A Chinese adolescent girl with Fechtner-like syndrome

We describe a 15-y-old girl with Fechtner-like syndrome, who is the first Chinese reported to have this rare syndrome. She presented with left homonymous hemianopia and neuroimaging revealed haemorrhage in both parietal and occipital lobes. Peripheral blood smear showed macrothrombocytopenia and intracytoplasmic inclusion bodies inside leucocytes. Thrombocytopenia and proteinuria responded to intravenous immunoglobulin and pulsed methylprednisolone. This case illustrates that life-threatening haemorrhage can occur in patients with Fechtner syndrome. Although there was no effective treatment reported in the literature, high dose steroid and immunoglobulin seemed to be useful in our patient. Our patient also had nephritic-nephrotic syndrome with renal insufficiency, which is unusual in adolescent female patients.     

甲基强的松龙对低温保存大鼠肝脏的保护作用

应用Ｗｉｓｔａｒ大鼠肝脏离体灌流模型，用ＣＭＵ—１号液，含有甲基强的松龙的ＣＭＵ—１号液，以及缺血前预用甲基强的松龙后再用ＣＭＵ—１号液分别对肝脏进行灌洗保存。检测ＬＤＨ、ＡＬＴ、肝组织匀浆ＳＯＤ活性和ＭＤＡ含量，并观察肝组织结构。结果表明：甲基强的松龙对低温保存的离体大鼠肝脏具有保护作用     

甲基强的松龙治疗实验性变态反应性脑脊髓炎的作用机制

目的:研究细胞因子、T细胞凋亡和淋巴细胞增殖在实验性变态反应性脑脊髓炎(EAE)形成中的作用及甲基强的松龙(MP)治疗EAE的作用机制。方法:采用人脑纯化的髓鞘碱性蛋白(MBP)与完全福氏佐剂免疫Lewis大鼠,建立EAE动物模型。用双抗体夹心ELISA法检测各组大鼠血清中IL-10、TNF-α、IFN-γ的含量:流式细胞仪检测外周血T细胞凋亡;3H-TdR释放法检测外周血淋巴细胞转化率。结果:与对照组比较,EAE组的外周血IFN-γ、TNF-α水平明显增高,IL-10水平明显降低,MP治疗后IFN-γ和TNF-α水平下降,IL-10浓度上调。MP还诱导外周血T细胞凋亡和抑制MBP致敏淋巴细胞增殖并呈剂量依赖性。结论:应用人MBP成功建立EAE大鼠模型,MP可能通过调节Th细胞因子格局、促进Th2细胞因子分泌、抑制MBP致敏淋巴细胞增殖及外周血T细胞凋亡而发挥治疗多发性硬化的作用。     

Eye movement quantitative evaluation before and after high-dose 6-methylprednisolone in multiple sclerosis

We studied saccadic and smooth pursuit eye movements in 24 patients suffering from multiple sclerosis during disease worsening, before and after high-dose 6-methylprednisolone infusions. Quantitative evaluation of saccades was based on amplitude/duration and amplitude/peak velocity relationships, precision (i.e. the ratio of actual to desired saccade amplitude) and the latency, whereas smooth pursuit eye movements were studied using target velocity/performance index relationship. At basal recordings, 22/24 (91.7%) of the patients showed at lest one abnormality. Eleven of the 24 patients (45.8%) showed modification of one or several parameters: improvement in 6 patients, worsening in 2, coexistence of both trends in 3. Latency improvement was the only significant modification when patients were considered as a group. Neurophysiological modifications did not correspond to clinical changes.     

脂多糖对大鼠肺微血管内皮细胞Gsα和Giα蛋白亚型的影响*

目的：观察脂多糖（LPS）对大鼠肺微血管内皮细胞（RPMVECs)G蛋白的影响及甲基强的松龙的干预作用。方法：用流式细胞技术（FCM）检测G蛋白亚型的变化。结果：①10μg/ml LPS作用于RPMVECs30和90min后,Gsα蛋白水平较对照组显著下降。甲基强的松龙干预30和90min后,对LPS致RPMVECs膜Gsα蛋白水平的变化有显著抑制作用。②10μg/ml LPS作用于RPMVECs30和90min后,Giα蛋白水平较对照组显著下降。甲基强的松龙干预30和90min后,对LPS致RPMVECs膜Giα蛋白水平的变化有显著抑制作用。结论：①LPS诱导RPMVECS Gsα和Giα蛋白水平的变化可能是LPS诱导RPMVECs单层通透性增加的机制之一。②甲基强的松龙参与抑制LPS诱导RPMVECs单层通透性增加的作用。     

