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Reductions in serum levels of Gc globulin, a hepatically synthesized component of the extracellular actin scavenger system responsible for complexing circulating actin and attenuating intravascular microthrombus formation, are associated with poor outcome in acute liver failure. Clinically applicable assays of the important actin-free fraction (Af-Gc) have not been available until now. We measured actin-free Gc globulin levels with a novel, rapid assay in 61 cases of acute liver failure (ALF) and in 91 patients with cirrhosis (40 of whom were clinically unstable with extrahepatic organ dysfunction), and studied associations with liver dysfunction, extrahepatic organ dysfunction, indices of disseminated coagulation, and outcome. Reductions in Af-Gc levels mirrored hepatic dysfunction and organ dysfunction in both groups, and discriminated patients with poor prognosis from those with good prognosis in the ALF cohort. Levels were lowest in patients with ALF (10% of control values), but levels were also markedly reduced in both unstable (28%) and stable (44%) patients with cirrhosis. Associations with markers of disseminated intravascular coagulation were seen in both groups, most notably in the cirrhosis cohort, supporting a pathophysiological role for reduced Af-Gc in the evolution of organ dysfunction. In acetaminophen-induced ALF, Af-Gc identified patients with poor prognosis as well as did the Acute Physiology and Chronic Health Evaluation (APACHE II) score (area under the receiver operating characteristic curve, 0.7), and in cirrhosis, Af-Gc was an independent predictor of mortality by multifactorial analysis. In conclusion, the importance of Af-Gc reductions in the development of multiple organ dysfunction in ALF and cirrhosis is highlighted, probably resulting from reduced hepatic production and peripheral exhaustion of this arm of the extracellular actin scavenger system.  相似文献   
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Melorheostosis is a rare sclerosing bone dysplasia that is characterized by a localized, diffuse thickening of the cortical bone. This condition usually affects the appendicular skeleton and associated soft tissue and rarely affects the craniofacial complex. The etiology of this condition is obscure. Diagnosis of melorheostosis relies on clinical, radiographic, and histological correlation. Only 8 cases of melorheostosis involving the craniofacial complex have been reported. We report 2 new cases of isolated melorheostosis involving the maxilla and mandible, together with differential diagnostic considerations. To our knowledge, involvement of the maxilla only has not been previously reported.  相似文献   
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This study presents a clinical validation of postoperative measurements of acetabular cup alignment following total hip arthroplasty (THA). The methodology was based on concurrent anatomic three-dimensional (3D) measurements of both the acetabular cup alignment and pelvic orientation, using an original CT/X-ray matching algorithm named Xalign. The subjects were 19 patients who had undergone bilateral THA using CT-based surgical navigation. All patients had postoperative pelvic CT scans and multiple antero-posterior (AP) pelvic X-rays. Using a proprietary software algorithm, the X-rays included in the study were matched with the corresponding postoperative CT scans. The goal of this method was to allow 3D anatomic pelvic and acetabular measurements on two-dimensional AP X-rays. The postoperative cup abduction, version and pelvic flexion angles were determined in three different ways: using CT images directly, applying the Xalign method, and finally by performing conventional (abduction only) measurements on AP pelvic X-rays. The cup orientation measured on CT images was taken as the ground truth. The Xalign measurement errors were defined as the difference between the CT cup values and those obtained by applying the matching method. The mean cup abduction error was 0.85 degrees +/- 1.3 degrees (+/- standard deviation) and the mean version error was 0.01 degrees +/- 1.99 degrees . Conventionally measured cup abduction ranged from 44 degrees to 62 degrees and correlated significantly (p = 0.001, r = -0.5) with pelvic flexion angle, proving the linear negative correlation between pelvic flexion and the error in conventional radiographic cup measurements. The Xalign method offered reasonable accuracy for cup orientation, and allowed cup and pelvic 3D anatomic measurements at different times.  相似文献   
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Split liver transplantation (SLT) benefits society by increasing the total number of transplants that can be performed, but it is yet unknown if a decreased post-transplant survival (in comparison to whole liver transplantation) would make participation in SLT less appealing to adult liver transplant candidates. A 20-item questionnaire was administered to 50 adult candidates to assess attitudes toward SLT and organ sharing. The overall attitudes of 60% of participants were classified as utilitarian (maximizing benefit to greatest number of candidates), while 26% were classified as self-preserving (maximizing individual benefit) and 14% were undecided. Ninety percent of participants would be willing to share even if expected survival was less than that of whole liver transplantation, and 69% felt that pediatric candidates should have priority over adult candidates. In conclusion, attitudes toward graft sharing and the possibility of compromised survival benefit are not barriers to SLT for most adult liver transplant candidates.  相似文献   
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The aim of the present study was to examine the regulation of exercise intensity in hot environments when exercise is performed at a predetermined, fixed subjective rating of perceived exertion (RPE). Eight cyclists performed cycling trials at 15°C (COOL), 25°C (NORM) and 35°C (HOT) (65% humidity throughout), during which they were instructed to cycle at a Borg rating of perceived exertion (RPE) of 16, increasing or decreasing their power output in order to maintain this RPE. Power output declined linearly in all three trials and the rate of decline was significantly higher in HOT than in NORM and COOL (2.35 ± 0.73 W min−1, 1.63 ± 0.70 and 1.61 ± 0.80 W min−1, respectively, P < 0.05). The rate of heat storage was significantly higher in HOT for the first 4 min of the trials only, as a result of increasing skin temperatures. Thereafter, no differences in heat storage were found between conditions. We conclude that the regulation of exercise intensity is controlled by an initial afferent feedback regarding the rate of heat storage, which is used to regulate exercise intensity and hence the rate of heat storage for the remainder of the anticipated exercise bout. This regulation maintains thermal homeostasis by reducing the exercise work rate and utilizing the subjective RPE specifically to ensure that excessive heat accumulation does not occur and cellular catastrophe is avoided.  相似文献   
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