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The biochemical genetics of amyloid fibril proteins. 淀粉样纤维蛋白的生化遗传学。
Pub Date : 1989-01-01 DOI: 10.1007/BF02871789
A O Carbonara, A Bottaro

Amyloidoses are a very heterogeneous set of diseases, characterized by extracellular deposition of fibrillar proteins in different tissues. It is still a matter of debate whether the different forms of amyloidosis can share some common etiological mechanisms, or they are completely unrelated. The biochemical characterization of the protein component of the deposits provides a powerful system of classification for the different amyloidotic disorders and shades light on the molecular mechanisms of selective precipitation from soluble precursors and of tissue-specific deposition. Furthermore, identification and analysis of the genes coding for the precursors, and clarification of the kind of inheritance in some familial forms of amyloidosis, make prevention through genetic counselling and predictive diagnosis possible.

淀粉样变性是一种非常异质性的疾病,其特征是纤维蛋白在不同组织中的细胞外沉积。不同形式的淀粉样变是否具有一些共同的病因机制,或者它们完全不相关,这仍然是一个争论的问题。沉积物中蛋白质成分的生化表征为不同的淀粉样变性疾病提供了一个强大的分类系统,并为可溶性前体选择性沉淀和组织特异性沉积的分子机制提供了线索。此外,对前体编码基因的鉴定和分析,以及对某些家族性淀粉样变性的遗传类型的澄清,使通过遗传咨询和预测性诊断进行预防成为可能。
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引用次数: 0
HBeAg/anti-HBe circulating immune complexes in patients chronically infected with hepatitis B virus. 慢性乙型肝炎病毒感染患者的HBeAg/抗hbe循环免疫复合物
Pub Date : 1989-01-01 DOI: 10.1007/BF02871796
D Sansonno, A Vacca, A Gernone, F Dammacco

An enzyme-linked immunosorbent assay based on the ability of polyethylene-glycol (PEG) fixed on a solid support to adsorb circulating macromolecules (PEG-solid phase test) was developed in order to provide evidence for the existence of immune complexes of HBeAg/anti-HBe (HBeAg/anti-HBe complex) in sera of HBsAg chronic carriers. The method can detect HBeAg in immune complexes whether antigen or antibody is in excess. In the chronic phase of HBV infection, HBeAg/anti-HBe complexes are formed transiently in the course of the disease, unrelated to the phases of virus replication or peaks of hepatocytolysis, or to the histologic picture of liver disease. Our study indicates that this method offers a new approach to the understanding of biological and clinical problems of the HBeAg/anti-HBe antigenic system in chronic HBV infection.

建立了一种基于固定在固体载体上的聚乙二醇(PEG)吸附循环大分子能力的酶联免疫吸附试验(PEG-固相试验),以证明HBsAg慢性携带者血清中存在HBeAg/anti-HBe免疫复合物(HBeAg/anti-HBe复合物)。该方法可检测免疫复合物中是否存在抗原或抗体过量的HBeAg。在HBV感染的慢性期,HBeAg/抗hbe复合物在病程中是短暂形成的,与病毒复制的阶段或肝细胞溶解的高峰无关,也与肝脏疾病的组织学表现无关。我们的研究表明,这种方法为理解慢性HBV感染中HBeAg/抗hbe抗原系统的生物学和临床问题提供了新的途径。
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引用次数: 0
A survey of inherited thrombotic syndromes in Italy. ad hoc Study Group. 意大利遗传性血栓综合征的调查。特设研究小组。
Pub Date : 1989-01-01 DOI: 10.1007/BF02871794
A Tripodi, P M Mannucci

A collaborative survey was conducted among Italian thrombosis centers to gather information about the number and clinical features of patients with inherited thrombotic syndromes. The survey, based on 74 unrelated kindreds, revealed that antithrombin III, protein C and protein S defects are the most frequent genetic disorders. Venous thromboembolism was more frequent than arterial thrombosis, which was seen in only a minority of cases, most frequently with dysfibrinogenemia. About half of the patients developed venous thrombosis with a similar incidence in antithrombin III, protein S and protein C defects. About half of the symptomatic patients had recurrences and 40% developed thrombosis after a triggering factor, most frequently after surgery, during the puerperium, pregnancy, oral contraceptive intake or bed rest. Deep venous thrombosis prevailed and superficial thrombophlebitis was rare in antithrombin III-deficient patients, whereas deep venous thrombosis was present in about half and superficial thrombophlebitis in about one third of the cases with protein S and protein C defects. The probability to be free of thrombosis decreases with increasing age and at 35 years can be estimated to be 47% both for men and women. There is, however, a group of patients who are still free of thrombosis despite their older ages.

在意大利血栓中心之间进行了一项合作调查,以收集有关遗传性血栓综合征患者数量和临床特征的信息。这项基于74个不相关的亲属的调查显示,抗凝血酶III、蛋白C和蛋白S缺陷是最常见的遗传疾病。静脉血栓栓塞比动脉血栓形成更常见,仅在少数病例中可见,最常见的是纤维蛋白异常血症。约一半的患者发生静脉血栓形成,抗凝血酶III、蛋白S和蛋白C缺陷发生率相似。有症状的患者中约有一半复发,40%在触发因素后发生血栓形成,最常见的是手术后、产褥期、妊娠期、口服避孕药或卧床休息期间。在抗凝血酶iii缺乏的患者中,深静脉血栓形成盛行,浅表性血栓性静脉炎罕见,而在蛋白S和蛋白C缺陷的患者中,深静脉血栓形成约占一半,浅表性血栓性静脉炎约占三分之一。无血栓形成的概率随着年龄的增长而降低,估计在35岁时,男性和女性的概率都为47%。然而,有一组患者尽管年纪大了,但仍然没有血栓形成。
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引用次数: 21
[Relation between blood viscosity and ischemic pathology of an organ]. [血液黏度与器官缺血病理的关系]。
V Bartoli, L Mannini, F Fantoni, C Lupi
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引用次数: 0
[Localization factors for arteriosclerosis and thrombosis]. 【动脉硬化和血栓形成的定位因素】。
M Mancini, P Rubba
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引用次数: 0
[Cellular rheology in obliterative arterial diseases of the lower limbs]. [下肢动脉闭塞性疾病的细胞流变学]。
R Lombardini, G Ciuffetti, D Palazzetti, E Mannarino, L Pasqualini, A M Scarponi, M Mercuri, D Siepi
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引用次数: 0
[Hypercholesterolemia and hemorheological parameters]. [高胆固醇血症和血液流变学参数]。
M G Ranieri, C Muggia, A Martignoni, L Bobba, G Perani
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引用次数: 0
[Hemorheological factors and diabetic arterial and arteriolar diseases]. [血液流变学因素与糖尿病动脉和小动脉疾病]。
G Pozza, C Rocco, O Carandente
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引用次数: 0
[Study of some hemorheological parameters in acute and chronic venous pathology]. 急慢性静脉病理血液流变学参数的研究
G Gelmini, R Quintavalla, P Ferretti, A Marcato, R Delsignore
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引用次数: 0
[Effects of buflomedil hydrochloride on the rheological properties of the blood in chronic cerebrovascular diseases]. 盐酸丁咯地尔对慢性脑血管病患者血液流变学特性的影响
R Lombardini, G Ciuffetti, R Paltriccia, A M Scarponi, V Savastano, D Palazzetti, M Mercuri, L Parnetti
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引用次数: 0
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