累及垂体和甲状腺的成人朗格汉斯细胞组织细胞增生症1例

朗格汉斯细胞组织细胞增生症（Langerhans cell histiocytosis,LCH）是以CD1a +/CD207 +髓样树突状细胞克隆增殖为特征的组织细胞性疾病。根据受累部位和范围的不同,LCH的临床表现及治疗差异均较大。本文对1例累及垂体和甲状腺的成人LCH进行临床特点分析,以探索...     

朗格汉斯细胞组织细胞增生症的诊治实例与体会

朗格汉斯细胞组织细胞增生症（Langerhans cell histiocylosis,LCH）是一组病因未明的组织细胞增生性疾病,是一罕见疾病。笔者回顾从事呼吸内科临床医疗教学工作的26年间,所在的科室仅收治4例。现结合其中3例的诊治情况谈一点粗浅的体会以飨读者。     

朗格汉斯细胞组织细胞增生症转化为慢性粒单核细胞白血病1例并文献复习

正朗格汉斯细胞组织细胞增生症(langerhans cell histiocytosis,LCH),原称组织细胞增生症,是一组原因未明的组织细胞增殖性疾病,组织上均为朗格汉斯细胞病理性增殖的结果。LCH的病因和发病机制尚未完全阐明,其发病率低,转化为慢性粒单核细胞白血病尤为罕见。我院收治1例LCH转化为慢性粒单核细胞白血病,现复习相关文献报告如下。1病例资料患者,男,62岁,2年半前因"皮肤瘙痒"就诊于皮肤科,给予皮炎平外用等对症治疗,效果不佳随     

下颌骨朗格汉斯细胞组织细胞增生症1例并文献复习

正朗格汉斯细胞组织细胞增生症(langerhans cell histiocytosis,LCH)是以大量朗格汉斯细胞增生、浸润和肉芽肿形成,伴有数量不等的中性粒细胞、嗜酸性粒细胞、淋巴细胞、浆细胞及多核巨细胞浸润,引起组织破坏,导致器官功能障碍为特征的一组肿瘤性疾病~([1])。LCH儿童多见,发病高峰在1~3岁,男女比例为(1.2~2):1,成人发病率很低~([2])。此病罕见,临床表现多样~([3]),缺乏特异性,极易误诊和漏诊。现收治1例LCH患者,报告如下并进行文     

成人朗格汉斯细胞组织细胞增生症的治疗进展

朗格汉斯细胞组织细胞增生症(LCH)是一种罕见的组织细胞肿瘤, 儿童发病率为(3～5)/100万, 成人发病率不足1/100万。LCH致死率低, 但可引起严重的器官功能障碍。目前尚无公认针对成人患者的治疗推荐。自2013年欧洲组织细胞协会发布了成人LCH的管理建议以来, 近年陆续报道了多项成人LCH队列研究。此外, 随着丝裂原活化蛋白激酶(MAPK)途径中的基因突变陆续被发现, 各种新型靶向药物也逐渐投入使用。本文就近10年成人LCH的治疗进展进行综述。     

以中枢性尿崩为首发表现的朗格汉斯细胞组织细胞增生症的临诊应对

报道3例以中枢性尿崩为首发表现的朗格汉斯细胞组织细胞增生症(LCH)患者,总结其临床表现、实验室检查、影像学检查、病理结果、诊断过程和治疗反应。3例患者早期均以中枢性尿崩症起病,垂体磁共振成像(MRI)均表现为垂体柄增粗,垂体后叶正常高信号消失。2例患者表现为孤立性下丘脑-垂体病变,1例表现为垂体和甲状腺多系统受累。病理结果显示典型的朗格汉斯细胞,免疫组织化学示S-100、CD1a、Langerin阳性。LCH临床表现呈现明显的异质性,容易误诊和漏诊。确诊依赖病理结果,孤立性下丘脑-垂体病变活检难度较大,建议积极筛查其他器官增加活检概率。LCH导致的神经垂体损害通常需要终生激素替代治疗。     

朗格汉斯与朗格汉斯细胞组织细胞增生症

朗格汉斯细胞组织细胞增生症(Langerhans' cell histiocytosis,LCH)是以组织细胞异常增殖和播散为特征的一种疾病.这种组织细胞与朗格汉斯(Paul Wilhelm Heinrich Langerhans)最早描述的树突状细胞几乎完全相同.典型表现为侵犯表皮、淋巴结、胃肠道和呼吸系统的黏膜上皮.在光镜下见到具有典型形态特征的朗格汉斯细胞,S-100蛋白染色阳性,可以拟诊该病.     

