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Maladie de Still de l'adulte 成人斯蒂尔氏病
Pub Date : 2006-01-01 DOI: 10.1016/S1634-6939(06)75427-8
J. Pouchot, P. Vinceneux
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引用次数: 0
[Lymphoma genesis in the context of HIV infection]. [HIV感染背景下的淋巴瘤发生]。
Pub Date : 2003-12-01
Caroline Besson, Martine Raphaël

The incidence of lymphomas is high among HIV infected patients. These lymphomas are non-Hodgkin's lymphoma (NHL) in 70% of cases and Hodgkin's disease (HD) in 30% of cases. Their localization is often extra-nodal with early dissemination. B-cell high grade NHL predominates. The most frequent histological types are diffuse large B-cell lymphoma (30 to 40%) and Burkitt's lymphoma (40 to 50%). Other histological types are low-grade B-cell lymphoma, polymorphic B cell lymphoma and primary effusion lymphoma. Three main factors are predominant in HIV-related lymphomagenesis: cellular immunodeficiency, oncogene viruses (Epstein-Barr and HHV8) and molecular lesions. HIV-related cellular immunodeficiency leads to the increase of EBV infected B-cells and to the diminution of antitumor immunity. Clonal EBV genome is found in lymphoma cells in 30 to 70% of cases of HIV-related NHL. It expresses oncogenic proteins including LMP-1 which behaves like an activated CD40. It induces the expression of intra-cellular genes which stimulate cell growth and inhibit apoptosis. Cytogenetic and molecular lesions are not specific to HIV-related NHL or to histological subtypes. A better knowledge of these mechanisms should lead to the development of specific targeted treatments (antiviral, cytotoxic anti-EBV lymphocytes, cell cycle regulators).

在艾滋病毒感染者中,淋巴瘤的发病率很高。这些淋巴瘤为70%的非霍奇金淋巴瘤(NHL)和30%的霍奇金病(HD)。它们的定位通常是淋巴结外的早期传播。以b细胞高分级NHL为主。最常见的组织学类型是弥漫性大b细胞淋巴瘤(30 - 40%)和伯基特淋巴瘤(40 - 50%)。其他组织学类型为低级别B细胞淋巴瘤、多形性B细胞淋巴瘤和原发性积液性淋巴瘤。hiv相关淋巴瘤发生主要有三个因素:细胞免疫缺陷、癌基因病毒(Epstein-Barr和HHV8)和分子病变。hiv相关的细胞免疫缺陷导致EBV感染的b细胞增加和抗肿瘤免疫降低。在30%至70%的hiv相关NHL病例中,在淋巴瘤细胞中发现EBV克隆基因组。它表达包括LMP-1在内的致癌蛋白,其行为类似于活化的CD40。它诱导细胞内基因的表达,刺激细胞生长,抑制细胞凋亡。细胞遗传学和分子病变不是hiv相关NHL或组织学亚型所特有的。更好地了解这些机制将导致开发特异性靶向治疗(抗病毒、细胞毒性抗ebv淋巴细胞、细胞周期调节剂)。
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引用次数: 0
[Necrolytic migratory erythema revealing glucagonoma without diabetes]. [坏死性迁移性红斑显示胰高血糖素瘤,无糖尿病]。
Pub Date : 2003-12-01
Cécile Marty, Antoine Bennet, Paule Bayle, Marie Danjoux, Thibaud Lalande, Marie-Claude Marguery, Jacques Bazex

We report a case of glucagonoma syndrome, revealed by a necrolytic migratory erythema that had developed for four Years, associated with anorexia, severe weight loss, anemia, hypoprotidemia, and hypoaminoacidemia. The fasting blood glucose level tended paradoxically to be low (0.6 g/l). Elevated plasma glucagon levels confirmed our diagnosis. The absence of diabetes was explained by an independent insulin secretion derived from this composite pancreatic tumor, authenticated by the histological analysis and the proinsulin level. This level was similar to those typically observed in insulinomas. Six Months after a complete surgical exeresis, symptoms disappeared and biological results returned to normal values.

