Spinocerebellar ataxia types 2 and 3 segregating simultaneously in a single family.

Spinocerebellar ataxia (SCA) types 2 and 3 are autosomal-dominant neurodegenerative disorders caused by mutations in two different genes. We identified mutations for SCA2 and SCA3 segregating simultaneously in a single Brazilian family. The index patient had SCA2, whereas her two second-degree cousins had SCA3. Disease was more rapidly progressive in the SCA2 patient, who presented severe brainstem and pancerebellar atrophy, as opposed to the two SCA3 patients, who had only mild cerebellar vermian atrophy. In such situations, molecular confirmation of all patients may avoid misdiagnosis of SCA subtypes and eventual errors in predictive testing of unaffected family members.     

APACHEⅢ评分对脑卒中病人预后的评估及相关因素的研究

目的 观察APACHEⅢ评分系统对脑卒中病人预后的预测效果，并对其相关影响因素进行分析．方法 136例脑卒中病人入院后在接受治疗前进行APACHEⅢ评分，再分别计算出每一例的预测死亡危险度；入院7d内每天作1次APACHEⅢ评分，实际观察结果分为死亡和存活两种．最后按病灶的位置分为两组：中线组和非中线组；再按脑出血的体积或脑梗死的容积分为两组：大病灶组和小病灶组。然后进行统计学处理．结果 死亡组的APACHEⅢ评分明显高于存活组；中线组的APACHEⅢ评分显著高于非中线组，其中，中线组的急性生理学评分和神经学评分均显著高于非中线组，而两组的年龄及慢性健康状况评分无显著性差异．大病灶组的评分高于小病灶组，其中，大病灶组的急性生理学评分和神经学评分均盟著高于小病灶组，而年龄及慢性健康状况评分无显著性差异．预测死亡危险度为0．4时准确度最高．结论 APACHEⅢ评分系统对评估脑卒中病人病情危重程度及预测死亡危险度具有重要的参考价值．     

超选择性动脉溶栓治疗急性缺血性脑卒中临床研究

目的评价超选择性动脉溶栓治疗急性缺血性脑卒中的疗效和安全性。方法选择2003年1月至2005年5月于本院进行超选择性动脉溶栓同时采用卒中登记方法收集的58例急性缺血性脑卒中患者,随访6个月,观察患者的预后。结果58例患者中并发脑出血6例(10．3%),其中症状性脑出血2例(3．4%)。1个月末、3个月末和6个月末分别死亡2例(3．4%)、4例(6．8%)和5例(9．3%)。3个月末和6个月未残疾或死亡例数分别为13例(22．4%)和9例(16．7%)。结论对选择的急性缺血性脑卒中患者进行超选择性动脉溶栓是有效和安全的。     

加强基层微生物室生物安全防护

近年来，尤其是2003年发生的SARS疫情以及新加坡、台北、北京陆续发生的SARS冠状病毒实验室感染传播事件，使实验室的安全管理隐患变成了巨大的现实危害。这给各类微生物或生物医学实验室的生物安全防护敲响了警钟。作为基层微生物室工作人员每天都在接触含有不同致病性的微生物标本，其中也不排除含有风险级别较高、传播途径不明的微生物样品，但是由于基层微生物实验室的结构和设施、安全操作规范、安全设备、防护措施以及试验人员的安全防护意识却相对薄弱，因此为了试验人员、实验室及生态环境安伞，消除实验室生物安全隐患，卫生行政部门应重视基层微生物室的生物安全问题。     

Ruptured distal anterior choroidal artery aneurysm.

Ruptured aneurysms of the distal anterior choroidal artery (AchA) are extremely rare and management is dictated by clinical presentation. This report describes a rare patient with a distal AchA aneurysm and subarachnoid and intraventricular haemorrhage. A 60-year-old woman presented with a sudden onset of severe headache and vomiting. No aneurysms could be found on initial angiograms. A repeat angiogram performed 10 days after admission demonstrated an aneurysm in a branch of the cisternal segment of the left AchA without a definite neck. Surrounding vessels showed multifocal stricture and dilatation. Microsurgical clipping was not performed because the patient died suddenly due to pulmonary failure. The aetiology of the aneurysm in this case and surgical strategy are discussed.     

Cognitive function after on or off pump coronary artery bypass grafting.

OBJECTIVE: To investigate cognitive outcome after on and off pump coronary artery bypass grafting. METHODS: Seventy patients between 50 and 80 years with stable angina pectoris, ejection fraction >30%, serum creatinine <150 micromol/l, and lack of tight main stem stenosis were randomized to on or off pump coronary artery bypass grafting. Standardized neuropsychological tests evaluated attention, verbal and visuo-spatial short-term and working memory, verbal learning, delayed recall, visuo-motor speed, and aspects of executive functions. Levels of anxiety and depression were also investigated. Testing was performed before and at 1 week, 1 and 6 months after surgery. RESULTS: There was no difference in cognitive impairment (defined as a 20% reduction in at least 20% of the tests) between groups. The incidence at 1 week post-operatively was 57% in the on pump group and 58% in the off pump group, after 1 month 30% and 12% and after 6 months 19% and 15%, respectively (p for interaction=0.19). There was no difference between groups in anxiety (p=0.18) or depression (p=0.48). CONCLUSIONS: This prospective, randomized study showed no differences in post-operative cognitive function after on pump compared to off pump coronary artery bypass grafting in low risk patients.     

