韦格纳肉芽肿病11例临床特征分析

目的韦格纳肉芽肿病(WG)是一病因不清的少见的系统性血管炎。通过分析病例系列以描述疾病的临床特征,治疗和病情随访。方法回顾性地收集11例来自1999—2005年我院风湿科住院WG患者,分析临床及实验室特征。结果所有WG患者都有上下呼吸道累及。肺、上呼吸道和肾是最常累及的器官。多数患者接受了静脉注射甲泼尼龙(MP)0．5g/d×3d,随后静脉注射环磷酰胺(CTX)750 mg/m~2,每月用1 g,共治疗6～8次,疗效好,长期随访病情稳定。结论WG是系统性血管炎,以上、下呼吸道及肾脏受累最多见；病理特点为坏死性肉芽肿和血管炎,联合免疫抑制治疗可取得较好的疗效。     

哮喘难以控制需警惕变应性肉芽肿性血管炎

变应性肉芽肿性血管炎（allergic granulomatous angiitis,AGA）是一种以哮喘、血和组织中嗜酸粒细胞增多、血管外坏死性肉芽肿为特征的系统性小血管炎”。1951年由Churg和Strauss首先描述,故又称Churg-Strauss综合征（Churg-StraussSyn-drome,CSS）”。本病临床少见,发病率约为2．4／百万,在哮喘病人中约64．4／百万。由于本病患者在出现嗜酸粒细胞增多和系统性血管炎症状前常有数年难治性哮喘（difficult-to-controlasthma）的病史,因而很容易误诊。本文结合我们最近诊治的一个病例,谈谈对CSS诊治的体会。     

系统性血管炎中抗内皮细胞抗体的检测及其临床意义分析

目的研究抗内皮细胞抗体（AECA）在系统性血管炎中阳性率,并分析其临床意义.方法固定的人脐静脉内皮细胞（HUVEC）为抗原,细胞酶联免疫吸附试验（ELISA）法检测129例系统性血管炎,57例系统性红斑狼疮（SLE）、25例类风湿关节炎（RA）等患者和85名正常对照者AECA阳性率并分析系统性血管炎组AECA与临床生化指标的相关性.结果系统性血管炎（58.9%）和SLE（46%）患者的AECA阳性率明显高于RA（4%）和正常人组（2.4%）（P＜0.01）.AECA在不同系统性血管炎中阳性率分别为：白塞病（BD）48%（28/59）、大动脉炎（TA）79%（22/28）、韦格纳肉芽肿病（WG）65%（13/20）、结节性多动脉炎（PAN）63%（5/8）、显微镜下多血管炎（MPA）44%（4/9）、变应性肉芽肿血管炎（CSS）80%（4/5）.AECA阳性的TA患者的血沉（ESR）平均水平明显高于AECA阴性组（P＜0.05）.PAN、WG、MPA、CSS患者中,AECA阳性者ESR水平（P＜0.05）和伯明翰系统性血管炎临床活动评分标准（BVAS）值（P＜0.01）明显高于AECA阴性者,BD患者中,AECA阳性病人具有皮肤针刺反应阳性率明显增多（P＜0.05）.随诊的11例系统性血管炎患者治疗前后AECA滴度和ESR的变化,差异有统计学意义（P＜0.05）.结论不同的系统性血管炎和SLE患者血清中均有较高的AECA阳性率,且此抗体与系统性血管炎的临床活动度明显相关,可作为观察病情活动的指标之一.     

Churg-Strauss综合征肺损害1例并文献复习

目的 加深对Churg-Strauss综合征又名变应性肉芽肿血管炎(CSS)的认识.方法 通过对1例CSS病例的诊断、治疗及相关文献进行研究学习,分析Churg-Strauss综合征的病因、临床症状、实验室检查、影像学表现、诊断、鉴别诊断及治疗.结果 CSS病因不清,临床表现无特异性,可累及多系统器官,最常见的临床表现是哮喘及血管炎引起的肺部表现,特征性的病理表现为外周血嗜酸粒细胞增多,目前治疗首选激素,预后较好.结论 Churg-Strauss综合征是一种少见的系统性血管炎,临床缺乏特异性表现,易被误诊,当患者表现为哮喘、外周血嗜酸性粒细胞增多及肉芽肿性血管炎时,应高度怀疑此病.     

