血同型半胱氨酸与危重新生儿弥漫性血管内凝血相关性研究

目的 探讨血同型半胱氨酸与危重新生儿弥漫性血管内凝(DIC)的相关性.方法 2004年8月至2007年1月收住我院NICU的危重新生儿共157例,其中诊断为DIC的36例为试验组,其余无合并DIC的危重新生儿中随机选取40例为对照组.对两组的血清同型半胱氨酸的水平进行对比,而且将试验组根据所处的时期分为A、B两组,A组20例,为DIC早中期,B组16例,为DIC晚期,研究在DIC早中期与晚期的疾病的转归及血清同型半胱氨酸的水平的相关性.结果 DIC新生儿组患儿血清同型半胱氨酸的水平明显高于无合并DIC的危重新生儿,而且DIC早中期血清同型半胱氨酸的水平明显升高,病情进展为DIC晚期血清同型半胱氨酸的水平迅速下降,而病情逐渐好转者,则逐渐下降.结论 血清同型半胱氨酸的水平与危重新生儿DIC是密切相关的.而且血清同型半胱氨酸的水平随DIC早中期病情的进展的不同而发生动态的变化.     

Severe cerebral involvement in adult-onset hemophagocytic lymphohistiocytosis

The diagnosis of hemophagocytic lymphohistiocytosis (HLH) with cerebral involvement is challenging given the rarity of HLH and its resemblance to the much more common severe sepsis. Timely diagnosis and treatment may be lifesaving. We report two cases demonstrating different and rare forms of severe brain involvement in adult patients with HLH: acute necrotizing encephalopathy, and diffuse hemorrhagic disease due to disseminated intravascular coagulation. Severe HLH with brain involvement in adults is rare. HLH with cerebral involvement should be considered in patients presenting with severe systemic inflammatory response syndrome (SIRS) but negative cultures and unusual or unexpectedly severe clinical and/or radiologic signs of cerebral dysfunction. Similar brain injury may occur in patients with cytokine storm syndrome due to COVID-19.BackgroundHemophagocytic lymphohistiocytosis (HLH) presents with fevers, rash, organomegaly, cytopenia, and increased triglycerides and ferritin (Ramos-Casals et al., 2014) [1]. Neurologic abnormalities are reported in about one-third of patients (Cai et al., 2017), including a few cases of acute necrotizing encephalopathy (ANE) (Xiujuan et al., 2015). Coagulation abnormalities are frequent in HLH patients (Valade et al., 2015).ObjectiveTo raise awareness about the importance of early diagnosis and treatment of HLH with neurological involvement to prevent serious complications and demise.     

产后失血性休克致DIC的抢救与护理1例分析

目前由产后出血引起的失血性休克在我国孕产妇死亡原因中居首位，若短时间内不能迅速止血纠正休克，会迅速发展为弥漫性血管内凝血（DIC）。DIC是在原发病基础上，促凝因素导致机体微血管内广泛地生成微血栓，消耗了大量凝血因子和血小板，并伴以继发性纤溶为特征的获得性血栓--出血综合征。DIC本身并不是一个独立的疾病，而是很多疾病发病的一个中间环节和病理过程。它的主要临床症状可归纳为出血、微循环障碍、多器官功能障碍和贫血。产后一旦发生DIC，将严重威胁产妇的生命安全。但是如果能及早发现，及时治疗，尚能挽救患者的生命。因此，当发生产后大出血时要时刻警惕DIC的发生，做到早发现，早诊断，早治疗，为抢救患者生命提供有利时机。在2013年12月21日，本院成功抢救了1例因宫缩乏力致产后失血性休克进而发生弥漫性血管内凝血的产妇，并报道如下。     

Label retaining cells in cancer – The dormant root of evil?

The phenomenon of cellular dormancy has been observed in normal adult stem cells in many different tissues such as the skin, the intestine and the hematopoietic system. These dormant cells have been proposed to be important for life-long self-renewal and for the generation of the different cellular lineages. As tumor cells can share properties with normal stem cells, dormant cells might also exist within a tumor. The term tumor dormancy has evolved from the clinical observation in cancer patients that relapse can occur years to decades after apparently successful treatment, suggesting that some cancer cells might resist chemotherapy and persist in a dormant state. Several studies investigating the role of cellular dormancy in normal stem cells and in cancer hint towards a complex network involving different pools of cells. These cells might interact with each other or even dynamically switch their phenotypes dependent upon so far unknown endogenous and microenvironmental stimuli. In this review, we will discuss the recent findings related to cellular dormancy in normal adult stem cells and in cancer. Furthermore, the clinical relevance of dormancy and its dynamic regulation in tumor cells will be highlighted.     

播散性浅表性光线性汗孔角化症21例临床病理分析

目的探讨播散性浅表性光线性汗孔角化症临床和组织病理学特点。方法回顾性分析本科1995年1月～2012年1月间断收治的21例播散性浅表性光线性汗孔角化症患者的临床和组织病理学资料。结果 21例患者中男女比例为1.1∶1(男11例,女10例)。20岁前发病者13例(61.91%),(20～50)岁7例(33.33%),>50岁1例(4.76%),平均发病年龄18.36岁。皮损位于曝光部位,累及面部者10例(47.62%),累及颈部者9例(42.86%),累及上肢者19例(90.48%),累及下肢者7例(33.33%),累及躯干者6例(28.57%)。其中4例有家族史(占19.05%)。3例合并有系统疾病。组织病理资料分析均可见典型的角化不全柱,其下方颗粒层减少或消失。结论播散性浅表性光线性汗孔角化症20岁前发病多见,男女发病率大致相当,皮损位于暴露部位。组织学上以表皮角质层内典型的角化不全柱伴其下方颗粒层减少或消失为特征。     

A case of disseminated blastomycosis in Vermont

Blastomyces dermatitides is increasingly found in Canada along the St. Lawrence River. However, there are only rare reports of this disease in New England. We describe a case of disseminated blastomycosis in a patient from northern Vermont who acquired the fungus while gardening.     

Superior mesenteric artery syndrome and disseminated tuberculosis: “Double troubleˮ

Superior Mesenteric Artery (SMA) Syndrome is a rare condition characterized by compression of the third part of the duodenum between the aorta and superior mesenteric artery due to decreased angle between these two vessels due to loss of intervening pad of fat. Tuberculosis is one of the causes, and its association with it is rare. However, SMA syndrome with significant gastrointestinal symptoms in TB poses a greater challenge in management, particularly in dissemination. Strong clinical suspicion, timely diagnosis and appropriate antituberculosis therapy are the keys to successful management.