沙依瓦克病的病理学研究

本文对沙依瓦克病患者的关节软骨、骨、滑膜、肌肉活检组织进行了光镜及电镜观察，主要的病理变化是关节软骨化生为纤维性软骨，基质内纤维成分多，主要为胶元纤维、软骨层变薄。软骨下骨质较致密，见纤维性软骨及骨化、有些区域软骨有聚积成巢现象。骨组织主要为松质骨、骨小梁瘦小、其间充满脂肪组织。滑膜及肌组织病变不明显。     

沙依瓦克病家庭聚集性分析

本文报告沙瓦克病具有明显的家庭聚集性，其遗传倾向较高，原因与遗传因素有关。同时提示沙依瓦克病的家庭聚集性与周围环境，饮水、饮食结构、居住条件、近亲婚配等因素无关。     

沙依瓦克病—7例新型畸形性骨关节病报告

本文首次报告在新疆喀什地区疏附县有一种具有百年历史,在近期才发现的以幼年起病,隐匿进展,四肢脊柱等负重关节呈多发性、对称性、无痛性和致残性畸形变,身材矮小且不妊育,但智力与头颅正常,无角膜及内脏损害,有遗传倾向等特点的新型畸形性骨关节病,在排除已知家族性和地方性类似疾病前提下,按其发生与发现的地区——沙依瓦克村而命名为沙依瓦克病(Sayiwak Disease)。报告了沙依瓦克病7例患者的临床表现、X线征象和部分实验室资料。有关流行病学、病原与发病学、治疗学等,尚在继续调研探讨之中。     

沙依瓦克病流行病学分布特征的调查研究

本文报告用流行病学方法调查了沙依瓦克病的分布特征，结果表明沙依瓦克病的发病有逐年增加趋势，且与出生的胎次有关。在亲缘关系人群中，沙依瓦克病的一级亲属患病率最高为５０．００％，二级亲属和三级亲属的患病率分别为２７．７８％，４．３５％；四级亲属为２０．５９％。沙依瓦克病家系中无血缘关系者不发病；迁移异地与原地患病无差别。     

沙依瓦克病与大骨节病骨与关节损害特点的比较研究

本文报道了新近发现的７例沙依瓦克病与２４８例大骨节病骨与关节损害特点的比较研究，通过分析两病的临床表现、Ｘ线征象与活体测量的结果认为：１．两病相似的临床表现有１８个，占８５．７１％，完全不同的临床表现有３个，占１４．２９％；２．沙依瓦克病的关节损害是外周四肢关节与中枢脊椎同时受侵，大骨节病是由外周四肢关节向中枢侵犯；３．沙依瓦克病身高、坐高低于正常人，躯干腿长指数在正常人范围值内；大骨节病人中仅Ⅲ°患者的身高与躯干腿长指数明显偏低，而坐高降低不显著。作者等认为，两病虽然有类似的临床表现，但属性质不同的两类骨关节病。     

沙依瓦克病人体中无机元素及饮用水卫生学的调查报告

本文报告了应用微分电位溶出法和示波极谱法检测８例沙依瓦克病人与同病区２４例健康人的血清，尿液，头发，以及病区饮用水中Ｃａ，Ｐ，Ｍｇ，Ｚｎ，Ｃｕ，Ｍｎ，Ｓｅ，Ｉ，Ｆ含量，以国家标准卫生检验方法对病区饮用水的ｐＨ值进行检测和卫生细菌学检查，用ＣＹＢＲ法做尿液粘多糖定性试验。检测结果：（１）血清Ｃａ，Ｐ，Ｍｇ含量略高于健康水平（Ｐ〈０．０５）。（２）尿液Ｉ含量在正常值低限。（３）头发Ｃａ，Ｐ，Ｍｇ含量略     

沙依瓦克病的异地病例调查

沙依瓦克病(Sayiwak disease,SD)是作者在新疆喀什地区疏附县百什克拉木乡沙依瓦克村发现的一种新型畸形性骨关节病。1992年,作者又对本病进行了异地病例的调查,结果在距离SD首发地沙依瓦克村2000余公里以外的新疆博乐市和阿勒泰市,发现2户共5例SD患者,男性3人,女性2人,他们都是来自沙依瓦克村近亲联姻者的后代,除1例生于原籍,另4例均在移居地出生。由此,进一步提示本病属于近亲联姻所致的遗传性、致残性骨关节病。     

沙依瓦克病—12例新型畸形性骨关节病的X线表现与鉴别

本文报告１２例以致残性，对称性，多关节，多部位的新型畸形性关节病，经过实地追踪调查与其家庭遗传有关，并按病人发病所有地新疆维吾尔自治区疏附县百什克拉木乡的沙依瓦克村而命名，提出了沙依瓦克病的Ｘ线表现，并与其他疾病相鉴别。     

新的遗传性骨软骨病—沙依瓦克病遗传方式的研究

本文采用分离分析和多基因阈值理论法，分析了沙依瓦克病的遗传方式。结果表明本病的遗传分离比率为０．３４０４，加权平均遗传度（率）为１８２．７２±１９．５０，作者认为沙依瓦克病符合常染色体隐性遗传方式，与多基因遗传方式不符。     

Higher Fitness Is Strongly Protective in Patients with Family History of Heart Disease: The FIT Project

Background

Cardiorespiratory fitness protects against mortality; however, little is known about the benefits of improved fitness in individuals with a family history of coronary heart disease. We studied the association between cardiorespiratory fitness and risk of incident coronary heart disease and all-cause mortality, hypothesizing an inverse relationship similar to individuals without a family history of coronary heart disease.

