Kimura病与ALHF的对比观察——附6例报告并文献复习

与国外早期文献不同，近年来国外诸多文献指出：Ｋｉｍｕｒａ病与血管淋巴组织增生伴嗜酸性细胞增多（ＡＬＥＨ）实际为两种疾病，而不是同一种疾病。本文报道了３例典型Ｋｉｍｕｒａ病与３例ＡＬＥＨ病，结合支持两者为两种不同病变的观点。     

连接蛋白CRKL在正常血细胞和表达BCR／ABL细胞中的研究进展

ＣＲＫＬ是由２２号染色体ｑ１１的ｃｒｋｌ基因编码的含ＳＨ２、ＳＨ３结构域的３９ｋＤａ的连接蛋白。ＣＲＫＬ羧基端ＳＨ３结构域与Ｃ３Ｇ、ＳＯＳ、Ｐ１３－Ｋ、ＨＰＫｌ、ｃ－ＡＢＬ或ＢＣＲ／ＡＢＬ结合,ＣＲＫＬ氨基端ＳＨ２结构域与酷氨酸磷酸化的蛋白如ＣＢＬ、ＨＥＦ１、ＣＡＳ、ＳＴＡＴ５或ｐａｘｉｌｌｉｎ结合,在正常造血细胞和白血病中ＢＣＲ／ＡＢＬ的主要底物,与ＢＣＲ／ＡＢＬ和ｃ－ＡＢＬ形成连接复活复合物。     

3－磷酸甘油脱氢酶偶联法测定血清醛缩酶活性

探讨用３－磷酸甘油脱氢酶偶联法测定血清中醛缩酶（ＡＬＤ）活性的最佳反应条件。反应体系的终末浓度：ＴＥＡ１００ｍｍｏｌ／Ｌ，ＦＤＰ４ｍｍｏｌ／Ｌ，碘乙酸０．２２ｍｍｏｌ／Ｌ，ＮＡＤＨ０．２６ｍｍｏｌ／Ｌ，ＧＤＨ≥１０００Ｕ／Ｌ，ＴＰＩ≥１５００Ｕ／Ｌ，ＬＤ≥１０００Ｕ／Ｌ。最适ｐＨ在７．８～８．０，Ｋｍ为７．２×１０－３ｍｍｏｌ／Ｌ。批内ＣＶ：酶活性在７．３４Ｕ／Ｌ和６５．０６Ｕ／Ｌ时，ＣＶ分别为５．７％和１．４％；批间ＣＶ：酶活性在１１．８９Ｕ／Ｌ和１００．０８Ｕ／Ｌ时，ＣＶ分别为６．０％和３．３％，酶活性线性范围至少可达１８０Ｕ／Ｌ。健康人６０名，ＡＬＤ活性为４．５３±１．１７（ｘ±ｓ）Ｕ／Ｌ，男女两组均值无显著性差异。本文对ＴＥＡ－ＨＣｌ（ｐＨ８．０）、Ｔｒｉｓ－ＨＣｌ（ｐＨ８．０）和Ｃｏｌｉｄｉｎｅ－ＨＣｌ（ｐＨ７．５）三种缓冲液用于ＡＬＤ活性测定效果进行了评价，结果表明在ＴＥＡ缓冲液中所测ＡＬＤ活性最高；ＴＥＡ和Ｃｏｌｉｄｉｎｅ两种缓冲液的浓度在２５～１５０ｍｍｏｌ／Ｌ范围内对ＡＬＤ活性无影响，Ｔｒｉｓ缓冲液在５０ｍｍｏｌ／Ｌ时测得酶活性较高，缓冲液浓度过高或过低，酶活性均有所下降     

