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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。 相似文献
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朗格汉斯细胞组织细胞增生症(LCH)临床上少见,合并肝功能损伤则更少见。本例患者长期间断发热、咳嗽、下肢疼痛,曾因骨痛行右股骨刮骨手术。后因食欲不振、尿黄就诊,发现患者有肝内胆汁淤积、肝脾大、肺多发囊性病变、骨质破坏、尿崩症,经多学科协作会诊,复诊股骨组织病理,最终诊断为多系统LCH。LCH起病隐袭、临床表现差异大,易被误诊或漏诊。现通过分享多学科协作诊断伴肝内胆汁淤积的多系统LCH的经过,为提高临床医师对LCH的认识和诊治水平提供参考。 相似文献
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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。 相似文献
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盛海燕 《中华结核和呼吸杂志》2009,32(6)
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. 相似文献
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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。 相似文献
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R B Low C Schmidt R J Wilder W Massion S M Barrett P Downs 《Annals of emergency medicine》1985,14(6):540-546
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. 相似文献
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Paul Roddy 《Viruses》2014,6(10):3699-3718
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. 相似文献
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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. 相似文献
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Roger A. C. Jones 《Viruses》2020,12(12)
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. 相似文献
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Majid A. Al Madi 《Saudi Journal Of Gastroenterology》2009,15(1):2-10
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. 相似文献
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Philippe Colomban Michele Gironda Divine Vangu Burcu Krmz Bing Zhao Vincent Cochet 《Materials》2021,14(23)
Two masterpieces of the Qing Dynasty (1644–1912 CE), one in gilded brass (incense burner) decorated with cloisonné enamels stylistically attributed to the end of the Kangxi Emperor’s reign, the other in gold (ewer offered by Napoleon III to the Empress as a birthday present), decorated with both cloisonné and painted enamels bearing the mark of the Qianlong Emperor, were non-invasively studied by optical microscopy, Raman microspectroscopy and X-ray microfluorescence spectroscopy (point measurements and mapping) implemented on-site with mobile instruments. The elemental compositions of the metal substrates and enamels are compared. XRF point measurements and mappings support the identification of the coloring phases and elements obtained by Raman microspectroscopy. Attention was paid to the white (opacifier), blue, yellow, green, and red areas. The demonstration of arsenic-based phases (e.g., lead arsenate apatite) in the blue areas of the ewer, free of manganese, proves the use of cobalt imported from Europe. The high level of potassium confirms the use of smalt as the cobalt source. On the other hand, the significant manganese level indicates the use of Asian cobalt ores for the enamels of the incense burner. The very limited use of the lead pyrochlore pigment (European Naples yellow recipes) in the yellow and soft green cloisonné enamels of the Kangxi incense burner, as well as the use of traditional Chinese recipes for other colors (white, turquoise, dark green, red), reinforces the pioneering character of this object in technical terms at the 17th–18th century turn. The low level of lead in the cloisonné enamels of the incense burner may also be related to the use of European recipes. On the contrary, the Qianlong ewer displays all the enameling techniques imported from Europe to obtain a painted decoration of exceptional quality with the use of complex lead pyrochlore pigments, with or without addition of zinc, as well as cassiterite opacifier. 相似文献