多发性硬化患者血清尿酸水平变化的研究

目的　探讨多发性硬化 (MS)患者血清尿酸 (UA)水平的变化及其临床意义。方法　采用酶定量分析法对 4 3例MS患者和 4 5名正常对照者的血清UA水平进行检测。结果　MS组血清UA水平明显低于对照组 (P <0 0 1)。MS组中病程越长 (P <0 0 1)、神经伤残程度越重 (DSS评分越高 ) (P <0 0 5 ) ,血清UA水平越低 ;女性患者UA水平明显低于男性患者 (P <0 0 0 1) ;经过糖皮质激素治疗后血清UA水平明显回升 (P <0 0 0 1) ,但治疗前血清UA水平越低则疗效越差 (P <0 0 1、P <0 0 5 )。结论　MS患者血清UA水平降低 ,且与MS的病程、伤残程度、疗效及性别密切相关。UA水平升高可能为激素治疗MS的一个作用机制     

早期扩髓髓内钉固定治疗合并胸部损伤的股骨干骨折

目的探讨合并胸部损伤的股骨干骨折早期扩髓及髓内钉固定是否增加并发症及死亡率. 方法采用回顾性对比分析,依据下列标准选择病例(1) 年龄在17～65岁;(2) 必须有胸部损伤[简明损伤定级(AIS)≥2], 且损伤严重度评分(ISS)≥16;(3) 住院时间≥48 h;(4) 病史中无明显影响全身状况的疾病, 如糖尿病, 慢性心、肺、肾功能不全等;(5) 有股骨干骨折, 且进行了扩髓髓内钉固定, 不含钢板、外固定支架或牵引及石膏固定者.并按受伤至手术时间划分为两组, A组为<24 h手术者(早期扩髓组), B组为≥24 h手术者(延期扩髓组),将两组间合并伤情况、ISS、住院时间、ICU时间、并发症、死亡及合并休克情况进行比较. 结果有96例符合上述标准, 其中A组57例, B组39例,经统计学处理, 两组间仅在股骨开放性骨折发生率(A组53%,B组31%,χ2=4.496, P<0.05)、合并休克率(A组51%, B组28%,χ2=4.895, P<0.05 )及住院时间[A组为(17.5±6.5)d, B组为(31.5±9.5)d,t=8.599, P<0.001]上差异有显著性意义和非常显著性意义, 而两组并发症发生率和死亡率等方面比较, 差异无显著性意义(P>0.05). 结论在合并胸部损伤的股骨干骨折中,只要能控制休克,保证生命体征平稳,对股骨干骨折行早期扩髓髓内钉固定不增加患者的并发症发生率及死亡率,并可促进患者早日康复,缩短住院时间.     

多系统萎缩的临床分型和影像学改变特点分析

目的探讨多系统萎缩（multiple system atrophy，MSA）的临床表现类型与神经影像学改变新特征（脑桥“十字征”和“壳核裂隙征”）的关系，为临床尽早做出诊断提供依据。方法按照Gilman诊断标准回顾性分析11例MSA患者的临床表现、分型和头颅MRI资料。结果本组诊断为很可能MSA11例，其中橄榄体脑桥小脑萎缩（MSA-C型）8例。2例在发病后3年头颅MRI脑桥“十字征”达Ⅰ期；1例在病后2年达Ⅱ期；3例分别在病后1年、3年、5年达Ⅲ期；另外2例分别在病后2年和7年达Ⅳ期。8例“壳核裂隙征”均为0期。黑质纹状体变性（MSA-P型）2例：1例病后6年脑桥“十字征”0期，“壳核裂隙征”Ⅰ期，另1例发病后9年“壳核裂隙征”Ⅱ期，脑桥“十字征”Ⅳ期。Shy-Drager综合征（MSA-A型）1例：病程5年，MRI检查脑桥“十字征”和“壳核裂隙征”分期均为0期。结论临床表现与头颅MRI检查发现的脑桥“十字征”和“壳核裂隙征”可作为及早识别MSA-C型的神经影像学改变特征，“壳核裂隙征”可作为识别MSA-P型的神经影像学改变特征。     