系统性红斑狼疮抗ENA抗体及抗核抗体的检测

系统性红斑狼疮（ＳＬＥ）是一种慢性多系统的疾病。伴有多种血清自身抗体的出现，我们采用免疫印迹法技术，对３０例ＳＬＥ病人和２０例正常人进行了抗ＥＮＡ抗体和ＡＮＡ的测定，测定结果，抗ｓｍ１０例为３３．３％，抗ｕｉＲＮＰ抗体８例为２６．６％两者混合型１１例为３６．６％，１例阴性。２０例正常人为阴性。     

血清酮体比值判断感染性多脏器功能失常综合征病情严重程度及预后的意义

目的：探讨血清酮体比值（ＡＫＢＲ）对判断感染性多脏器功能失常综合征病情轻重程度和预后的意义。方法：测定正常对照组、肝硬化组、急性感染组（又分Ａ、Ｂ、Ｃ３组）患者乙酰乙酸（ＡｃＡｃ）、β羟丁酸（３ＨＢ）及ＡＫＢＲ等指标水平。结果：肝硬化组ＡｃＡｃ〔（０．４２±０．１５）ｍｍｏｌ／Ｌ〕和３ＨＢ〔（０．８２±０．１８）ｍｍｏｌ／Ｌ〕明显低于正常对照组〔（０．９９±０．２０）ｍｍｏｌ／Ｌ和（１．４０±０．２５）ｍｍｏｌ／Ｌ〕，Ｐ均＜０．０１；急性感染Ａ、Ｂ、Ｃ３组的ＰａＯ２、乳酸、阴离子间隙具有显著性差异（Ｐ均＜０．０１）；ＡＫＢＲ与ＡＰＡＣＨＥⅢ评分呈明显负相关（ｒ＝－０．８１２，Ｐ＜０．００１）；血清酮体水平与ＡＰＡＣＨＥⅢ评分间无明显直线相关关系（ｒ＝０．１６３，Ｐ＞０．０５）；ＡＫＢＲ和ＰａＯ２与ＡＰＡＣＨＥⅢ评分间有明显相关性（ｒ＝０．７２２，Ｐ＜０．０１）。结论：ＡＫＢＲ能更早期、直接、准确地反映肝细胞线粒体的受损程度和能量代谢状态，可以作为评估感染性ＭＯＤＳ病情严重程度及预后的重要指标。     

第2节系 系统红斑狼疮性肺动脉高压

1　概　况11　定义　同源病是指同一致病因素使不同脏器或系统同时或相继受到损害。虽同源,但受损脏器各异,故其临床表现多样。12　概念　ＳＬＥ是一种病因不明的自身免疫缺陷性疾病,全身各脏器系统可同时或相继受累,肺动脉亦可受累,发生一系列病理生理变化,导致肺动脉高压(ｐｕｌｍｏｎａｒｙａｒｔｅｒｉａｌｈｙｐｅｒｔｅｎｓｉｏｎ,ＰＡＨ)。ＰＡＨ是ＳＬＥ的临床表现,其他如心包炎、心内膜炎、皮肤病变、胸膜炎等属同源同因,又各有其临床特征,故ＰＡＨ的发生与ＳＬＥ属同源病。121　发病率　近年文献报道的ＳＬＥ致ＰＡＨ发病率有增高…     

可溶性白细胞介素－2受体与系统性红斑狼疮疾病活动性的关系

本实验以双抗体夹心ＥＬＩｓＡ检测方法，发现ＳＬＥ病人血清ｓＩＬ－２Ｐ水平显著高于正常人，疾病活动期高于非活动期，且与ＳＬＥ疾病活动分数和血沉成正相关，而与Ｃ３、Ｃ４和ＣＨ５０成负相关，但与抗ｄｓ－ＤＮＡ抗体和ＡＮＡ无相关性。提示血清ｓＩＬ－２Ｒ是监测ＳＬＥ疾病活动性的敏感定量指标。     