克山病人体组织中硒水平研究

本文对云南克山病及其他疾病死亡者的心、肝、肾和胸肌四种具有代表性的组织进行了硒含量测定分析,表明克山病人四种组织中硒含量均低于国内和国外健康人相应组织;亚急克四种组织中硒含量均相应地较慢克低;心肌硒含量又比其肾、肝组织为低,均有显著性差异。进一步证实了克山病人体内环境确实处于低硒状态。     

Deficiency of the Eighth Component of Complement and Recurrent Meningococcal Disease: A Case and Family Study

Abstract: A 16-year-old boy presented on four occasions to a New Zealand hospital. On the first admission he had severe Neissera meningitidis (group Y) meningitis requiring treatment. Sixteen, 17 and 26 months later he presented with similar but less severe symptoms which resolved spontaneously. On the latter admission the same meningococcal serogroup was isolated from his blood. He had no detectable serum C8 activity: investigation of his close family revealed four maternal and two paternal relatives tentatively designated heterozygotes for C8 deficiency.     

牙酸蚀症患者胃食管反流病相关知识横断面调查

目的 调查牙酸蚀症患者对胃食管反流病(GERD)相关知识的了解状况.方法 使用自行设计的调查问卷,采用随机抽样方法,对门诊148例牙酸蚀症患者进行GERD相关知识和症状调查.结果 34.5％牙酸蚀症患者存在GERD症状,仅有9.5％患者知晓GERD是牙酸蚀症的病因,对GERD危险因素知晓率均低于50％.结论 牙酸蚀症患者对GERD相关知识了解较少,应对其进行GERD相关知识的教育,以改善其预后.     

Influence of Caregiving on Lifestyle and Psychosocial Risk Factors Among Family Members of Patients Hospitalized with Cardiovascular Disease

Background  Few data have evaluated the relationship between caregiving and cardiovascular disease (CVD) risk. Objective  The purpose of this study was to determine the prevalence and predictors of caregiver strain and to evaluate the association between caregiving and CVD lifestyle and psychosocial risk factors among family members of recently hospitalized CVD patients. Design and Participants  Participants in the NHLBI Family Intervention Trial for Heart Health (FIT Heart) who completed a 6-month follow-up were included in this analysis (n = 263; mean age 50 ± 14 years, 67% female, 29% non-white). Measurements  At 6 months, standardized information was collected regarding depression, social support, and caregiver strain (high caregiver strain = ≥7). Information on lifestyle risk factors, including obesity, physical activity, and diet, were also collected using standardized questionnaires. Logistic regression models on the association between caregiving and CVD risk factors were adjusted for significant confounders. Results  The prevalence of serving as a CVD patient’s primary caregiver or caring for the patient most of the time was 50% at 6 months. Caregivers (primary/most) were more likely to be women (81% vs 19%, p < .01), married/living with someone (p < .01), >50 years old (p < .01), have ≤ high school education (p < .01), be unemployed (p < .01), get less physical activity (p < .01), and have a higher waist circumference (p < .01) than non-caregivers (some/occasional/none). Mean caregiver strain scores were significantly higher among those with depressive symptoms (p < .01) and low social support (p < .01) in a multivariable adjusted model. Conclusions  Caregivers of cardiac patients may be at increased risk themselves for CVD morbidity and mortality compared to non-caregivers due to suboptimal lifestyle and psychosocial risk factors.     

Prevalence of Coronary Artery Disease in Patients with Valvular Heart Disease

ABSTRACT. To evaluate the usefulness of preoperative coronary angiography in patients undergoing preoperative investigation because of valvular heart disease, we performed coronary angiography in a consecutive series of 329 patients. The prevalence of significant coronary artery disease was 32%. Asymptomatic coronary artery disease was present in 13%. Angina pectoris proved to be a poor predictor of coronary artery disease in aortic valve disease. In mitral valve disease, however, the specificity was high. A cost-benefit calculation was carried out in order to assess what advantage routine coronary angiography might have. According to this, coronary angiography should be performed in all patients suffering from valvular heart disease with angina pectoris, whereas it can be omitted in younger patients without angina. A cut-off point of 60 years seems appropriate for aortic valve disease and 65 years for mitral valve disease.