Spray coated pellets as carrier system for mucoadhesive drug nanocrystals.

High pressure homogenization can be employed to produce drug nanocrystals with a number of advantages, like improved solubility behaviors, better drug targeting or even increased mucoadhesiveness. To obtain a controlled drug delivery system it is necessary to transform the resulting nanosuspension into a solid dosage form. The present study shows the feasibility to use a mucoadhesive nanosuspension of poorly soluble hydrocortisone acetate produced by high pressure homogenization as layering dispersion in a fluidized bed process, followed by the application of an enteric coating to achieve a controlled drug release. To point out the advantages of drug nanocrystals the new fomulation was compared with a formulation containing micronized drug. Both formulations were characterized with regard to their particle size and crystallinity by using laser diffractometry, photon correlation spectroscopy and X-ray diffraction. The pellet morphology was characterized by using the environmental scanning electron microscopy (ESEM). In the in vitro dissolution tests an accelerated dissolution velocity and an increased drug release could be shown for the pellets containing drug nanocrystals.     

Blunted renal dopaminergic system activity in puromycin aminonucleoside-induced nephrotic syndrome.

BACKGROUND: A primary tubular sodium handling abnormality has been implicated in the edema formation of nephrotic syndrome. Dopamine synthesized by renal proximal tubules behaves as an endogenous natriuretic hormone by activating D(1)-like receptors as a paracrine/autocrine substance. METHODS: We examined the time courses of the urinary excretion of sodium, protein and dopamine in puromycin aminonucleoside (PAN)-treated and control rats. The rats were sacrificed during greatest sodium retention (day 7) as well as during negative sodium balance (day 14) for the evaluation of renal aromatic l-amino acid decarboxylase (AADC) activity, the enzyme responsible for the synthesis of renal dopamine. Also, the influence of volume expansion (VE) and the effects of the D(1)-like agonist fenoldopam (10 microg/kg bw/min) on natriuresis and on proximal tubular Na(+),K(+)-ATPase activity were examined on day 7. RESULTS: The daily urinary excretion of dopamine was decreased in PAN-treated rats, from day 5 and beyond. This was accompanied by a marked decrease in the renal AADC activity, on days 7 and 14. During VE, the fenoldopam-induced decrease in proximal tubular Na(+),K(+)-ATPase activity was more pronounced in PAN-treated rats than in controls. However, the urinary sodium excretion during fenoldopam infusion was markedly increased in control rats but was not altered in PAN-treated animals. CONCLUSION: PAN nephrosis is associated with a blunted renal dopaminergic system activity which may contribute to enhance the proximal tubular Na(+),K(+)-ATPase activity. However, the lack of renal dopamine appears not to be related with the overall renal sodium retention in a state of proteinuria.     

孕妇产前心理状态与需求的调查

目的对孕妇产前心理状态及其影响因素进行调查和评定,以探讨心理因素对分娩方式的影响。方法用综合医院焦虑/抑郁情绪测定量表,采用调查问卷法。结果有焦虑/抑郁症状组与无焦虑/抑郁症状组剖宫产率分别为80%和30%,差异有非常显著性(χ2=25.25,P<0.01);两组产后出血和新生儿阿氏评分,差异也有非常显著性,对婴儿的哺乳喂养方式(母乳、混合、人工)两组亦存在显著性差异(χ2=7.36,P<0.05)。结论产前对有焦虑/抑郁障碍的孕妇进行科学、整体的护理,实施有效的心理干预,可以减轻甚至消除焦虑/抑郁情绪,使孕妇处于最佳身心状态,保障母婴平安度过分娩期。     

上海市闵行区性病门诊本地和外来病人的比较研究

目的分析和比较上海市闵行区性病门诊本地与外来病人的流行病学特征。方法对2001~2004年上海市闵行区3家设有标准化性病门诊的公立医院进行门诊病历的回顾性分析。结果外来人口约占全部性病门诊病人的50%,其中女性病例占18.2%,未婚者占23.8%,均高于本地同类人员的比例;外来病人年龄偏低,多重感染者比例较高;男女外来病人与本地病人的首诊原因和症状排位相同,均以淋病感染为主;外来病人随访率(81.2%)较本地病人低(86.0%)。结论性病门诊本地和外来病人在人口学特征、诊疗行为上存在差异,防制措施应有所不同和侧重。