可溶性白细胞介素—2受体与系统性红斑狼疮疾病活动性的关系

本实验以双抗体夹心ＥＬＩＳＡ检测方法，发现ＳＬＥ病人血清ＳＩＬ－２Ｒ水平显著高于正常人，疾病活动期高于非活动期，且与ＳＬＥ疾病活动分数和血沉成正相关，而与Ｃ３，Ｃ４和ＣＨ５０成负相关，但与抗ｄｓ－ＤＮＡ抗体和ＡＮＡ无相关性，提示血清ｓＩＬ－２Ｒ是监测ＳＬＥ疾病活动性的敏感定量指标。     

重组抗原在戊型肝炎病毒感染诊断中的应用

目的探讨重组抗原在戊型肝炎病毒（ＨＥＶ）感染诊断中的应用。方法用ＨＥＶ重组抗原建立酶免疫试验（ＥＩＡ）检测肝病患者和健康人群血清中抗戊型肝炎病毒抗体（抗－ＨＥＶ）。结果５５份用新加坡ＤＢＬ公司抗－ＨＥＶ诊断试剂盒检测为抗－ＨＥＶ阳性的肝炎患者血清中，５３份（９６．４％）用本方法检测也为阳性；４５份ＤＢＬ试剂盒检测为抗－ＨＥＶ阴性的血清中，本法检测有３份为抗－ＨＥＶ阳性。检测甲、乙、丙型肝炎患者血清各３０份，抗－ＨＥＶ阳性率分别为１３．３％（４／３０）、１３．３％（４／３０）和１０．０％（３／３０）。健康人抗－ＨＥＶ阳性率为５．０％（８／１６０）。重组抗原与合成肽抗原混合用于ＥＩＡ，可以检出两种抗原单独阳性的抗－ＨＥＶ。结论以重组抗原为基础的ＥＩＡ特异性强、灵敏度高、快速简便，是戊型肝炎血清学诊断及流行病学调查的一种可靠方法     

APACHEⅡ对高龄外科危重患者的预后评估

为了准确评估高龄外科危重患者的预后及治疗效果，作者对２０６例高龄危重患者进行了分析，采用ＡＰＡＣＨＥⅡ评分系统对疾病的危重程度进行评分，并对前后两个阶段病例进行分组对照，结果显示：ＡＰＡＣＨＥⅡ评分均值为２０．３分，死亡患者（２０例）的ＡＰＡＣＨＥⅡ评分（２６．３分）明显高于存活患者（１８６例）的ＡＰＡＣＨＥⅡ评分（１５．４分），差异有显著性。随着ＡＰＡＣＨＥⅡ评分增高，死亡率亦逐渐增高。通过ＡＰＡＣＨＥⅡ评分的动态观察，显示了对高龄危重患者治疗水平的变化，第一组患者经过两天治疗ＡＰＡＣＨＥⅡ评分下降２．７分，第二组下降６．６分，两组病种及最初进入ＩＣＵ病房的ＡＰＡＣＨＥⅡ评分无差异，在ＩＣＵ病房两组出现了不同的死亡率：１２．６％与６．８％，有显著性差异（ｐ＜０．０５）。作者认为：在高龄外科危重患者的临床工作中，ＡＰＡＣＨＥⅡ评分系统作为评估其预后指标有着重要作用。     

Comparison between Kimura’s disease and angiolymphoid hyperplasia with eosinophilia: case reports and literature review

Kimura’s disease (KD) is a rare chronic inflammatory or allergic disease. Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vascular neoplasm. Their relationship has always been debated. This article reports two rare cases, one of each disease. One patient was a 48-year-old female that presented with a mass on her right mandible. She also had oedema erythema and wheals on her lower limbs. She was diagnosed with Kimura’s disease complicated with chronic urticaria. The second patient was a 23-year-old female that presented with multiple nodules of unequal size on the scalp. She was diagnosed with angiolymphoid hyperplasia with eosinophilia. The first patient recovered after being treated with surgical resection, glucocorticosteroids, cyclophosphamide and radiotherapy. The second patient underwent the first stage of surgical excision and is currently being followed-up. Comparison of the clinical and histopathological features of these two cases supports the theory that KD and ALHE are two separate disease entities.     