成人朗汉斯细胞组织细胞增生症

目的:探讨我国成人朗汉斯细胞组织细胞增生症(LCH)的临床分型、分级及治疗情况。方法:对我院近10年来收治的成人LCH9例和1995~2005年国内文献中报告的成人LCH286例作一综合分析。结果:成人LCH295例,男207例,女88例,男女之比为2.4∶1,年龄18~75岁,平均35.0岁。骨为最多受侵犯(81.4%),其次为淋巴结、肺和皮肤。按传统分型,骨嗜酸肉芽肿(EGB)最多见(75.9%),其次为单一器官型(13.9%)。按国际组织细胞协会分型,绝大多数为单系统型(96.3%),其中单系统单部位型63.4%,单系统多部位型32.9%。结论:成人LCH发病隐袭,常为慢性病程,多为侵犯骨骼的单系统疾病。原发性肺受累是成人LCH的又一特征,成人LCH总体预后良好。     

郎格罕氏组织细胞增生症20例临床分析

郎格罕组织细胞增生症（LCH）临床表现多样,可从单一组织器官、单部位的侵犯或损害发展为威胁生命的多组织器官、多部位的侵犯及多功能损害。现将我院收治的LCH患者20例的临床特点及治疗情况报告如下。     

小儿朗格汉斯细胞组织细胞增生症9例临床分析

目的提高小儿朗格汉斯细胞组织细胞增生症（LCH）的诊断与鉴别诊断水平。方法回顾性分析9例LCH患儿的临床资料,总结其症状、影像学表现及治疗。结果 9例患儿均以头部一过性软组织包块就诊,其中伴局部疼痛2例,突眼1例,咳嗽1例,乏力及低热3例,多尿1例;CT表现为局限性或多发性溶骨性骨质缺损,边界清晰,周围无破坏,伴有软组织肿块,MR示板障破坏累及颅骨内外板,伴软组织肿块,病变部位呈长T1长T2信号,增强后强化明显。9例经病理证实为LCH,术后3例辅以泼尼松＋长春新碱＋依托泊苷化疗6周,2例行局部放疗（36 GY/20 F）,随访3个月～10年,死亡1例,余8例预后良好,无复发。结论 LCH确诊需结合临床表现、影像学检查和病理活检;对以一过性头部软组织包块为首诊的小儿患者,要考虑到LCH的可能。     

A Call to Action to Enhance Filovirus Disease Outbreak Preparedness and Response

The frequency and magnitude of recognized and declared filovirus-disease outbreaks have increased in recent years, while pathogenic filoviruses are potentially ubiquitous throughout sub-Saharan Africa. Meanwhile, the efficiency and effectiveness of filovirus-disease outbreak preparedness and response efforts are currently limited by inherent challenges and persistent shortcomings. This paper delineates some of these challenges and shortcomings and provides a proposal for enhancing future filovirus-disease outbreak preparedness and response. The proposal serves as a call for prompt action by the organizations that comprise filovirus-disease outbreak response teams, namely, Ministries of Health of outbreak-prone countries, the World Health Organization, Médecins Sans Frontières, the Centers for Disease Control and Prevention—Atlanta, and others.     