颅内多发动脉瘤18例临床分析

目的 探讨颅内多发动脉瘤的临床特点及诊治要点。方法 采用回顾性分析对18例共39个颅内多发动脉瘤临床资料进行分析。结果 女性13例，男5例，以1cm左右直径多见，全部经开颅手术治疗，疗效优良率83％(15／18)。结论 ①本组颅内多发动脉瘤多发于女性；②中等大小动脉瘤较常见，主要发生在前循环；③手术治疗效果较理想；④微型动脉瘤极易漏诊；⑤手术应先处理出血侧或易出血侧动脉瘤，并注意手术技巧及要点。     

Fertility in patients with multiple sclerosis: current knowledge and future perspectives

Abstract The issue of fertility in patients with multiple sclerosis (MS) has not been exhaustively studied. Epidemiological data have suggested that spontaneous fecundity might be reduced; several endocrine and sexual disturbances potentially interfering with reproduction have been evidenced in MS patients of both sexes. Moreover, some medical treatments used in MS (e. g., mitoxantrone, cyclophosphamide) may exert detrimental effects on spermatozoa as well as on oocytes, leading to early impairment of fertility. This review illustrates the factors potentially interfering with fertility in MS and discusses the therapeutic tools that may be used to promote fertility in these patients. The safety of hormonal therapies in MS is also examined. The current applications of assisted reproductive technology (ART) are discussed, including in vitro fertilisation (IVF) techniques. Currently available methods to preserve fertility in patients that undergo cytotoxic treatments by means of sperm/oocyte cryostorage or by ovarian fragment cryopreservation and autografting are considered.     

Multiple sclerosis: myelin basic protein induced mRNA expression of proinflammatory cytokines in mononuclear cells is suppressed by interferon-β 1b in vitro

Beta-interferon (IFN-β) is a promising treatment in multiple sclerosis (MS), reducing the exacerbation rate and MRI lesion burden, as well as the disease progression in relapsing-remitting MS. IFN-β was originally defined by its antiviral effects, but the interest has recently been focused on its immunomodulatory properties. Myelin basic protein (MBP) is one of several autoantigens considered to be the target for autoaggressive immune responses, which eventually might lead to the development of MS. To study in-vitro effects of IFN-β1b on MBP induced cytokine expression, mRNA for the Th1 cytokines IFN-γ and TNF-α, the Th2 related IL-4 and IL-6, the cytolytic perforin and the immune response downregulating TGF-β was measured with in situ hybridization after culture of blood mononuclear cells (MNC) in the presence and absence of MBP. Numbers of cells expressing IFN-γ, TNF-α, perforin and IL-4 mRNA were significantly suppressed after culture with 10 U/ml IFN-β1b. No such effect was seen on MBP induced IL-6 or TGF-β mRNA expression. These observations suggest that one of the major effects of IFN-β1b is the induction of a shift in the cytokine mRNA profile towards a more immunosuppressive pattern. In parallel in vitro tests, the control substance dexametasone (40 μg/ml) reduced the numbers of cells expressing mRNA for all cytokines under study with the exception of TGF-β, to an extent equal to or even more pronounced than IFN-β1b.     

Unusual presentation and clinical variability in Belgian pedigrees with progressive external ophthalmoplegia and multiple deletions of mitochondrial DNA

We studied 14 patients from three unrelated Belgian pedigrees with a familial mitochondrial disorder and multiple deletions of mitochondrial DNA (mtDNA). In one family with an oculopharyngeal presentation there is a clear autosomal dominant inheritance. Progressive external ophthalmoplegia (PEO), “ragged red fibres” (RRF) and multiple deletions of mtDNA are common to all three families. Therefore a diagnosis of autosomal dominant progressive ophthalmoplegia with multiple deletions of mtDNA (adPEO) was made in one family at least. Our data confirm the previous observations that adPEO is a systemic disorder rather than a pure myopathy. In our pedigrees frequently associated features include axonal peripheral neuropathy, dysphagia, psychiatric illness, and sudden death. Mild ataxia, pes cavus and mitral valve prolapse with associated mitral insufficiency also occur. In some cases onset is atypical with neuropathy, adolescent onset myopathy or psychiatric illness. In such cases the common features of PEO and muscle weakness always complete the clinical phenotype later during the course of the disease. Biochemical studies on mitochondrial fractions prepared from one patient's muscle, revealed no abnormalities of respiratory chain enzyme activities.     

