帕金森病痴呆危险因素的研究进展

原发性帕金森病（Parkinson's disease,PD）是一种好发于中老年人的常见中枢神经系统变性疾病。痴呆是PD患者的一种常见非运动症状,表现为痴呆的患者被称为帕金森病痴呆（PD dementia,PDD）。PDD严重影响了PD患者的社会功能及生活质量,对PD患者的预后造成不良影响。目前的研究发现,PDD危险因素包括人口学特征、生活习惯、运动症状、非运动症状、生物学标志物、基因、影像学类标志等各种因素,本文旨在对这些因素的相关研究结果进行综述。     

Modern surgical strategies for perianal Crohn's disease

One of the most challenging phenotypes of Crohn’s disease is perianal fistulizing disease (PFCD). It occurs in up to 50% of the patients who also have symptoms in other parts of the gastrointestinal tract, and in 5% of the cases it occurs as the first manifestation. It is associated with severe symptoms, such as pain, fecal incontinence, and a significant reduction in quality of life. The presence of perianal disease in conjunction with Crohn’s disease portends a significantly worse disease course. These patients require close monitoring to identify those at risk of worsening disease, suboptimal biological drug levels, and signs of developing neoplasm. The last 2 decades have seen significant advancements in the management of PFCD. More recently, newer biologics, cell-based therapies, and novel surgical techniques have been introduced in the hope of improved outcomes. However, in refractory cases, many patients face the decision of having a stoma made and/or a proctectomy performed. In this review, we describe modern surgical management and the most recent advances in the management of complex PFCD, which will likely impact clinical practice.     

Paget's Disease of Bone: Diagnosis and Treatment

Paget's disease of bone is a common bone disorder characterized by disorganized bone remodeling. The most likely etiology is a slow paramyxoviral infection in genetically susceptible individuals; however, the exact cause is unknown. Enhanced bone resorption due to an increased activity of osteoclasts recruits numerous osteoblasts to resorption sites, with large quantities of new bone matrix produced as a result. The accelerated bone resorption and formation are not as closely coupled as in a healthy bone; a disorganized bone tissue is formed. Many patients are asymptomatic; rising serum alkaline phosphatase or incidental finding of characteristic radiographic lesions are often the only diagnostic clues. Common clinical manifestations include bone pain, bowing of long bones, enlarged skull, and hearing loss. An elevated serum alkaline phosphatase level correlates with the disease activity. The diagnosis is confirmed by characteristic radiographic findings and by nuclear scintigraphy of the bone (the most sensitive test). Bisphosphonates, such as zoledronic acid, pamidronate, alendronate, and risedronate, are the mainstay of treatment. Patients who do not tolerate bisphosphonates can be treated with calcitonin.     

Microbial manipulation as primary therapy for Crohn’s disease

While antimicrobials are clinically effective in preventing post-operative recurrence,the role for antibiotics in primary therapy for Crohn’s disease(CD) remains unclear.The recent multicenter phase 2 trial by Prantera et al received wide attention because it demonstrated an increase in the week 12 remission rate in patients with moderately active CD treated with rifaximin and renewed interest in microbial manipulation as primary therapy for CD.In this commentary,we discuss aspects of durability,immune cell polarization,and safety of microbial manipulation as primary therapy for CD.     

Optical coherence tomography findings in Parkinson's disease

The aim of this study is to compare optical coherence tomography (OCT) findings of retinal thickness (RT) and retinal nerve fiber layer thickness (RNFLT) of idiopathic Parkinson's disease (IPD) patients to those of healthy subjects, and to investigate whether there is any relationship between the severity of the disease and the RNFLT values. This prospective study was included 25 IPD patients and 29 healthy controls. In the IPD group, the Hoehn and Yahr (H&Y), Unified Parkinson's Disease Rating Scale (UPDRS), and Mini-Mental State Exam (MMSE) were performed. Intraocular pressure (IOP), visual acuity (VA), spherical equivalent, axial length (AL), and central corneal thickness (CCT) were measured using OCT in both groups. The RT was measured in the central retinal (RTc), nasal (RTn), and temporal (RTt) segments. Nasal (RNFLTn), nasal superior (RNFLTns), nasal inferior (RNFLTni), temporal (RNFLTt), temporal superior (RNFLTts), and temporal inferior (RNFLTti) measurements were made and mean RTFLT was calculated (RNFLTg) for each individual. In the patient group, IOP and VA values were statistically significantly lower The RTn and RNFLTg were significantly thinner in the patient group. There was no statistically significant relationship between the severity of IPD and these findings. In our study, RNFLTg and RTn were found to be thinner in the IPD group, which may have caused lower VA scores. The effects of retinal dopamine depletion on RT and RNFLT, and lower IOP values in the non-glaucomatous IPD patients should be further investigated.     

26例胸内局限性Castleman病诊治分析

目的 探讨胸内局限性Castleman病的诊断及治疗,提高对该病的认识及诊治水平.方法 回顾性分析2010年1月至2016年12月中国医科大学附属第一医院胸外科诊治的26例胸内局限性Castleman病患者的临床资料及病理资料.结果 26例患者中男14例,女12例;中位年龄为36.5 (24.8～69.3)岁;病程中位数为2.8(0.2～7.8)个月.所有患者均行手术治疗,其中常规开胸手术16例,电视辅助胸腔镜手术10例.26例患者术后均获得随访,中位随访时间为46.6 (4.2～70.6)个月.22例病理类型为透明血管型,4例为浆细胞型.除1例浆细胞型Castleman病患者于术后42个月复发外,其余患者术后均达到持续完全缓解,复发患者行术区局部放疗后目前处于部分缓解.结论 胸内局限性Castleman病手术治疗效果良好,尤其是透明血管型术后无复发,但浆细胞型术后有复发的可能性.     