老年变应性肉芽肿性血管炎1例诊治体会

变应性肉芽肿性血管炎,又称Churg-Strauss综合征(CSS).是一种以哮喘、血和组织中嗜酸粒细胞增多、血管外坏死性肉芽肿为特征的系统性小血管炎.该病临床少见,国外报道其发病率约2.2～6.8/百万~([1]),临床上易被漏诊.本文通过总结1例老年CSS患者的诊治体会,以期提高临床医师对该病的认识.     

变应性肉芽肿血管炎

变应性肉芽肿血管炎（CSS）临床病情以支气管哮喘（哮喘）为主,其归类属嗜酸粒细胞增多症范畴,又属中性粒细胞胞浆抗体（ANCA）相关性血管炎范畴,故类似的疾病和需鉴别的疾病较多而易于误诊,我科最近收治一例亦被误诊为哮喘达6年之久,为此临床医生认识此病甚为重要。     

孟鲁司特与变应性肉芽肿血管炎之间的相关性述评

孟鲁司特是1998年初问世的一种新型预防和治疗慢性支气管哮喘（哮喘）的药物，它是特异性阻断白三烯D4（lukotriene D4，LTD4）与半胱氨酰白三烯1受体结合的高亲和性拮抗剂（LRA）。其副作用一般来说都很轻微，但现在不断有一些哮喘患者在服用孟鲁司特后出现了变应性肉芽肿血管炎（Churg-Strauss syndrome，CSS）的表现，并进一步得到确定诊断。孟鲁司特的使用与CSS的发生究竟有无相关性，本文将就此问题综合发表文献进行述评，以期引起临床医生的重视。     

疑诊为肺癌1例变应性肉芽肿性血管炎报道

正变应性肉芽肿性血管炎(allergic angitis granulomatosis,AGA或Churg-Stuauss syndrome,CSS)是一种主要累及中、小动脉和静脉,以哮喘、血和组织中嗜酸性粒细胞增多、嗜酸细胞性坏死性血管炎伴有坏死性肉芽肿为特征的系统性血管炎~[1]。1951年由Churg和Strauss两位病理学家首先报道,故又称CSS。该病临床少见,国外报道发病率为2.2-6.8/     

老年患者嗜酸细胞肉芽肿性血管炎的临床特征

嗜酸细胞肉芽肿性血管炎（EGPA）是以外周血和受累脏器组织中嗜酸性粒细胞增高并伴有坏死性肉芽肿为特征的系统性血管炎,以呼吸系统受累（如哮喘）最为常见。影像学表现为肺部非固定性斑片状浸润影、磨玻璃影、实变、结节、胸腔积液等,也可出现肺间充质改变。该病在成年人中常见,无明显性别差异;年龄是预后不良的影响因素之一。临床研究分析发现老年患者有更高的患病率和死亡率,本文就老年患者EGPA的临床特征进行文献复习,并综述如下。     

CD146在血管炎患者外周血白细胞表达的意义初探

目的探讨血管炎患者外周血白细胞CD146表达与临床活动性间的关系。方法流式细胞术检测39例活动期系统性血管炎患者[显微镜下多血管炎（MPA）13例,韦格纳肉芽肿（WG）9例,变应性肉芽肿性血管炎（CSS）2例,大动脉炎（TA）9例,白塞病（BD）4例,结节性多动脉炎（PAN）2例]及24例系统性红斑狼疮（SLE）患者外周血白细胞CD146表达,其中18例（MPA5例,WG4例,CSS2例,SLE4例,PAN2例,TA1例）患者经糖皮质激素和环磷酰胺治疗后于病情好转时再次检测。结果（1）与健康者相比,血管炎患者活动期中性粒细胞、淋巴细胞CD146表达增多,尤以中性粒细胞最多,差异均有统计学意义（P〈0．05）。（2）中性粒细胞CD146表达与淋巴细胞、单核细胞CD146表达相关（r值分别为0．66、0．853,P=0．000）,与病程、年龄、血沉、C反应蛋白、抗中性粒细胞胞浆抗体（ANCA）、PR3-ANCA、MPO-ANCA、血肌酐、伯明翰血管炎活动指数（BVAS）、系统性红斑狼疮疾病活动指数（SLEDAI）等无明显相关（r值分别为-0．108、-0．059、-0．073、-0．103、0．012、-0．5、-0．232、0．001、-0．08、0．089,P〉0．5）。（3）18例患者经治疗后好转期中性粒细胞、淋巴细胞CD146表达多数呈逐渐减少的趋势（P〈0．05）。结论CD 146在血管炎患者活动期外周血白细胞尤其是中性粒细胞中表达明显升高,随着糖皮质激素和免疫抑制剂治疗病情好转后呈下降或转阴趋势,其在血管炎发病机制中的意义有待深入研究。     

