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41.
Polycystic Kidney Disease Re-evaluated: A Population-based Study   总被引:2,自引:0,他引:2  
A genetic register of all known cases of autosomal dominantpolycystic kidney disease occurring in South and Mid-Wales hasbeen established. In a population of 2.1 million, 209 familieswith affected members were identified, 303 of whom are currentlyalive, 70 on renal replacement therapy. An additional 551 caseswould be predicted amongst family members at 50 per cent and25 per cent risk, giving an apparent prevalence of 1:2459 inthe general population. Five possible new mutations were seenwhere adults with phenotypic autosomal dominant polycystic kidneydisease had both parents alive, age > 55 years with no cystsvisible on ultrasound. The take-on rate for renal replacementtherapy increased during 1970–79 but has apparently reacheda plateau of 4.8 cases per million population per year overthe last 8 years, despite a rapidly increasing acceptance ofuraemic patients as a whole (72/106/year in 1988–89).Considerably more patients with autosomal dominant polycystickidney disease aged over 50 years were started on treatmentin 1980–89 than in 1970–79, but the survival overallimproved with time. All cases of autosomal dominant polycystickidney disease reaching end-stage renal disease are now beingtreated, but the apparent clinical prevalence of this conditionin our region is less than half the supposed gene frequency,suggesting that undiagnosed cases have a benign prognosis.  相似文献   
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OBJECTIVE: To assess the ability of contrast enhanced computed tomography (CECT) to detect active hemorrhage and other vascular injuries in chest, abdominal, and pelvic trauma patients, using angiographic findings and need for intervention as paired gold standards. METHODS: We obtained approval from the Institutional review board for a retrospective search of the radiology information system: seeking trauma patients undergoing angiography within 24 hours of CECT for chest, abdominal, or pelvic injuries. CECT protocol was standard trauma CT, not specialized for CT angiography. Angiographic techniques varied with indication. Clinical and imaging reports and selected radiologic studies were reviewed, but the original dictated report was the interpretive standard. We used Fisher's exact test for statistical analysis. RESULTS: During the 30-month study period, 466 patients underwent emergent interventional radiologic procedures. Of those, 418 were excluded for nontrauma indications or neuroangiographic procedures. Fourty-eight patients (33 male, 15 female, average age 43.4) thus constituted the study population in whom we evaluated 63 traumatic injuries. CT findings had statistically significant associations (p < 0.0001) with both angiographic evidence of active hemorrhage and the need for intervention, which were tabulated separately. CT had 94.1% sensitivity and 97.6% negative predictive value (NPV) for detection of active hemorrhage, and 92.6% sensitivity and 91.2% NPV for predicting need for surgical or endovascular intervention. CONCLUSIONS: CECT findings correlate strongly with angiographic findings, though sensitivity remains imperfect. However, when CT is used in the context of other clinical features, particularly hemodynamic instability, it may enable clinicians to reserve emergent angiography for those patients in whom emergent intervention is planned.  相似文献   
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Ku70 was first characterized as a nuclear factor that binds DNA double-strand breaks in nonhomolog end-joining DNA repair. However, recent studies have shown that Ku70 is also found in the cytoplasm and binds Bax, preventing Bax-induced cell death. We have shown that, in neuroblastoma cells, the binding between Ku70 and Bax depends on the acetylation status of Ku70, such that, when Ku70 is acetylated, Bax is released from Ku70, triggering cell death. Thus, to survive, in neuroblastoma cells, cytoplasmic Ku70 acetylation status is carefully regulated such that Ku70 is maintained in a deacetylated state, keeping Bax complexed with Ku70. We have shown that overexpression of CREB-binding protein (CBP), a known acetyltransferase that acetylates Ku70, releases Bax from Ku70, triggering apoptosis. Although we have shown that blocking deacetylase activity using non-type-specific inhibitors also triggers Ku70 acetylation and Bax-dependent cell death, the targets of these deacetylase inhibitors in neuroblastoma cells remain unknown. Here, we demonstrate that, in neuroblastoma cells, histone deacetylase 6 (HDAC6) binds Ku70 and Bax in the cytoplasm and that knocking down HDAC6 or using an HDAC6-specific inhibitor triggers Bax-dependent cell death. Our results show that HDAC6 regulates the interaction between Ku70 and Bax in neuroblastoma cells and may be a therapeutic target in this pediatric solid tumor.  相似文献   
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Yip  Ronald ML  Cheung  Tommy T  So  Ho  Chan  Julia PS  Ho  Carmen TK  Tsang  Helen HL  Yu  Carrel KL  Wong  Priscilla CH 《Clinical rheumatology》2023,42(8):2013-2027
Clinical Rheumatology - Gout is one of the most common noncommunicable diseases in Hong Kong. Although effective treatment options are readily available, the management of gout in Hong Kong remains...  相似文献   
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A study of violent behaviour among psychiatric inpatients in a large general hospital is presented. Over the study period of one year a total of 36 incidents of violence involving 26 patients were recorded. Schizophrenia was the most common diagnosis among assailants. Fellow patients were the main victims. Incidence of serious violence was low. Most incidents occurred in the night hours, from inmates of acute wards and mostly without any provocation.KEY WORDS: Violent behaviour, Psychiatric patients  相似文献   
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细粒棘球蚴重组抗原B的表达提取及其血清学检测初探   总被引:4,自引:0,他引:4  
用基因工程技术制备出的细粒棘球绦虫重组抗原B(rAgB)表达载体,经诱导表达、亲和层析纯化获得具生物活性的重组蛋白质rAgB,用Western-Blot法检测病人血清。结果显示rAgB敏感性为91.6%(44/48),特异性为93.8%(30/32),其中10例泡球蚴(AE)病人及10例肿瘤病人血清均无交叉反应。说明rAgB具有较高的敏感性及特异性,可用于包虫病的常规血清学诊断,rAgB在宿主菌JM109内稳定表达,因此可在实验室内大量制备用于血清学诊断的rAgB。  相似文献   
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