我们报告一例胰高血糖素综合征,表现为坏死性迁移性红斑,已发展4年,伴有厌食症、严重体重减轻、贫血、低蛋白血症和低胺酸血症。空腹血糖水平反而很低(0.6 g/l)。血浆胰高血糖素水平升高证实了我们的诊断。没有糖尿病的原因是该复合胰腺肿瘤产生了独立的胰岛素分泌,经组织学分析和胰岛素原水平证实。该水平与胰岛素瘤中典型观察到的水平相似。手术结束6个月后,症状消失,生物学指标恢复正常。
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引用次数: 0
[Bilateral primary non-Hodgkin's lymphomas of the adrenals]. [双侧原发性肾上腺非霍奇金淋巴瘤]。
Pub Date : 2003-12-01
Lamia Rezgui-Marhoul, Kamel Bouslama, Mohamed Bouchriha, Ali Harmel, Sabah Mzabi-Rgaya, Lotfi Hendaoui, Mohamed Ben Dridi

Bilateral primary non-Hodgkin's lymphomas of the adrenals are rare: only 32 cases have been reported to date in the literature. We report a new case and recall the contribution of radiography to diagnosis.

双侧原发性肾上腺非霍奇金淋巴瘤是罕见的:只有32例已报道的文献。我们报告一个新病例,并回顾放射摄影对诊断的贡献。
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引用次数: 0
[A survey of pain prevalence at Albert-Chenevier Hospital. Comparison of patient, caregivers and physician assessments]. 阿尔伯特-舍内维尔医院疼痛患病率调查。患者、护理人员和医生评估的比较]。
Pub Date : 2003-12-01
Valérie Jeanfaivre, Catherine Diviné, Marie-Noëlle Akalin, Françoise Anacharsis, Annie Montagne, Jean-Claude Le Parco

The purpose of this study is to estimate the prevalence of pain in hospitalized patients and to compare pain assessments performed by patients, caregivers and physicians. This cross-sectional study was based on evaluating pain with three questionnaires. Two structured questionnaires explored the patient's and physician's points of view about hospital pain management. Caregivers used the validated Doloplus-2 scale. The three assessments were considered consistent when all three indicated the presence or absence of pain. All inpatients (447) were included. The prevalence of pain was 57% when assessment was performed by caregivers and 53% when it was performed by physicians. Among patients included in the prevalence study, 366 could express themselves and agreed to answer the self-assessment questionnaire (82%): 66% of these patients reported pain. Patients and physicians often reported leg pain. Assessment of pain produced a higher level of pain intensity when performed by patients than when by physicians. Furthermore, 88% of patients reporting pain received pain relief treatment: nevertheless patients felt that their pain was satisfactorily managed in only half of their locations, and they had a less positive appreciation of their level of pain management than physicians did. Our study showed that many psychiatric patients declared they suffered pain. Full consistency between the three assessments was noted for half the cases. One out of ten patients experiencing pain was not identified.

本研究的目的是估计住院患者疼痛的患病率,并比较患者、护理人员和医生进行的疼痛评估。本横断面研究以三份问卷评估疼痛为基础。两份结构化问卷探讨了患者和医生对医院疼痛管理的看法。护理人员使用经过验证的Doloplus-2量表。当所有三个评估都表明存在或不存在疼痛时,这三个评估被认为是一致的。纳入所有住院患者(447例)。当由护理人员进行评估时,疼痛的患病率为57%,当由医生进行评估时,疼痛的患病率为53%。在纳入患病率研究的患者中,有366名患者能够表达自己并同意回答自我评估问卷(82%),其中66%的患者报告疼痛。病人和医生经常报告腿部疼痛。由患者进行的疼痛评估产生的疼痛强度水平高于由医生进行的疼痛评估。此外,88%报告疼痛的患者接受了缓解疼痛的治疗:然而,患者认为他们的疼痛仅在一半的部位得到了满意的控制,他们对疼痛管理水平的评价不如医生。我们的研究表明,许多精神病患者声称他们感到疼痛。在一半的病例中,三种评估结果完全一致。十分之一的疼痛患者没有被识别出来。
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引用次数: 0
[Investigation of the complement system in clinical practice]. 临床实践中补体系统的研究
Pub Date : 2003-12-01
Véronique Frémeaux-Bacchi, Marie-Agnès Dragon-Durey, Jacques Blouin, Luc Mouthon, Wolf Herman Fridman