13例血管淋巴样增生伴嗜酸粒细胞增多症的临床和病理分析

目的：探讨血管淋巴样增生伴嗜酸粒细胞增多症（ALHE）的临床表现、病理特点及诊治情况，提高对本病诊断和治疗的认识。方法：分析华山医院1999年1月-2004年12月13例确诊为ALHE患者的临床和病理资料、随访及其转归。结果：本病临床上表现为单发或多发红色至褐色的丘疹或结节，无淋巴结受累，通常局限在头面部和颈部。本病容易被误诊，但特征性的病理改变（包括血管增生、内皮细胞突入管腔以及混合性淋巴细胞、嗜酸粒细胞浸润）有助于确诊。手术切除对单发损害疗效好，而多发损害则治疗困难且客易复发。结论：组织病理学检查对于诊断ALHE是必需的。手术切除的疗效最好，但可以复发。     

性病患者淋病和非淋病性尿道炎调查

目的 了解成都地区性病患者淋球菌、沙眼衣原体和解脲支原体感染现状及其变化情况。方法 用聚合酶链反应杂交梳方法对８２０例性病患者标本进行三种病原体的检测,并进行统计分析。结果 淋球菌检出率为２１．３％,居首位,三种病原体感染情况均以男性高于女性,这与两年前调查结果略有不同,当时淋球菌感染率为女性高于男性。两次调查均以年轻者感染率较高。结论 所查三种病原菌是该地区引起性病的主要致病菌,其感染有年轻化真     

The clinical pattern of group C streptococcal pharyngitis in children

Group A streptococcal pharyngitis of is a common infectious disease with a well-recognized clinical pattern, as opposed to that attributed to group C assault. The aim of this study was to identify the clinical features of group C streptococcal pharyngitis in children. In total, 144 children participated in the study, consisting of three distinct categories. Group I patients consisted of children with group A streptococcal pharyngitis, group II patients included children with group C streptococcal pharyngitis,, while children with pharyngitis with no isolated causative agent constituted group III patients. With the exception of sore throat, which was three times more common in group I patients compared to group II patients, there were no other differences in symptoms and signs between these two groups. Similarly, no difference was found between group II patients and group III patients with respect to clinical features. Two or more Centor criteria were found in 77% of the children with group C streptococcal pharyngitis and in 89% of the children with group A streptococcal pharyngitis. It is concluded that the clinical features of group C streptococcal pharyngitis are similar to but milder than those of group A streptococcal pharyngitis.     

Foreign material as a cause of choledocholithiasis.

BACKGROUND AND STUDY AIMS: There are sporadic reports concerning foreign material as a cause of choledocholithiasis. Gallstones obtained endoscopically from the biliary duct were analyzed to determine their structure and the presence of foreign material. The possible correlation between foreign material in gallstones and clinical data was investigated. PATIENTS AND METHODS: Gallstones were examined qualitatively using a crystallographic analysis by X-ray powder diffraction, and the foreign material which was found was subjected to morphological analysis using polarization microscopy. Patients' clinical data were obtained from clinical records, physical examination and documentation. RESULTS: Out of a group of 54 patients, foreign material was found in CBD stones of six individuals (11.1%). Four of these patients were from the group who had undergone a previous cholecystectomy (29/54, 13.8%), and surgical suturing material was found in these four. On the other hand, in the group who had not undergone the operation, foreign material was found in two patients out of 25 (8%); in one case it was cellulose and in the other case the fibre was not identified. CONCLUSIONS: Foreign material can account for lithiasis in the biliary duct. In contrast to present reports in the literature, we have proved that this is possible even in patients without previous surgical intervention in the pancreatobiliary region.     

