Modified Damus–Kaye–Stansel procedure using aortic flap technique for systemic ventricular outflow tract obstruction in functionally univentricular heart

Damus–Kaye–Stansel procedure is a useful method to relieve the systemic ventricular outflow tract obstruction in functionally univentricular heart. Regurgitation of pulmonary valve and recurrence of systemic ventricular outflow obstruction are the major concerns at the late phase of this procedure. Modification of original Damus–Kaye–Stansel procedure that can prevent the use of prosthetic materials is evaluated. The modified Damus–Kaye–Stansel procedure using aortic flap technique was performed in eight patients with functionally univentricular heart. Patients’ ages ranged from 3 to 28 months (mean 14 months). Follow-up period was 37 months as a mean (9–71 months), and the follow-up was complete. There was no operative mortality and no late death. In addition, there was no recurrence of systemic ventricular outflow tract obstruction throughout the follow-up period. Regurgitation of the pulmonary valve estimated by echocardiography at the latest follow-up was none to trivial in seven patients and mild in one. The modified Damus–Kaye–Stansel procedure using aortic flap technique is a safe, useful and reproducible technique to solve systemic ventricular outflow tract obstruction in functionally univentricular heart, and it can be an alternative for original technique or the so-called double-barrel modification.     

国产氟康唑对小鼠隐球菌病的疗效

以５×１０３ｃｆｕ／鼠的新型隐球菌给小鼠脑室感染（ｉｃ）１ｈ后，分别口服氟康唑（Ｆｌｕ）５和１０ｍｇ／（ｋｇ·ｄ）或酮康唑（Ｋｅｔ）５０和１００ｍｇ／（ｋｇ·ｄ），ｂｉｄ×９ｄ。对ｉｃ小鼠连续观察４０天的存活率和平均存活时间，Ｆｕｌ分别为５０％～７０％和８～１５天，Ｋｅｔ是３０％～４０％和５～９天。由小鼠侧尾静脉注射１０６ｃｆｕ／鼠新型隐球菌３天后产生的全身感染，分别给小鼠口服Ｆｌｕ２．５～１０ｍｇ／（ｋｇ·ｄ）或Ｋｅｔ２５～１００ｍｇ／（ｋｇ·ｄ），ｂｉｄ×９ｄ。感染２４、２８和３２天的ＰＤ５０Ｆｌｕ是２．７、３．５和６．７ｍｇ／ｋｇ，Ｋｅｔ为２８．４、７０．４和８３ｍｇ／ｋｇ；Ｆｌｕ和Ｋｅｔ的平均存活时间分别是２３～３４天和２０～３１天。在上述两种实验模型中分离脑和肺做细菌培养，Ｆｌｕ均与对照和５０ｍｇ／（ｋｇ·ｄ）的Ｋｅｔ比较，对ｉｃ１０天及静脉注射感染１３天的小鼠，口服５和１０ｍｇ／（ｋｇ·ｄ）或２．５～１０ｍｇ／（ｋｇ·ｄ）Ｆｌｕ均显著抑制新型隐球菌生长。     

系统性红斑狼疮肺胸膜病变(附50例报告)

50例SLE患者有肺胸膜病变,其中女性47例(94％),男性3例(6％),小于40岁者41例(82％)。50例中表现为胸膜炎和(或)胸腔积液26例,狼疮肺炎15例,弥漫性肺间质纤维化5例,肺不张3例,肺纹理增多17例。经激素治疗随SLE症状改善,肺胸膜病变好转。     

神经精神性狼疮MRI检查的临床应用价值

目的：探讨神经精神性狼疮(NPSLE)MRI表现特点及其与临床的关系。方法：52例NPSLE患者在出现症状后1天～2个月内进行头部MR检查。另选取26例无神经系统症状的SLE患者和27例健康志愿者行MR检查并与NPSLE组比较。结果：NPSLE的MRI主要表现为斑点状和大片状脑炎样病变、出血、脑梗死和脑萎缩，额、顶叶和基底节区好发。表现为点状病灶的临床以精神障碍和头痛为主要表现，片状病灶以癫痫和偏瘫为主要表现。临床病情严重患者的片状病灶的发生率、病灶总面积较轻微组显著增高。片状的，边缘模糊的，T1WI等、略低信号，T2WI高信号的病灶变化迅速。结论：NPSLE的MRI检查有助于明确脑病性质及更准确的评估病情和预后，从而指导治疗和评价疗效。     

Phenotypic and genetic analyses of T-cell-mediated immunoregulation in patients with Type 1 diabetes.