七叶皂苷钠治疗喘息患者的研究

目的　评价七叶皂苷钠注射液治疗慢性喘息型支气管炎和支气管哮喘的有效性与安全性。方法　将 84例慢性喘息型支气管炎和支气管哮喘患者随机分为 2组 :观察组用七叶皂苷钠 2 0mg加入生理盐水 4 0mL中缓慢静脉注射 ,每日 2次 ;对照组用甲基强的松龙 4 0mg加入生理盐水 4 0mL中静脉注射 ,每日 1次。 2组疗程均为 5d。结果　 2组肺功能改善情况、临床疗效和综合疗效均无显著性差异 (P均 >0 .0 5 ) ,观察组和对照组不良反应发生率分别为12 %和 2 1% ,经相应对症处理后不影响治疗。结论　七叶皂苷钠治疗喘息患者安全、有效。     

Peroperative cardiovascular stability during brainstem surgery. The use of high-dose methylprednisolone compared to dexamethasone. A retrospective analysis

BACKGROUND: In neurosurgical procedures within brainstem structures, corticosteroids are routinely administered to prevent oedema and to reduce intraoperative trauma. After replacing the routine administration of dexamethasone (DX) by high-dose methylprednisolone (MP) during surgery for tumours within brainstem structures, a decreased incidence of intraoperative haemodynamic instability events was observed. To test this hypothesis, a retrospective analysis was performed. METHODS: Peroperative data of 62 surgical procedures of brainstem tumours were retrospectively analysed with respect to haemodynamic instability requiring changes in surgical strategy and/or emergence medication with vasoactive drugs. Severe changes in haemodynamic parameters were defined as a significant increase or decrease in heart rate and/or mean arterial blood pressure greater than 30% compared to baseline values. From 1988 to 1994, intravenous dexamethasone was given peroperatively in 33 patients. After a bolus of 1 mg kg(-1) body weight (BW) 30 min preoperatively, 0.2 mg kg(-1) were given every 4 h. From 1994 until now, methylprednisolone was administered instead of dexamethasone in 29 patients. After an initial bolus of 30 mg kg(-1) BW immediately before surgery, 5.4 mg kg(-1) h(-1) were given 23 h postoperatively. RESULTS: The results of this retrospective analysis suggest that the number of operations with episodes of bradycardia, arterial hypotension (P<0.05), tachycardia and arterial hypertension (P<0.005) was significantly decreased in the group of patients treated with high-dose methylprednisolone. CONCLUSION: The retrospective analysis of the clinical data showed that the routine use of high-dose methylprednisolone was associated with a decreased incidence of haemodynamic instability in a selected group of patients undergoing brainstem surgery. This finding has to be proven in prospective double-blind controlled studies.     

肾囊内注射甲泼尼龙治疗儿童难治性紫癜性肾炎

目的探讨肾囊内注射甲泼尼龙治疗儿童难治性紫癜性肾炎(HSPN)的疗效。方法难治性HSPN患儿22例随机分为3组:Ⅰ组,口服泼尼龙组;Ⅱ组,静脉注射大剂量甲泼尼龙组;Ⅲ组,肾囊内注射甲泼尼松组。连续观察8周,分别于0、4、8周检测患儿24 h尿蛋白量、血清清蛋白(Alb)、肌酐(Scr)、血浆胆固醇(Cho)。结果4周时3组24 h尿蛋白分别为(2.35±1.09)(、0.97±0.37)、(0.99±0.52)g,3组间有显著差异(P<0.01);8周时3组24 h尿蛋白分别为(2.13±1.68)(、1.57±0.89)(、0.19±0.11)g,3组间有显著差异(P<0.05)。观察治疗期间肾囊注药组患儿血清Alb、血浆Cho渐恢复至正常水平。结论肾囊内注射甲泼尼龙可减少难治性HSPN儿童尿蛋白排出。     