The immunoneuroendocrine role of melatonin

Abstract: A tight, physiological link between the pineal gland and the immune system is emerging from a series of experimental studies. This link might reflect the evolutionary connection between self-recognition and reproduction. Pinealectomy or other experimental methods which inhibit melatonin synthesis and secretion induce a state of immunodepression which is counteracted by melatonin. In general, melatonin seems to have an immunoenhancing effect that is particularly apparent in immunodepressive states. The negative effect of acute stress or immunosuppressive pharmacological treatments on various immune parameters are counteracted by melatonin. It seems important to note that one of the main targets of melatonin is the thymus, i.e., the central organ of the immune system. The clinical use of melatonin as an immunotherapeutic agent seems promising in primary and secondary immunodeficiencies as well as in cancer immunotherapy. The immunoenhancing action of melatonin seems to be mediated by T-helper cell-derived opioid peptides as well as by lymphokines and, perhaps, by pituitary hormones. Melatonin-induced-immuno-opioids (MHO) and lymphokines imply the presence of specific binding sites or melatonin receptors on cells of the immune system. On the other hand, lymphokines such as -γ-interferon and interleukin-2 as well as thymic hormones can modulate the synthesis of melatonin in the pineal gland. The pineal gland might thus be viewed as the crux of a sophisticated immunoneuroendocrine network which functions as an unconscious, diffuse sensory organ.     

Changes in connexin43, the gap junction protein of astrocytes, during development of the rat pineal gland

Abstract: The abundance of gap junctions between rat pineal astrocytes formed by connexin43 (Cx43) was studied during development. Levels and distribution of Cx43 were measured by immunoblotting and indirect immunofluorescence, respectively. The amount of Cx43 in cells located within the gland was low until about the 7th postnatal day and increased to adult values between the 14th and 21st days postpartum. Although astrocytes, recognized by their vimentin immunoreactivity, were scarce before birth, they were abundant by the 7th postnatal day suggesting that the low levels of Cx43 found at this age corresponded to a low expression of this protein. Localization of the immunoreactivity to Cx43 and vimentin showed a close correlation, indicating that mature or immature pineal astrocytes form gap junctions made of Cx43. Since Cx43 levels attained their adult values at about the time the innervation and the functional state of the gland reached maturity (2–3 weeks after birth), it is proposed that astrocyte gap junctions are involved in the function of the adult rat pineal gland.     

Conservative management of perforated duodenal diverticulum： A case report and review of the literature

Duodenal diverticula are a relatively common condition. They are asymptomatic, unless they become complicated, with perforation being the rarest but most severe complication. Surgical treatment is the most frequently performed approach. We report the case of a patient with a perforated duodenal diverticulum, which was diagnosed early and treated conservatively with antibiotics and percutaneous drainage of secondary retroperitoneal abscesses. We suggest this method could be an acceptable option for the management of similar cases, provided that the patient is in good general condition and without septic signs.     

Response of rat pineal melatonin to calcium, magnesium, and lithium is circadian stage dependent

Abstract: Herein we documented the response of pineal melatonin production to electrolytes known to be effective on pineal function in view of a possible circadian stage dependence. We studied the release of melatonin by perifused rat pineal glands at 2 different circadian stages corresponding to the middle of the light and dark periods, i.e., respectively, 7 and 19 HALO (Hours After Light Onset, L:D = 12:12). The initial efflux rates were, as expected, much higher in the perifusates of glands removed from rats sacrificed during the dark phase than of those removed during the light phase. After 3 hr of perifusion, melatonin release reached similar levels which were found constant up to the 8th hr of perifusion, whatever the circadian stage. Perifusion of the glands with physiological concentrations for the rat of calcium (5.2 mmol/1) and magnesium (1.34 mmol/1) resulted in a stimulatory effect on the pineal glands removed from rats sacrificed in the middle of the dark period (19 HALO), whereas no effects were observed on the pineal glands removed from rats sacrificed during the light (7 HALO). Lithium (0.28 and 0.55 mmol/1) was ineffective on melatonin release in pineal glands removed 7 and 19 HALO. Our results show differences in the initial efflux rates of melatonin and in the response of perifused pineal glands to calcium and magnesium according to the circadian stage.     