Interplay between interferon-mediated innate immunity and porcine reproductive and respiratory syndrome virus

Innate immunity is the first line of defense against viral infection, and in turn, viruses have evolved to evade host immune surveillance. As a result, viruses may persist in host and develop chronic infections. Type I interferons (IFN-α/β) are among the most potent antiviral cytokines triggered by viral infections. Porcine reproductive and respiratory syndrome (PRRS) is a disease of pigs that is characterized by negligible induction of type I IFNs and viral persistence for an extended period. For IFN production, RIG-I/MDA5 and JAK-STAT pathways are two major signaling pathways, and recent studies indicate that PRRS virus is armed to modulate type I IFN responses during infection. This review describes the viral strategies for modulation of type I IFN responses. At least three non-structural proteins (Nsp1, Nsp2, and Nsp11) and a structural protein (N nucleocapsid protein) have been identified and characterized to play roles in the IFN suppression and NF-κB pathways. Nsp's are early proteins while N is a late protein, suggesting that additional signaling pathways may be involved in addition to the IFN pathway. The understanding of molecular bases for virus-mediated modulation of host innate immune signaling will help us design new generation vaccines and control PRRS.     

Control of intraabdominal hemorrhage and shock: a comparison of fluid resuscitation, MAST, and balloon occlusion

Our study examined the efficacy of four treatment modalities in controlling hemorrhage and achieving hemodynamic stabilization in hemorrhagic shock: intravenous fluid replacement (IV); military antishock trousers used concomitantly with fluids (MAST); balloon occlusion at the level of the diaphragm with concomitant fluid replacement (balloon); and a combination of MAST inflation, balloon occlusion, and fluid resuscitation (MAST and balloon). Twenty-eight mongrel dogs were anesthetized, and the spleen was exposed and completely crushed. The abdomen was closed, and treatment was initiated and continued for four hours or until the dog died. For all conditions the hematocrit dropped during the course of the experiment; balloon occlusion was effective at slowing this drop (P less than .0001), but MAST had no statistically significant effect. Animals with balloons bled more slowly into the abdominal cavity than did animals in the other two groups (P less than .0001). MAST also were effective at slowing the bleeding (P less than .05). Of the balloon and the MAST and balloon dogs, all except one survived the entire four hours; this difference between balloon and nonballoon dogs is significant (P = .002). MAST did not have a statistically significant effect on survival. Perfusion pressure (PP) declined during the course of the experiment, and the balloon was effective at slowing this decline (P less than .0001); none of the other comparisons was statistically significant.     

Disease Pandemics and Major Epidemics Arising From New Encounters between Indigenous Viruses and Introduced Crops

Virus disease pandemics and epidemics that occur in the world’s staple food crops pose a major threat to global food security, especially in developing countries with tropical or subtropical climates. Moreover, this threat is escalating rapidly due to increasing difficulties in controlling virus diseases as climate change accelerates and the need to feed the burgeoning global population escalates. One of the main causes of these pandemics and epidemics is the introduction to a new continent of food crops domesticated elsewhere, and their subsequent invasion by damaging virus diseases they never encountered before. This review focusses on providing historical and up-to-date information about pandemics and major epidemics initiated by spillover of indigenous viruses from infected alternative hosts into introduced crops. This spillover requires new encounters at the managed and natural vegetation interface. The principal virus disease pandemic examples described are two (cassava mosaic, cassava brown streak) that threaten food security in sub-Saharan Africa (SSA), and one (tomato yellow leaf curl) doing so globally. A further example describes a virus disease pandemic threatening a major plantation crop producing a vital food export for West Africa (cacao swollen shoot). Also described are two examples of major virus disease epidemics that threaten SSA’s food security (rice yellow mottle, groundnut rosette). In addition, brief accounts are provided of two major maize virus disease epidemics (maize streak in SSA, maize rough dwarf in Mediterranean and Middle Eastern regions), a major rice disease epidemic (rice hoja blanca in the Americas), and damaging tomato tospovirus and begomovirus disease epidemics of tomato that impair food security in different world regions. For each pandemic or major epidemic, the factors involved in driving its initial emergence, and its subsequent increase in importance and geographical distribution, are explained. Finally, clarification is provided over what needs to be done globally to achieve effective management of severe virus disease pandemics and epidemics initiated by spillover events.     

