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A case of mixed connective tissue disease (MCTD) is presented in which mediastinal lymphadenopathy was the most prominent radiological finding detected by plain chest radiographs and computed tomography. Pulmonary arterial hypertension, which is a rare and often fatal complication of MCTD, also developed in this patient. 相似文献
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An enzyme-linked immunosorbent assay has been developed for the quantitation of activated Hageman factor-C1 inactivator (HF-C1 INH) complexes. Addition of increasing quantities of either of the major forms of activated Hageman factor (HFa or HFf) to normal plasma or to Hageman factor-deficient plasma leads to a dose-dependent increase in activated HF-C1 INH complexes. As little as 0.5 micrograms/mL of activated HF added to plasma can be detected, corresponding to activation of approximately 2% of plasma HF. The sensitivity of the assay is increased at least tenfold when complexes are formed in HF- deficient plasma, indicating competition between unactivated HF and activated HF-C1 INH complexes for binding to the antibody. Specificity is demonstrated in that addition of activated HF to hereditary angioedema plasma yields less than 1% of the activated HF-C1 INH complex formation obtained with normal plasma. Kaolin activation of HF- deficient plasma yields no detectable complex formation. Kaolin activation of prekallikrein-deficient plasma demonstrates a time- dependent increase in formation of activated HF-C1 INH complex consistent with the ability of HF in this plasma to autoactivate as the time of incubation with the surface is increased. Kaolin treatment of high-molecular weight (HMW) kininogen-deficient plasma yields an even more profound abnormality in the rate of formation of activated HF-C1 INH complexes reflecting the complex role of HMW kininogen in the initiation of contact activation. Although addition of corn inhibitor to plasma prevents activated HF-C1 INH complex formation, it does not inhibit activated HF sufficiently fast to prevent prekallikrein activation. 相似文献
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Linking a genome‐wide association study signal to a LRRK2 coding variant in Parkinson's disease 下载免费PDF全文
Jia Nee Foo PhD Sun Ju Chung MD PhD Louis C. Tan MD Herty Liany MS Ho‐Sung Ryu MD Myunghee Hong MS Tat Hung Koh MS Ishak D. Irwan MS Wing‐Lok Au MD Kumar‐M. Prakash MD Tin Aung MD PhD Ching‐Yu Cheng MD PhD Siow‐Ann Chong MD Chiea Chuen Khor MD PhD Jimmy Lee MD E‐Shyong Tai MD PhD Eranga N. Vithana PhD Tien‐Yin Wong MD PhD Kyuyoung Song PhD Eng‐King Tan MD 《Movement disorders》2016,31(4):484-487
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Elizabeth Barrett‐Connor Gail A Laughlin Hong Li Carrie M Nielson P Ying Wang Tien T Dam Jane A Cauley Kristine E Ensrud Marcia L Stefanick Edith Lau Andrew R Hoffman Eric S Orwoll for the Osteoporotic Fractures in Men Research Group 《Journal of bone and mineral research》2012,27(11):2306-2313
Low 25‐hydroxyvitamin D (VitD), low sex hormones (SH), and high sex hormone binding globulin (SHBG) levels are common in older men. We tested the hypothesis that combinations of low VitD, low SH, and high SHBG would have a synergistic effect on bone mineral density (BMD), bone loss, and fracture risk in older men. Participants were a random subsample of 1468 men (mean age 74 years) from the Osteoporotic Fractures in Men Study (MrOS) plus 278 MrOS men with incident nonspine fractures studied in a case‐cohort design. “Abnormal” was defined as lowest quartile for VitD (<20 ng/mL), bioavailable testosterone (BioT, <163 ng/dL), and bioavailable estradiol (BioE, <11 pg/mL); and highest quartile for SHBG (>59 nM). Overall, 10% had isolated VitD deficiency; 40% had only low SH or high SHBG; 15% had both SH/SHBG and VitD abnormality; and 35% had no abnormality. Compared to men with all normal levels, those with both SH/SHBG and VitD abnormality tended to be older, more obese, and to report less physical activity. Isolated VitD deficiency, and low BioT with or without low VitD, was not significantly related to skeletal measures. The combination of VitD deficiency with low BioE and/or high SHBG was associated with significantly lower baseline BMD and higher annualized rates of hip bone loss than SH abnormalities alone or no abnormality. Compared to men with all normal levels, the multivariate‐adjusted hazard ratio (95% confidence interval [CI]) for incident nonspine fracture during 4.6‐year median follow‐up was 1.2 (0.8–1.8) for low VitD alone; 1.3 (0.9–1.9) for low BioE and/or high SHBG alone; and 1.6 (1.1–2.5) for low BioE/high SHBG plus low VitD. In summary, adverse skeletal effects of low sex steroid levels were more pronounced in older men with low VitD levels. The presence of low VitD in the presence of low BioE/high SHBG may contribute substantially to poor skeletal health. © 2012 American Society for Bone and Mineral Research. 相似文献
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The effect of anti‐emetic doses of dexamethasone on postoperative blood glucose levels in non‐diabetic and diabetic patients: a prospective randomised controlled study 下载免费PDF全文
M. Tien T. J. Gan I. Dhakal W. D. White A. J. Olufolabi R. Fink B. M. Mishriky H. J. Lacassie A. S. Habib 《Anaesthesia》2016,71(9):1037-1043
There are few data regarding postoperative hyperglycaemia in non‐diabetic compared with diabetic patients following postoperative nausea and vomiting prophylaxis with dexamethasone. Eighty‐five non‐diabetic patients and patients with type‐2 diabetes were randomly allocated to receive intravenous dexamethasone (8 mg) or ondansetron (4 mg). Blood glucose levels were measured at baseline and then 2, 4 and 24 h following induction of anaesthesia. In non‐diabetic patients, the mean (SD) maximum blood glucose was higher in those who received dexamethasone compared with ondansetron (9.1 (2.2) mmol.l?1 vs. 7.8 (1.4) mmol.l?1, p = 0.04). In diabetic patients, the mean (SD) maximum blood glucose was also higher in those who received dexamethasone compared with ondansetron (14.0 (2.5) mmol.l?1 vs. 10.7 (2.4) mmol.l?1, p < 0.01). Multivariate analysis demonstrated that dexamethasone administration was a significant predictor of maximum postoperative blood glucose increase (p < 0.01) after adjusting for potential confounders. There was no interaction between baseline blood glucose level, or presence or absence of diabetes, and dexamethasone administration. We conclude that dexamethasone increases postoperative blood glucose levels in both non‐diabetics and diabetics. 相似文献
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Yu-Wen Tien Ching-Yao Yang Yao-Ming Wu Rey-Heng Hu Po-Huang Lee 《Journal of gastrointestinal surgery》2009,13(5):929-937
Background
Since gastroparesis is unavoidable in a certain proportion of patients after pancreaticoduodenectomy, measures to avoid its occurrence or at least minimize its impact are needed. A prospective randomized trial was performed to test the effectiveness of biliopancreatic diversion with modified Roux-en-Y gastrojejunostomy reconstruction and of enteral feeding to minimize impacts of gastroparesis after pancreaticoduodenectomy. 相似文献30.