Immunologic aspects of Graves' and Hashimoto's diseases

It is hypothesized that Graves' hyperthyroidism, exophthalmos, and Hashimoto's thyroiditis (as well as other closely related organ-specific autoimmune disorders) may each be attributed to separate, albeit very closely related, inherited isolated defects in immunoregulation (possibly specific defects in suppressor T lymphocyte function). The gene responsible for each of these disorders may lie in close linkage disequilibrium with HLA-Dw3 on chromosome 6. Each of the defects in immunoregulation, however, would permit a specific randomly mutating self-reactive “forbidden” clone of “helper” T lymphocytes to survive (if it chanced to appear), interact with its complementary antigen, and induce a cell-mediated immune response. This would not require any alteration of the antigen, only the mere availability of the specific antigen. The clone of self-reactive T lymphocytes, so arising and escaping immunoregulation, would presumably then expand upon interaction with its antigen, and consequently direct and cooperate with appropriate groups of (already present) B lymphocytes, which in turn would produce specific immunoglobulins that appear necessary for the full expression of these disorders. Adjunctive roles for immune complexes, nonspecific cells (macrophages, “killer” cells), and chemical mediators are undoubtedly important. The role of stress in the induction of hyperthyroidism may be by means of its effect in further reducing immunosuppression in those persons with only a partial isolated defect. On the other hand, remissions may be brought about by restoring immunoregulation to its previous state. Those persons having an isolated complete defect in immunoregulation would not be expected to achieve remissions, except by destruction of thyroid parenchyma.     

Graves病甲状腺功能亢进症性肝损害的相关因素分析

目的 探讨影响Graves病甲状腺功能亢进症(甲亢)性肝功能损害的相关因素.方法 回顾性分析天津医科大学总医院2013年1月至2015年12月收治的Graves病住院患者254例,根据肝功能将患者分为Graves病甲亢性肝损害组(A组,n=159)和甲亢肝功能正常组(B组,n=95),比较两组的基础代谢率(BMR)、甲状腺重量、FT3、FT4、促甲状腺激素(TSH)、TSH受体抗体(TRAb)、甲状腺球蛋白抗体(TgAb)、甲状腺过氧化物酶抗体(TPOAb).采用Pearson相关性分析甲状腺重量、BMR、FT3、FT4、TRAb与甲亢性肝损害的相关性,应用Logistic回归分析甲亢性肝损害的独立危险因素.结果 A组的甲状腺重量、BMR、FT3、FT4、TRAb、TPOAb均高于B组,而TSH低于B组(t或z=-4.720～-2.276,P均＜0.05).Pearson相关性分析显示,甲状腺重量、BMR、FT3、FT4、TRAb与甲亢性肝损害的发生呈正相关(r=0.157～ 0.270,P均＜0.05).Logistic回归分析显示,FT3(OR=1.052,95％ CI:1.001～1.105)、BMR(OR=1.019,9.5％ CI:1.006 ～ 1,033)是Graves病甲亢性肝损害发生的独立危险因素(P均＜0.05).结论 Graves病甲亢性肝损害与FT3、FT4、TRAb、BMR、甲状腺重量有关.其中FT3、BMR为甲亢性肝损害发生的独立危险因素.     

Differences in clinical features of Crohn’s disease and intestinal tuberculosis

AIM: To investigate the clinical features of Crohn’s disease(CD) and intestinal tuberculosis(ITB) with a scoring system that we have developed.METHODS: A total of 25 CD and 40 ITB patients were prospectively enrolled from August 2011 to July 2012.Their characteristics and clinical features were recorded. Laboratory, endoscopic, histologic and radiographic features were determined. The features with a high specificity were selected to establish a scoring system. The features supporting CD scored +1, and those supporting ITB scored-1; each patient received a final total score. A receiver operating characteristic(ROC) curve was used to determine the best cut-off value for distinguishing CD from ITB.RESULTS: Based on a high specificity of differentiating between CD and ITB, 12 features, including longitudinal ulcers, nodular hyperplasia, cobblestone-like mucosa, intestinal diseases, intestinal fistula, the target sign, the comb sign, night sweats, the purified protein derivative test, the interferon-γ release assay(T-SPOT.TB), ring ulcers and ulcer scars, were selected for the scoring system. The results showed that the average total score of the CD group was 3.12 ± 1.740, the average total score of the ITB group was-2.58 ± 0.984, the best cutoff value for the ROC curve was-0.5, and the diagnostic area under the curve was 0.997, which was statistically significant(P < 0.001). The patients whose total scores were higher than-0.5 were diagnosed with CD; otherwise, patients were diagnosed with ITB. Overall, the diagnostic accuracy rate and misdiagnosis rate of this scoring system were 97% and 3%, respectively. CONCLUSION: Some clinical features are valuable for CD and ITB diagnosis. The described scoring system is key to differentiating between CD and ITB.