琥珀酸钠甲泼尼龙联合纤维支气管镜术治疗难治性肺炎支原体肺炎的疗效观察

目的：比较观察琥珀酸钠甲泼尼龙联合纤维支气管镜术治疗难治性肺炎支原体肺炎（ RMPP）的临床疗效。方法选择120例RMPP患儿作为研究对象,随机分为激素组、支气管镜组和联合治疗组。常规治疗的基础上,激素组仅给予静脉注射琥珀酸钠甲泼尼龙进行治疗;支气管镜组仅给予纤维支气管镜术进行治疗;联合治疗组给予琥珀酸钠甲泼尼龙静脉注射联合使用纤维支气管镜术治疗。评价3组胸片恢复正常时间、咳嗽缓解时间、体温恢复时间、治疗前后超敏C-反应蛋白（ CRP）恢复时间及不良反应。结果3组患者各体温分布人数比较（ F ＝9?．929, P ＝0．042）,组1和组3（χ2＝9．043, P ＝0．011）,组2和组3（χ2＝8．402, P ＝0．015）差异有统计学意义;患儿在治疗2周后,体温恢复时间比较（F＝7．024, P ＝0．013）,组1与组3（ t ＝7．505, P ＝0．010）、组2与组3（ t ＝5．005, P ＝0．043）差异有统计学意义;患儿胸片恢复时间比较（ F ＝6．559, P ＝0．028）,组1与组3（ t ＝6．384, P ＝0．022）、组2与组3（ t ＝6．102, P ＝0．0．028）,差异有统计学意义;患儿CRP恢复时间比较（ F ＝8．066, P ＝0．007）,组2与组3（t＝6．432, P ＝0．030）,差异有统计学意义;3组患儿治疗效果比较（ F ＝20．557, P ＝0．000）,组1和组3（χ2＝14．607, P ＝0．001）、组2和组3（χ2＝8．098, P ＝0．017）、组1和组2（χ2＝6．857, P ＝0．032）,差异统计学意义;患儿在血清铁蛋白比较（ F ＝6．605, P ＝0．024）,组1和组3比较（ t ＝－5．509, P ＝0．040）、组2和组3比较（ t ＝－7．159, P ＝0．015）,差异均有统计学意义;在CRP的恢复程度差异有统计学意义（ F ＝6．605, P ＝0．024）,组1和组3比较（ t ＝8．082, P ＝0．006）、组2和组3比较差异有统计学意义（ t ＝8．892, P ＝0．004）。结论琥珀酸钠甲泼尼龙联合纤维支气管镜术治疗难治性肺炎支原体肺炎能更快地缓解临床症状,具有一定的临床疗效。     

Combination therapy of tacrolimus,high doses of glucocorticosteroids,and cyclophosphamide against existing historical treatment for patients in severe conditions of interstitial lung diseases complicated with dermatomyositis: A retrospective analysis

The high-dose glucocorticosteroid (GC) treatment is the first choice for dermatomyositis complicated with interstitial lung disease (DM-ILD) but patients are resistant to the high-dose GC monotherapy. Besides, the high dose of GC, the secondary immunosuppressive agent(s) is necessary but there is controversy for the selection of immunosuppressive agent(s). The objectives of the study were to analyze the efficacy of different therapeutic options for DM-ILD to identify the optimal therapy. A total of 60 patients had received intravenous 1.0–2.0 mg/ kg/day prednisolone for DM-ILD. In severe conditions, patients had received oral 1 to 3 mg/day tacrolimus (TAC), 500 mg/ m²/month cyclophosphamide (CY), and/or 1 g/ day methylprednisolone pulse (TI cohort, n = 24). In severe conditions, patients had received 1 g/day methylprednisolone pulse and 2–3 mg/ kg/day cyclosporine A (CsA) and/or 500 mg/ m²/month CY (existing historical treatment; CT cohort, n = 36). Patients of the TI cohort did not receive CsA. Patients in the CT cohort were received CY in significantly fewer numbers than those of the TI cohort during treatment (P = .0112). A total of 11 (46%) patients from the TI cohort and 14 (39%) patients from the CT cohort were developed relapsed. At the end of the 30-months, higher numbers of patients of the TI cohort had an event(s) free survival than those of the CT cohort (7 (29%) vs 2 (6%), P = .0229). Also, higher numbers of patients of the TI cohort had survived irrespective of an event(s) than those of the CT cohort (21 (87%) vs 22 (61%), P = .0399). Patients of the TI cohort had developed herpes zoster (2 (8%)) and cytomegalovirus (4 (17%)) infections. Patients of the CT cohort developed renal dysfunction (10 (28%)). Hyperglycemia, hyperlipidemia, and fracture (GC-related toxicities) were also reported in both cohorts and these toxicities were fever in the TI cohort. The addition of TAC to high doses GC with CY is an ideal treatment for severe conditions of DM-ILD (Level of Evidence: III; Technical Efficacy Stage: 4).