mutations in genes encoding members of the dystrophin-associated glycoprotein complex. The recent introduction of pharmaceutical agents such as prednisone has shown great promise in delaying the progression of Duchenne muscular dystrophy but there remains a need to develop more long-term therapeutic interventions. Knowledge of the nature of the dystrophin gene and the glycoprotein complex has led many researchers to think that somatic gene replacement represents the most promising approach to treatment. The potential use of this strategy has been shown in the mdx mouse model of Duchenne muscular dystrophy, where germ line gene transfer of either a full-length or a smaller Becker-type dystrophin minigene prevents necrosis and restores normal muscle function.  相似文献   
928.
Timing of onset of autoimmunity is a prerequisite for unmasking triggers and pathogenesis of type 1 diabetes. We followed 4,590 consecutive newborns with 8 or 3% HLA-DQB1 conferred risk for type 1 diabetes at 3-, 6-, or 12-month intervals up to 5.5 years of age. Islet cell autoantibodies (ICAs) and, in the 137 children with ICAs, insulin autoantibodies (IAAs), GAD65 autoantibodies (GADAs), and IA-2 protein autoantibodies (IA-2As) were measured. Children with high genetic risk developed ICAs more often than those with moderate risk (log-rank P = 0.0015); 85 and 91% remained ICA negative by 5 years of age, respectively. The time of appearance of biochemical autoantibodies was then compared with the appearance of ICAs. IAAs and GADAs emerged usually before ICAs (means -1.8 and -1.5 months, respectively) and IA-2As after ICAs (mean 2.0 months). Ninety-five percent of all IAAs, GADAs, and IA-2As seroconversions occurred in a cluster (-12 to 8 months) around the ICA seroconversion. We conclude that diabetes-associated autoantibodies emerged in children with predisposing HLA-DQB1 alleles after 3 months of age at a constant tempo, determined by the genetic risk level, usually in the order of IAA, GADA, ICA, and IA-2A. Seroconversion to multiple autoantibody positivity usually occurred tightly clustered in time.  相似文献   
929.
BACKGROUND AND OBJECTIVES: In dentistry, low-power lasers have been used in the treatment of dentin hypersensitivity, gingivitis, periodontitis, and different forms of oral ulcers. This in vitro study focuses on the biostimulation of NIH-3T3 fibroblasts by a low-power Ga-As-pulsed laser. STUDY DESIGN/MATERIALS AND METHODS: We have studied cell growth and procollagen synthesis of cultured fibroblasts submitted to low-power laser irradiation with energy densities varying from 3 to 5 J/cm(2) over a period of 1-6 days. The light source was a 120 mW Ga-As diode laser (lambda = 904 nm). Growth curves and procollagen immunoprecipitation were obtained. RESULTS: Irradiation of 3 and 4 J/cm(2) increased the cell numbers about threefold to sixfold comparing to control cultures. However, this effect was restricted to a small range of energy densities since 5 J/cm(2) had no effect on cell growth. The energy density of 3 J/cm(2) remarkably increased cell growth, with no effect on procollagen synthesis, as demonstrated by the immunoprecipitation analysis. CONCLUSIONS: Our results showed that a particular laser irradiation stimulates fibroblast proliferation, without impairing procollagen synthesis.  相似文献   
930.
Several studies have revealed that undergraduate medical education does not adequately prepare students for their work as physicians. There have been attempts to solve this problem in curriculum reforms in medical faculties. In this article, Finnish physicians' opinions on their undergraduate medical education are analysed. In 1988, a postal questionnaire was mailed to 2632 physicians registered during 1977-86, and altogether 1745 questionnaires were returned (66.3%). A follow-up study was done in 1998, and a questionnaire was sent to 2529 physicians who graduated between 1987 and 1996; 1822 questionnaires were returned (73.1%). Half of the respondents considered undergraduate education to correspond well with the requisite diagnostic skills and hospital doctors' work in general. In older and more traditional medical faculties (Helsinki, Oulu and Turku) education in primary healthcare work was considered insufficient. Also, more than 80% of the respondents felt they received too little teaching in administrative work. They reported that both traditional and younger, community-oriented faculties (Kuopio and Tampere) had considerably improved their education, especially in primary healthcare, during the 10-year follow-up. However, there were still clear differences between the education in the respective types of faculty as evaluated by their graduates. There is still room for improvements in undergraduate medical education, the better to meet the real needs of practising physicians in different fields of health care.  相似文献   
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