The humoral immune system in inflammatory bowel disease. II. Immunologlobulin levels

As there have been reports of differences in mean levels of serum immunoglobulins between patients with ulcerative colitis and Crohn's disease, serum IgG, IgA, and IgM were estimated in 158 patients with inflammatory bowel disease and the results correlated with the clinical features of the patients. Although a higher mean IgG level in ulcerative colitis compared to Crohn's disease was confirmed, no difference was found when the comparison was limited to patients with colonic Crohn's disease. Patients with either disease had higher mean IgM levels than controls, and the IgM levels were higher on treatment with corticosteroids and showed a tendency to rise in remission. IgG and IgM levels were also higher in both diseases if extraintestinal manifestations were present. It is concluded that if clinical features, particularly disease site, are taken into account, the overall immunoglobulin responses in these two diseases show no differences.     

Angio-immunoblastic Lymphadenopathy: Report of Ten Cases and Review of the Literature

Angio-immunoblastic lymphadenopathy (AIL) is a disease of unknownaetiology and pathogenesis. It has features of hyperimmunity,immune deficiency and can behave like a malignant lymphoma.An underlying abnormality of T-cell regulatory function hasbeen proposed. We report ten patients with AIL followed prospectivelyand review 200 cases from the literature. As well as showingthe typical features described in previous retrospective seriesnamely: constitutional symptoms, generalized lymphadenopathy,hepatosplenomegaly, skin eruptions, hypergammaglobulinaemiaand characteristic lymph node histology; four patients had oedemawith ascites or pleural effusions associated with hyponatraemiaand hypoalbuminaemia. We have also observed low free thyroxineindices in three patients with elevated TSH levels in two, butwithout clinical features of hypothyroidism. Seven of the patientsin this study were treated with prednisone and cyclophosphamide.Three achieved complete remission but only one patient has survivedlonger than two years. Failure to achieve complete remissionhas been associated with a 90 per cent mortality within oneyear of the onset of disease in the reports reviewed. Corticosteroidsalone have produced only a 40 per cent rate of remission. Themanagement of poor responders, non-responders and many relapsecases is unsatisfactory. More instensive chemotherapy is veryhazardous, increasing the already high risk of severe infections,and is often unsuccessful. Histological features recently reportedto represent co-existent immunoblastic lymphoma at diagnosiswere recognized in three of our patients, two of which wenton to develop definite lymphoma.     

Angio-immunoblastic lymphadenopathy: report of ten cases and review of the literature.

Angio-immunoblastic lymphadenopathy (AIL) is a disease of unknown aetiology and pathogenesis. It has features of hyperimmunity, immune deficiency and can behave like a malignant lymphoma. An underlying abnormality of T-cell regulatory function has been proposed. We report ten patients with AIL followed prospectively and review 200 cases from the literature. As well as showing the typical features described in previous retrospective series namely: constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, skin eruptions, hypergammaglobulinaemia and characteristic lymph node histology; four patients had oedema with ascites or pleural effusions associated with hyponatraemia and hypoalbuminaemia. We have also observed low free thyroxine indices in three patients with elevated TSH levels in two, but without clinical features of hypothyroidism. Seven of the patients in this study were treated with prednisone and cyclophosphamide. Three achieved complete remission but only one patient has survived longer than two years. Failure to achieve complete remission has been associated with a 90 per cent mortality within one year of the onset of disease in the reports reviewed. Corticosteroids alone have produced only a 40 per cent rate of remission. The management of poor responders, non-responders and many relapse cases in unsatisfactory. More instensive chemotherapy is very hazardous, increasing the already high risk of severe infections, and is often unsuccessful. Histological features recently reported to represent co-existent immunoblastic lymphoma at diagnosis were recognized in three of our patients, two of which went on to develop definite lymphoma.     

Coexisting Trigeminal Autonomic Cephalalgias and Hemicrania Continua

The trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) share many clinical characteristics including unilateral pain and ipsilateral autonomic features. We report a patient with a history of migraine without aura who developed cluster headache and HC simultaneously. The distinctive clinical features and differential response profiles to various treatments indicates that they are distinct disorders. We then review previous reports of patients with coexisting TACs and HC and discuss the relationship between these families of primary headache disorders.