Peripheral Blood Lymphocyte Surface Antigen Expression and Prognosis in Myeloma: Australian Leukaemia Study Group Study

The Australian Leukaemia Study Group myeloma study (MM1) aimed to determine the prognostic significance of clinical and immunophenotypic markers in patients with multiple myeloma. All patients were treated with standard dose melphalan and prednisone. Seventy-four patients were entered and the median survival was 27 months. Serum beta 2-microglobulin (βM) and albumin levels were the only significant clinical factors influencing survival (p = 0.007 and p = 0.008, respectively). Patients with raised levels of CD38+ lymphocytes at presentation had a significantly shorter survival than patients with normal levels (p = 0.01, logrank test, median 19 months vs 33 months). CD38 antigen expression was independent of β2M but patients with raised levels of CD38 had significantly lower levels of albumin than patients with normal levels (p = 0.001) which may explain their poorer survival. Salmon and Durie stage was not associated with antigen expression. No other B-cell antigens (CD10, CD19, CD20, CD21, CD22, CD23, FMC1 or FMC7) or plasma cell antigens tested (PCA-1) were found to be associated with prognosis. Patients who achieved plateau phase had a better prognosis than those who did not (p = 0.04 in a landmark analysis). Patients who achieved plateau phase following an objective response appeared to have a better prognosis than those who were in plateau phase at presentation (p = 0.09 in a landmark analysis). Light chain isotype suppression (LCIS) was not associated with a significant survival advantage and did not correlate with any known prognostic indicator. We conclude that phenotypic analysis of peripheral blood lymphocytes for CD38 antigen at diagnosis may be useful as a prognostic indicator in patients with myeloma.     

凝血机制紊乱在SIRS发展中的作用

凝血机制紊乱在全身炎症反应综合症(SIRS)发展、恶化过程中起重要作用．对SIRS时凝血系统紊乱的形成机制、在SIRS发生发展过程中的作用及其对病情危重程度的预测价值作一综述．     

Glutamine and Other Amino Acid Losses During Continuous Venovenous Hemodiafiltration

Abstract: Serum amino grams and daily losses of glutamine (Gin) and other amino acids (AAs) into diafiltrate were measured during the first 5 days of continuous venovenous hemodiafiltration (CVVHDF) in 6 ICU patients with acute renal failure (ARF). Four patients had ARF as a part of multiple organ failure (MOF) of septic origin, and 2 patients had isolated ARF because of primary renal disease. During the study, all the patients received defined total parenteral nutrition (TPN). The mean daily AA losses into dialysate were relatively low (0.61 ± 0.1 g N ) and reached 4.5% of the daily AA substitution. Gln represented 32.7 ± 5.9% of the total AA losses (0.19 ± 0.04 g N ). Serum levels of Gin (p = 0.002) and of most other AAs were significantly lower in the patients than in the control subjects (AA analysis in 16 healthy volunteers). Phenylalanine (Phe) was the only AA that was increased significantly (p < 0.01) in the patients. The mean patient serum concentrations of Phe and tyrosine were significantly higher (p < 0.03) than the correspondent concentrations in dialysate, but the lysine concentration was higher in dialysate (p < 0.03). The serum and dialysate concentrations of other AAs did not differ. Gin in serum decreased significantly (p < 0.03) on the second day of CVVHDF but returned to the baseline levels subsequently. Serum concentrations of Phe increased on the second day of CVVHDF (p < 0.05). Serum concentrations of other AAs remained stable during the whole study. We conclude that Gin losses into dialysate during CVVHDF are relatively low, but CVVHDF itself may induce changes in Gin metabolism and distribution that are reflected by a decrease of serum Gin levels at the institution of this treatment. Therefore, the need for Gin supplementation in ICU patients is even greater in the first days of CVVHDF.