The complement system plays an important role in defence of the host against infection and in the clearance of immune complexes. Defects in complement proteins are often associated with infections or auto/immune complex diseases. Investigation of complement is useful for diagnosis and following of auto-immune diseases. The aim of this Article is to provide an overview of important applications of complement in medicine, emphasizing the role of complement in pathogenesis and the usefulness of measurements of complement proteins in diagnosis and assessment of the evolution of disease states. Emphasis has been placed on practical applications and understanding basic mechanisms of disease. The best screen for complement deficiencies or significant activation is the CH50, which measures total classical pathway activity and the measurement of C3 and C4. The absence or decrease of multiple components is usually due to consumption of complement. Complete lack of CH50 associated with normal C3 antigen is a strong indication for complement deficiency and should be followed up with further tests to determine which component is missing.

补体系统在防御宿主感染和清除免疫复合物方面起着重要作用。补体蛋白缺陷常与感染或自身/免疫复合物疾病有关。补体的研究对自身免疫性疾病的诊断和随访有重要意义。本文的目的是概述补体在医学中的重要应用,强调补体在发病机制中的作用以及补体蛋白测量在诊断和评估疾病状态演变中的有用性。重点放在实际应用和了解疾病的基本机制。补体缺乏或显著激活的最佳筛选是CH50,它测量总的经典途径活性以及C3和C4的测量。多种成分的缺乏或减少通常是由于补体的消耗。完全缺乏与正常C3抗原相关的CH50是补体缺乏的强烈指示,应进行进一步的检查以确定缺失的是哪一种成分。
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引用次数: 0
[Meteorologic conditions and esophageal varices rupture]. [气象条件与食管静脉曲张破裂]。
Pub Date : 2003-12-01
Nabil Tahri, Ali Amouri, Habib Fekih, Faïçal El-Euch, Mohamed Salah Krichen

Background: While meteorologic conditions are thought to be related to the incidence of certain cardiac and cerebrovascular events, very little information is available concerning the onset of variceal bleeding. We undertook this study to determine whether there is any correlation between esophageal variceal hemorrhage and several meteorologic and astronomic parameters.

Methods: Sixty-seven episodes of bleeding from ruptured esophageal varices observed in 52 patients from southern Tunisia during a 7-Year period were studied retrospectively. All patients underwent endoscopic examination within 24 hours of admission. The date of the event (variceal rupture) was considered--if no stigmates of active bleeding were found at endoscopy--as the day of occurrence of the hematemesis and the day preceding melena. Meteorologic readings (11 parameters) plus moon-cycle, circadian and seasonal distribution of the 67 days of variceal bleeding in comparison with a control period of days randomly selected among 134 days without rupture.

Results: No relationship was found between variceal bleeding and mean atmospheric pressure, daily hours of sunshine, nebulosity, direction and velocity of wind and mean humidity. On the contrary, a significant correlation was observed with the mean temperature (18 degrees C vs 21.16 degrees C; p=0003), rainfall (p<0.01) and stormy weather (p=0.008), the latter being the only parameter retained as an independent factor at multivariate analysis: OR=13.37 (95% CI=1.5-118.5). Furthermore, a negative correlation was found with full moon but only at univariate analysis (p=0.04). The seasonal distribution of variceal bleeding episodes showed the highest percentage during winter with a significant variation at multivariate analysis: OR=3.2 (95% IC=1.6-6.54). Occurrence of variceal bleeding also showed a circadian variation with the higher prevalence night between 6 and 0 PM (p<0.001).