Presence of immunoreactive growth hormone and prolactin in the ovine pineal gland

Abstract: The use of antisera raised against bovine growth hormone (GH) and ovine prolactin (PRL) enabled the detection of related immunoreactive (ir) sequences of proteins in ovine pineal tissue. The isolation of PRL-like ir-material was accomplished using a 0.25 M ammonium sulphate (pH 5.5) extraction followed by ethanol precipitation, whereas the resulting 2.0 M ammonium sulphate (pH 7.0) precipitate contained a GH-like immunoreactivity. Gel chromatography of the GH-like immunoreactivity (Sephadex G-100) indicated the presence of several GH-like fragments ranging in the M_r range of 7,000 to 55,000. Analyses of the PRL-like ir-material found in pineal tissue on HPLC using a TSK 545-DEAE column led to the resolution into a single peak of immunoreactivity. A single peak of activity was also observed following chromatofocusing and hydrophobic interaction chromatography of the ir-peak from the TSK 545-DEAE column. The PRL-like ir-material inhibited the binding of [¹²⁵I]ovine PRL-S14 to anti-ovine PRL antibodies without showing an affinity for binding to anti-rat PRL or anti-bovine GH antibodies. Scatchard analysis of the binding of pineal PRL-like ir-material and pituitary ovine PRL-S14 to liver membranes from day-20 pregnant rats revealed similar affinity constants (K_a of 4.7 ± 0.2 × 10⁹ M^-1). In addition, the replication of Nb 2 Node rat lymphoma cells was stimulated by pineal PRL-like ir-material, an effect known to be specific for lactogenic hormones. The pineal PRL-like immunoreactivity appeared on sodium dodecyl sulfate polyacrylamide gels as a single major band of M_r 24,000. The functional status of PRL-and GH-like ir-material in the ovine pineal remains to be determined, but evidence is presented that the overall protein synthesis rate of the rat pineal responded to circulating concentrations of PRL.     

Microbiology of human immunodeficiency virus anorectal disease

PURPOSE: Individuals who are seropositive for the human immunodeficiency virus are at high risk for opportunistic infection and anorectal disorders. Little prospective information is available regarding anorectal pathogens in these patients. METHODS: One hundred sixty-three HIV-seropositive patients presented to the colorectal clinic between 1989 and 1992. Forty-seven (29 percent) patients were thought to have an infectious process and were prospectively studied using a standardized multiculture protocol. RESULTS: Mean age was 33 (range, 19–59) years. All were male; high-risk behavior accounted for 87 percent of HIV transmissions. Presenting complaints included anorectal pain (79 percent), pus per anum (28 percent), and blood per anum (26 percent). Examination revealed perianal tenderness (60 percent), condyloma (38 percent), perianal ulcers (38 percent), and anal fissures (34 percent). Sixty-six sets of cultures were performed; 28 patients had one set, 15 had two sets, and 4 had three sets. Thirty-two of these 47 patients (68 percent) had positive cultures including herpes (50 percent), cytomegalovirus (25 percent),Neisseria gonorrhoeae (16 percent), chlamydia (16 percent), acidfast bacilli (2 percent), and others (9 percent). Six of 32 patients with positive cultures had more than one organism cultured. Sixteen (50 percent) patients with positive cultures were treated medically, 8 (25 percent) were treated surgically and 8 (25 percent) were treated with both modalities. Sixty-one procedures were performed on 17 patients for condylomata. Eighteen patients had 20 procedures for abscesses, 50 percent of whom had positive cultures for other than common bowel flora; all improved. Fourteen patients underwent 33 procedures for perianal fistulas.Mycobacterium fortuitum was cultured from one patient who required 13 procedures for abscesses and fistulas. Forty-five (96 percent) patients were followed for an average of 12.5 months ±2.9 SEM (range, 1–94 months). Symptoms were improved or resolved in 22 of 32 (69 percent) patients with positive cultures and in 11 of 13 (84 percent) with negative cultures. CONCLUSIONS: Specific pathogens may often be identified in human immunodeficiency virus-seropositive patients with anorectal disorders if aggressively sought. Although patients without specific pathogens identified may be expected to improve with planned empiric treatment, positive identification allows more directed therapy.