AIMS: To investigate the contribution of regulatory T cells and co-stimulatory molecules in CD4(+) T cells to the development of Type 1 diabetes (T1D). METHODS: Twelve patients with T1D, nine patients with systemic lupus erythematosus (SLE), and 12 age-matched healthy control subjects participated. We analysed the proportions of CD25(+)CD4(+) T cells and natural killer T cells (NKT cells), and the expression levels of Foxp3, CTLA-4, CD28, ICOS, PD-1 and BTLA in peripheral blood mononuclear cells and purified CD4(+) T cells. RESULTS: There were no significant differences in the proportions of CD25(+) CD4(+) T cells or NKT cells among the three groups. PD-1 expression levels of peripheral CD4(+) T cells from T1D patients were significantly lower than those from healthy control subjects (P = 0.00066). In contrast, PD-1 expression levels were similar in SLE patients and healthy control subjects. The expression levels of Foxp3, CTLA-4, CD28, ICOS and BTLA were similar in the three groups. CONCLUSIONS: Decreased expression of the PD-1 gene in CD4(+) T cells may contribute to the development and/or maintenance of autoimmune T1D. As the population studied was small and heterogeneous, further studies are required to confirm the findings.     

Monospecific but not polyreactive human hybridoma rheumatoid factors exhibit preferential binding specificities for IgG3 and IgG4

Summary Among 38 human hybridoma-derived monoclonal rheumatoid factors (RFs) generated from patients with either rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE), two groups of RFs can be identified. Monospecific RFs were derived primarily from patients with RA and are characterized by a binding specificity for IgG3 and/or IgG4. Polyreactive RFs were derived largely from patients with SLE and show a broader pattern of reactivity to all four isotypes of IgG. Neither population of RFs was exclusive to either disease. The binding specificities identified appear to be different from the RFs isolated from patients with mixed cryoglobulinemia and may reflect a different antigen selection mechanism.     

COX-2在系统性红斑狼疮病人PBMC中的表达及意义

目的：检测环氧全酶-2(Cyclooxygenase-2，COX-2)蛋白在系统性红斑狼疮(Systemic lupus erythematosus．SLE)患外周血单核细胞(Peripheral blood monoeytes，PBMC)中的表达情况。探讨COX-2蛋白表达水平与SLE病情活动程度之间的关系。方法：从外周血中分离PBMC后涂片，用免疫组化染色的方法测定COX-2蛋白的表达情况。结果：COX-2蛋白在SLE实验组呈高表达状态，与SLE疾病的活动性之间呈正相关。结论：COX-2的表达与SLE的发病有关，可作为今后辅助治疗措施的目标和病情变化和疗效评价的参考指标之-。     

神经精神性红斑狼疮的脑部MRI表现

目的 分析神经精神性红斑狼疮（NP-SLE）的脑部MRI表现,探讨MRI对NP-SLE的诊断价值.方法 收集2000年1月～2005年4月符合NP-SLE临床诊断标准病例13例,均为女性,年龄10～40岁,平均30岁,病程30天～25年.采用Simens Impact 1.0T MR成像仪,SE序列行轴位T1WI、T2WI、矢状位T2WI及增强扫描,分析其脑部MRI表现.结果 13例20次MRI检查均有异常,阳性率为100%,表现为点状、斑片状长T1长T2异常信号,主要分布在大脑皮层、层下白质及基底节区,病灶周围无水肿,占位效应不明显.2例增强扫描病灶呈不规则强化,7例有脑萎缩.结论 MRI对NP-SLE脑部病变有较高的价值,但作出NP-SLE诊断需结合临床和实验室检查.