背景:虽然气象条件被认为与某些心脑血管事件的发生有关,但关于静脉曲张出血的发生的信息很少。我们进行这项研究是为了确定食道静脉曲张出血与一些气象和天文参数之间是否存在相关性。方法:回顾性分析突尼斯南部地区52例食管静脉曲张破裂致出血的67例。所有患者均在入院24小时内接受内镜检查。如果内窥镜检查未发现活动性出血,则考虑事件(静脉曲张破裂)发生的日期为呕血发生的当天和黑黑的前一天。将67天静脉曲张出血的气象读数(11个参数)加上月周期、昼夜节律和季节分布与在134天无破裂中随机选择的对照期进行比较。结果:静脉曲张出血与平均气压、日日照时数、浊度、风向、风速、平均湿度无明显关系。相反,与平均温度呈显著相关(18℃vs 21.16℃;P =0003),降雨量(P =0003)
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引用次数: 0
[Chronic fever revealing an urachal cyst]. 慢性发热,显露尿管囊肿。
Pub Date : 2003-12-01
Thierry Weitten, Maher Ben Abdelgheni, Serge Rohr, Fazel Boujan, Jean-Frédéric Blicklé, Emmanuel Andrès

In the adult, the urachus remains as a non-specific fibrous formation extended from the bladder dome to the Retzius space. This urachal remnant is commonly asymptomatic or may be revealed by a cyst. This later may also be asymptomatic or lead to local inflammation or inflammatory pseudo-tumor. We report an original observation of chronic fever revealing an urachal cyst in a 21-Year-old male.

在成人中,尿管仍然是一种非特异性纤维结构,从膀胱穹窿延伸到Retzius间隙。尿管残余通常无症状或可由囊肿显露。后来也可能无症状或导致局部炎症或炎性假性肿瘤。我们报告一个最初的观察慢性发烧显示尿管囊肿在一个21岁的男性。
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引用次数: 0
Inflammatory pseudotumor of lymph nodes. 淋巴结炎性假瘤。
Pub Date : 2003-12-01
Marco Trevenzoli, Anna Maria Cattelan, Massimiliano Lanzafame, Lolita Sasset, Ercole Concia, Sandro Vento

A case of inflammatory pseudotumor in a 22-Year-old man is described. Symptoms and signs showed high fever and prominent cervical lymphadenopathy; laboratory findings were consistent with an acute inflammatory process. Diagnosis was reached through a lymph node biopsy which allowed a definitive diagnosis and ruled out several mimicking pathological processes such as malignant lymphoma, infectious diseases and autoimmune disorders. Inflammatory pseudotumor should be included in the differential diagnosis of patients with persistent fever and lymph node enlargement.

一例炎性假瘤在一个22岁的男子被描述。症状体征为高热、颈部淋巴结肿大;实验室检查结果符合急性炎症过程。诊断是通过淋巴结活检,允许明确的诊断,并排除了一些模拟病理过程,如恶性淋巴瘤,传染病和自身免疫性疾病。炎性假瘤应列入持续发热和淋巴结肿大患者的鉴别诊断。
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引用次数: 0
[Progress in the management of pyogenic cerebral abscesses in non-immunocompromised patients]. [非免疫功能低下患者化脓性脑脓肿治疗进展]。
Pub Date : 2003-12-01
Jérôme Tourret, Patrick Yeni

Advances in radiological techniques over the last two decades have enabled establishing well-defined clinical and radiological signs for the diagnosis of cerebral abscess. Conversely, no consensus has been reached on the appropriate therapeutic management. To date, there has been no published randomized therapeutic trial dedicated to cerebral abscesses, probably because of low incidence. Progress in bacterial epidemiology, imaging techniques allowing early diagnosis, and antibiotic therapy have however generated a shift towards more frequent use of shorter, exclusively medical, treatment. Based on a review of the literature, we present and discuss the treatments usually applied in specialized departments for non-immunocompromised patients.

在过去的二十年里,放射学技术的进步已经能够为脑脓肿的诊断建立明确的临床和放射学征象。相反,在适当的治疗管理上没有达成共识。到目前为止,还没有发表过针对脑脓肿的随机治疗试验,可能是因为发病率低。然而,在细菌流行病学、允许早期诊断的成像技术和抗生素治疗方面取得的进展,促使人们转向更频繁地使用较短时间的纯医学治疗。在回顾文献的基础上,我们提出并讨论了非免疫功能低下患者在专科通常采用的治疗方法。
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引用次数: 0
期刊
Annales de medecine interne
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