干燥综合征合并中枢神经系统脱髓鞘疾病的临床特点分析

目的:探讨干燥综合征合并中枢神经系统脱髓鞘疾病的临床特点。方法:回顾分析2007年至2011年收治的原发性干燥综合征(primary Sj觟gren′s syndrome,pSS)合并中枢神经系统脱髓鞘疾病患者的相关资料。结果:297例pSS患者中,有4例(1.35%)合并中枢神经系统脱髓鞘疾病。4例患者中,脱髓鞘病变在脑部者2例,在脊髓者2例。脱髓鞘病变位于脑部的患者主要表现为头晕、肢体乏力,而病变位于脊髓的主要表现为肢体乏力、损伤平面以下感觉减退、括约肌功能障碍。病变发生在脊髓的2例患者全部累及颈胸髓,1例患者纵向脊髓节段累及超过4个,为国内外较罕见。经激素及环磷酰胺治疗后,4例患者均好转出院。结论:pSS合并脱髓鞘疾病具有异质性特点,应结合临床特点及体液神经免疫学指标、神经影像具体分析诊断。     

Impact of tobacco smoking upon disease risk,activity and therapeutic response in systemic lupus erythematosus: A systematic review and meta-analysis

Systemic lupus erythematosus (SLE) is a complex disease with variable presentations, course and prognosis. Published studies present conflicting data regarding the impact of cigarette smoking on SLE risk, disease activity, clinical manifestations and treatment response. We performed a comprehensive literature search using Medline, EMBASE and the Cochrane Collaboration database, and hand searches of relevant bibliographies. All original studies investigating the relationship between smoking and SLE were included in TABALUP. Two investigators systematically extracted data from the relevant studies. When possible, meta-analyses were performed. The meta-analysis of 9 case-controls studies show an increased risk of SLE in current-smokers compared to never-smokers (OR: 1.49 [95%CI: 1.06–2.08]), while former-smokers were not at increased risk of SLE. Data on passive smoking remains scarce and controversial. Pooled analysis studies did not find an over-risk of anti-dsDNA, anti-Sm or anti-SSA positivity according to smoking status. Tobacco smoking significantly reduced the therapeutic effectiveness of hydroxychloroquine in cutaneous lesions (pooled OR 0.53; 95%CI: 0.305–0.927) and belimumab in systemic manifestations (HR 0.10; 95% CI 0.02–0.43). In addition to its usual adverse effects, cigarette smoking is a risk factor of SLE and negatively influences the course of the disease and its treatment. We believe that smoking cessation should be one of the main target of physicians treating SLE patients.     

Is the Management of Rectal Cancer Using a Watch and Wait Approach Feasible,Safe and Effective in a Publicly Funded General Hospital?

AimsAlthough there is emerging evidence to suggest equivalent oncological outcomes using a watch and wait approach compared with primary total mesorectal excision surgery, there is a paucity of evidence about the safety and efficacy of this approach in routine clinical practice. Here we report the long-term outcomes and quality of life from patients managed with watch and wait following a clinical complete response (cCR) to neoadjuvant therapy.Materials and methodsPatients with adenocarcinoma of the rectum with cCR following neoadjuvant therapy managed using watch and wait were retrospectively identified. Demographic data, performance status, pretreatment staging information, oncological and surgical outcomes were obtained from routinely collected clinical data. Quality of life was measured by trained clinicians during telephone interviews.ResultsOver a 7-year period, 506 patients were treated for rectal cancer, 276 had neoadjuvant therapy and 72 had a cCR (26.1%). Sixty-three were managed with watch and wait. Thirteen patients had mucosal regrowth. There was no significant difference in the incidence of metastatic disease between the surgical and watch and wait cohorts (P = 0.38). The 13 patients with mucosal regrowth underwent salvage surgery. Eleven of the patients who underwent surgical resection had R0 resections. There was also a statistically and clinically significant improvement in the Functional Assessment of Cancer Therapy – Colorectal (FACT-C) trial outcome index (P = 0.022).ConclusionThis study shows that watch and wait is safe and effective outside of tertiary referral centres. It suggests that an opportunistic cCR is durable and when mucosal regrowth occurs it can be salvaged. Finally, we have shown that quality of life is probably improved if a watch and wait approach is adopted.