骨髓增生异常综合征最新研究进展:第56届美国血液学会年会报道

骨髓增生异常综合征(MDS)是一组起源于造血干/祖细胞水平损伤而产生的获得性克隆性疾病,以无效造血和易转化为急性白血病为特点.第56届美国血液学会(ASH)年会报道了大量MDS的相关发病机制及对患者预后、指导用药意义的研究,并就不同MDS的药物治疗及造血干细胞移植等方面做了详尽报道.     

Lambert–Eaton myasthenic syndrome – Diagnosis,pathogenesis and therapy

Lambert–Eaton myasthenic syndrome (LEMS) describes a rare human autoimmune disorder of the neuromuscular junction (NMJ). Clinically, LEMS patients suffer from characteristic muscle weakness that is caused by the presence of antibodies directed against their voltage-gated calcium channels (VGCC). These channels are localized in the presynaptic membrane of their motor nerve terminals. Binding of autoimmune antibodies to the VGCCs leads to reduced neuromuscular transmission. In approximately 50% of the patients, LEMS is reflected by a paraneoplastic manifestation and most commonly associated with a small cell lung carcinoma (SCLC) whose cells also express VGCCs in their plasma membrane. Better understanding of the pathophysiological mechanisms of LEMS has helped with the development of new diagnostic approaches and has led to targeted symptomatic and immunosuppressive therapy. For LEMS patients with an underlying malignancy, tumor therapy is the first choice to date.     

眼弓蛔虫病的研究进展

眼弓蛔虫病(Ocular toxocariasis)是由犬弓蛔虫的蚴侵入眼部各组织所引起的寄生虫病。一般认为是一种儿童病。本病遍布全世界。诊断试验虽有多种,直到近年才获得一定的突破。随着此病在全球范围内的传播,其研究工作也日益引起关注。本研究主要从眼弓蛔虫病的致病性原虫、临床特点、发病机制、诊断、治疗以及预防等最新研究成果的诸多方面进行了综述,为眼弓蛔虫病的诊断治疗、知识普及等奠定了基础。     

Clinical features and radiological manifestations of COVID-19 disease

Coronavirus disease 2019 (COVID-19) was discovered after unusual cases of severe pneumonia emerged in December 2019 in Wuhan Province (China). Coronavirus is a family of single-stranded RNA viruses. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is transmitted from person to person. Although asymptomatic individuals can transmit the virus, symptomatic patients are more contagious. The incubation period ranges from 3-7 d and symptoms are mainly respiratory, including pneumonia or pulmonary embolism in severe cases. Elevated serum levels of interleukins (IL)-2, IL-6, IL-7 indicate the presence of cytokine release syndrome, which is associated with disease severity. The disease has three main phases: Viral infection, pulmonary involvement, and hyperinflammation. To date, no treatment has proved to be safe or effective. Chest X-ray and computed tomography (CT) are the primary imaging tests for diagnosis of SARS-CoV-2 pneumonia, follow-up, and detection of complications. The main radiological findings are ground-glass opacification and areas of consolidation. The long-term clinical course is unknown, although some patients may develop pulmonary fibrosis. Positron emission tomography-computed tomography (PET-CT) is useful to assess pulmonary involvement, to define the affected areas, and to assess treatment response. The pathophysiology and clinical course of COVID-19 infection remain poorly understood. However, patterns detected on CT and PET-CT may help to diagnose and guide treatment. In this mini review, we analyze the clinical manifestations and radiological findings of COVID-19 infection.     

肝硬化腹水发病机制研究现状

腹水是肝硬化常见的并发症,也是临床上腹水的常见原因[1,2]。新发肝硬化患者10年内腹水的发生率高达50%～70%,腹水的出现被视为肝硬化病程的一个重要转折点,是肝硬化疾病进展和肝功能失代偿的重要表现。肝硬化患者腹水的发生不但会导致患者生活质量的下降[3],而且与肝硬化的其他并发症如自发性细菌性腹膜炎、肝肾综合征等密切相关。肝硬化腹水还与不良预后相关,1a病死率约为15%,5a病死率则高达44%[4]。对肝硬化腹水发病机制的研究以及新的治疗方法的探索有助于改善患者的生活质量和预后。然而,目前肝硬化腹水发病机制仍未完全阐明。     

胰源性区域性门静脉高压症的发病机制及影像学表现

胰源性区域性门静脉高压症与临床上常见的肝硬化型门静脉高压症的发病机制、影像学表现、诊治方法截然不同。作为为数不多的可以治愈的门静脉高压症,胰源性区域性门静脉高压症具有重要的临床研究价值。本文就胰源性区域性门静脉高压症的发病机制及影像学表现进行综述。     

基于痰湿毒瘀论对新型冠状病毒肺炎的中医治疗

中医将新型冠状病毒肺炎归属为“寒湿疫”、“湿毒疫”范畴,是“疫”邪引起,以湿邪为重要特征,由寒湿起病,在疾病早中期呈现寒湿袭表、阻肺、碍脾的临床表现,故其病位在表、在肺、在脾,呈现痰湿内蕴。随着病情的进展,出现聚湿成痰、化热化燥、湿毒壅肺,伤阴致瘀,甚者闭脱危象,总体病理变化表现为“痰、湿、毒、瘀”,痰湿为本,毒瘀为标。治疗当以化痰祛湿,贯穿始终,早期应用清热解毒、活血化瘀药可截断病情,提高疗效。     

炎症性肠病中药物性肝损伤的研究进展

炎症性肠病(inflammatory bowel disease,IBD)的治疗常使用氨基水杨酸制剂、免疫抑制剂、生物制剂等,这些药物均可能导致肝细胞损伤、胆汁淤积性肝炎,甚至暴发性肝衰竭。使用免疫抑制剂及生物制剂还可能激活潜在的肝炎病毒。除此之外,硫唑嘌呤还可能导致肝血管源性病变,如再生性结节。本文将概述治疗IBD的药物导致肝损伤的相关研究进展。     

乳腺癌皮肤转移患者误诊为皮脂腺囊肿伴感染一例诊断过程分析

乳腺癌是女性发病率和死亡率最高的恶性肿瘤,乳腺癌皮肤转移在临床上并不常见,且容易造成误诊。本文报道了1例于2019-02-25入住中国人民解放军第518医院的乳腺癌皮肤转移患者的诊断经过以及被误诊为皮脂腺囊肿伴感染的文献分析。初步探讨了乳腺癌皮肤转移复杂多样的临床表现以及发病机制,以提高临床医生对乳腺癌皮肤转移的认识,当诊断困难时需结合电子计算机断层扫描（CT）、病理学检查辅助诊断,从而早发现、早治疗,以免造成误诊、漏诊。     

基因多态性与PCOS发病机制相关性研究近况

多囊卵巢综合征（ PCOS）是一种以高雄激素血症、排卵障碍以及多囊卵巢为特征的病变。其发病机制至今尚未阐明,目前研究方向主要在基因多态性领域,基因研究表明胰岛素作用、类固醇激素的合成与代谢、性激素功能失调、卵泡形成等相关影响基因以及慢性炎症因子均可能参与PCOS的发病机制。