Routine diagnosis of intestinal tuberculosis and Crohn’s disease in Southern India

AIM:To investigate whether routinely measured clinical variables could aid in differentiating intestinal tuberculosis(ITB)from Crohn’s disease(CD).METHODS:ITB and CD patients were prospectively included at four South Indian medical centres from October 2009 to July 2012.Routine investigations included case history,physical examination,blood biochemistry,ileocolonoscopy and histopathological examination of biopsies.Patients were followed-up after 2 and 6 mo of treatment.The diagnosis of ITB or CD was re-evaluated after 2 mo of antituberculous chemotherapy or immune suppressive therapy respectively,based on improvement in signs,symptoms and laboratory variables.This study was considered to be an exploratory analysis.Clinical,endoscopic and histopathological features recorded at the time of inclusion were subject to univariate analyses.Disease variables with sufficient number of recordings and P<0.05 were entered into logistic regression models,adjusted for known confounders.Finally,we calculated the odds ratios with respective confidence intervals for variables associated with either ITB or CD.RESULTS:This study included 38 ITB and 37 CD patients.Overall,ITB patients had the lowest body mass index(19.6 vs 22.7,P=0.01)and more commonly reported weight loss(73%vs 38%,P<0.01),watery diarrhoea(64%vs 33%,P=0.01)and rural domicile(58%vs 35%,P<0.05).Endoscopy typically showed mucosal nodularity(17/31 vs 2/37,P<0.01)and histopathology more frequently showed granulomas(10/30vs 2/35,P<0.01).The CD patients more frequently reported malaise(87%vs 64%,P=0.03),nausea(84%vs 56%,P=0.01),pain in the right lower abdominal quadrant on examination(90%vs 54%,P<0.01)and urban domicile(65%vs 42%,P<0.05).In CD,endoscopy typically showed involvement of multiple intestinal segments(27/37 vs 9/31,P<0.01).Using logistic regression analysis we found weight loss and nodularity of the mucosa were independently associated with ITB,with adjusted odds ratios of 8.6(95%CI:2.1-35.6)and 18.9(95%CI:3.5-102.8)respectively.Right lower abdominal quadrant pain on examination and involvement of≥3 intestinal segments were independently associated with CD with adjusted odds ratios of 10.1(95%CI:2.0-51.3)and 5.9(95%CI:1.7-20.6),respectively.CONCLUSION:Weight loss and mucosal nodularity were associated with ITB.Abdominal pain and excessive intestinal involvement were associated with CD.ITB and CD were equally common.     

Second and third line treatment options for Helicobacter pylori eradication

Helicobacter pylori is a highly successful bacterium with a high global prevalence and the infection carries significant disease burden. It is also becoming increasingly difficult to eradicate and the main reason for this is growing primary antibiotic resistance rates in a world where antibiotics are frequently prescribed and readily available. Despite knowing much more about the bacterium since its discovery, such as its genomic makeup and pathogenesis, we have seen declining treatment success. Therefore, clinicians today must be prepared to face one, two or even multiple treatment failures, and should be equipped with sufficient knowledge to decide on the appropriate salvage therapy when this happens. This article discusses the factors contributing to treatment failure and reviews the second and thirdline treatment strategies that have been investigated. Established empiric second line treatment options include both bismuth based quadruple therapy and levofloxacin based triple therapy. Antibiotic testing is recommended prior to initiating third line treatment. In the event that antibiotic susceptibility testing is unavailable, third line treatment options include rifabutin, rifaximin and sitafloxacin based therapies.     

Barrett's Esophagus: Where Do We Stand?

Barrett''s esophagus (BE) is a precursor for esophageal adenocarcinoma, which has an increased incidence rate over the last few decades. Its importance stems from the poor five-year survival of esophageal adenocarcinoma and current data that suggest a survival benefit when surveillance programs are implemented. In this review, we will cover the pathophysiology and natural history of BE and the different endoscopic findings. The prevalence of BE in different geographic areas and the incidence of high-grade dysplasia and adenocarcinoma in this patient population is reviewed. Recent recommendation for screening and surveillance of BE has been covered in this review as well as the efficacy of nonconventional imaging modalities and endoscopic ablation therapies.