Inclusion body myositis: The interplay between ageing,muscle degeneration and autoimmunity

Inclusion body myositis (IBM) is a slowly progressive muscle disease affecting ageing individuals. IBM presents with a distinctive pattern of weakness involving the quadriceps and finger flexor muscles, although other muscles including pharyngeal muscles become affected over time. Pathological hallmarks of IBM include autoimmune features, including endomysial infiltration by highly differentiated T cells, as well as degenerative features marked by intramyofibre protein aggregates organised into inclusion bodies. Despite some progress in understanding the cellular pathways involved in IBM, it remains untreatable, and the progression of the disease leads to progressive weakness, disability, wheelchair dependency and loss of independence. Therefore, there is an urgent need to improve our understanding of the underlying mechanisms and pathways involved in this disease to identify new treatment targets. Here, we discuss the current understanding of aetiopathogenesis, the interrelationship between autoimmunity and degeneration, and how ageing is a major influencer of both these features.     

多发性肌炎发病机制中的血管因素研究

目的　研究多发性肌炎中细胞间黏附分子 1(ICAM 1)、IL 1α在微血管的表达情况,探讨血管因素在多发性肌炎中的作用。方法　收集 10例多发性肌炎患者和 6例非炎性肌病,采用图像分析法计算CD34、ICAM 1、IL 1α阳性微血管面积与面密度,进行定量分析和比较。结果　多发性肌炎患者肌组织中微血管面密度较非炎性肌病组明显减少 (P=0 009 );微血管内皮ICAM 1、IL 1α的表达强度较对照组增强。结论　多发性肌炎患者肌组织微循环功能受损,提示血管因素可能参与了多发性肌炎的发病。     

儿童急性肺源性心脏病的发病机制

急性肺源性心脏病通常继发于严重肺部疾病或肺循环障碍所导致的肺动脉高压,以急性右心功能障碍、右心衰竭为主要特征。其中,低氧血症、高碳酸血症及正压通气均可导致肺动脉高压的发生。在危重患儿救治过程中,特别是呼吸支持过程中,应早期识别急性肺源性心脏病,考虑实施以右心功能监护和保护为核心的"肺保护"及"循环保护"。     

The role of apoptosis in childhood Henoch–Schonlein purpura

The pathogenesis of vasculitis is complex and is yet to be fully elucidated, although it is known that inflammatory cells play a major role. Dysregulation of apoptosis and defective clearance of inflammatory cells could lead to the persistence of inflammation and excessive tissue injury. In this study we aimed to investigate Fas (CD95) and apoptosis on peripheral blood (PB) neutrophil and lymphocytes in Henoch–Schonlein purpura, both in the acute phase and after resolution to determine the role of apoptosis in this self-limited vasculitis. Leukocytoclastic vasculitis presenting with Henoch–Schonlein purpura (HSP) was diagnosed according to ACR 1990 criteria and confirmed by skin biopsy. Thirty-seven patients (22 boys, 15 girls) aged 2.5–17 years (9 ± 3.3) were enrolled in the study. Expression of CD95 and apoptosis were investigated by the annexin/PI method on peripheral blood neutrophils and lymphocytes in both the acute and the resolution phases of the disease. The mean neutrophil and lymphocyte CD95 expression was 65.4 ± 37.6% and 33.3 ± 7.3%, respectively, in the acute stage and 62.8 ± 44.2% and 41 ± 20%, respectively, in the resolution (P > 0.05). The percentage of apoptotic peripheral blood neutrophils and lymphocytes as determined by annexin positivity was 13.3 ± 11.31% and 8.6 ± 9.5%, respectively, during the acute phase and 4.6 ± 3.4% and 3.1 ± 3.1%, respectively, in the resolution (P = 0.002, P = 0.008). These results suggest that increased apoptotic process in the immune effector cells in the acute phase of the disease may play an important role in the early control of inflammatory response and repair in leukocytoclastic vasculitis, thereby contributing to the self-limited nature of the disease.     

The Effect of Melatonin in Patients with Fibromyalgia: A Pilot Study

The aim of the study was to determine the possible effect of melatonin treatment on disturbed sleep, fatigue and pain symptoms observed in fibromyalgia (FM) patients. Twenty-one consecutive patients with FM were included in an open 4-week-duration pilot study. Before and after treatment with melatonin 3 mg at bedtime, patients were evaluated using tender point count by palpation of 18 classic anatomical regions, pain score in four predesignated areas, pain severity on a 10 cm visual analogue scale (VAS), sleep disturbances, fatigue, depression, anxiety, and patient and physician global assessments, also by a VAS. Urine 6-sulphatoxymelatonin levels (aMT-6S) were measured in the patients and 20 age- and sex-matched controls. Nineteen patients completed the study. One patient withdrew because of migraine and another was lost to follow-up. At day 30, median values for the tender point count and severity of pain at selected points, patient and physician global assessments and VAS for sleep significantly improved with melatonin treatment. Other variables improved but did not reach statistical significance. Adverse events were mild and transient. Lower levels of aMT-6S were found in FM patients compared with normal median controls (±SD, 9.16 ± 7.9 μg/24 h vs 16.8 ± 12.8 μg/24 h) (p= 0.06). Although this is an open study, our preliminary results suggest that melatonin can be an alternative and safe treatment for patients with FM. Double-blind placebo controlled studies are needed. Received: 14 September 1998 / Accepted: 14 May 1999     

Human growth hormone as a regulator of blood glucose concentration and as a diabetogenic substance

Summary Human growth hormone (HGH) has recently been shown to play a prominent role in the control of blood glucose homeostasis. Furthermore, it has long been known that administration of growth hormone in animals can induce a diabetes-like state. In human subjects, exogenous administration of HGH or hypersecretion of the endogenous hormone in acromegaly is accompanied by glucose intolerance in only about 25 per cent of the cases. — In this paper, data are presented which give a more diversified picture of the so-called diabetogenic action of HGH. It is suggested that HGH, although decreasing the peripheral utilization of glucose, is not a primary diabetogenic factor, since its insulinogenic action causes a compensatory hyperinsulinism, with normal glucose tolerance as the result. HGH is diabetogenic only in prediabetic subjects whose pancreas is unable to respond to the insulinogenic effect of the hormone. In such subjects, the diabetogenic action of HGH not being counterbalanced by a compensatory hyperinsulinism, glucose intolerance may result. Thus, HGH may be regarded as anadditional factor for the development of diabetes, the major prerequisite being a preëxisting prediabetic state.Presented as an invited lecture at the VI Acta Endocrinologica Congress, Helsinki, Finland, August 8th–12th, 1967.     

浅谈糖尿病肾病的研究进展

糖尿病肾病(Diabetic nephropathy,DN)是糖尿病的主要并发症,在我国,该病的发病率逐年增加,目前已成为终末期肾脏病的第2位原因,仅次于各种肾小球肾炎,该疾病已严重影响患者的生命健康及生活质量。该文从糖尿病肾病的发病机制出发,对其发病相关因素进行分析与概括,并结合现有临床诊断方法对糖尿病肾病五期诊断提供指标参考依据,同时对该病治疗手段做综述分析,进一步了解近期糖尿病肾病的研究动向。     

肝细胞损伤的分子生物学机制

随着医学研究的不断进展,我们对各种肝脏疾病的病因有了更加深入的了解,对疾病的治疗也渐渐从对症治疗转移到病因治疗,但是,在强调病因学治疗的同时,不应忽略对肝细胞的保护,因为肝细胞损伤是各型肝病共同的病理基础,是各种原因引起肝脏疾病的共同的表现.