甲基强的松龙联合静注丙种球蛋白治疗小儿格林巴利综合征

目的:现察甲基强的松龙联合静注丙种球蛋白治疗小儿格林巴利综合征的疗效。方法:选择1999～2001年的26例格林巴利综合征患儿给予甲基强的松龙联合两种球蛋白静注作为治疗组,与1996～1998年的25例作为对照组,观察其用药后症状、体征改善情况。结果:甲基强的松龙配合丙种球蛋白静注治疗后症状、体征尤其是呼吸困难、肢体无力、饮水呛咳的开始缓解时间(约8～12h)较对照组明显提前(P<0.01),总有效率(100％)明显高于对照组(36％)(P<0.01),病死率(0％)和致残率(0％)低于对照组(8％、16％)(P<0.05),治疗组肌力、行走完全恢复正常的时间平均14、23d,对照组平均58、35d。两组对比差异显著(P<0.01)。结论:甲基强的松龙联合静注丙种球蛋白治疗小儿格林巴利综合征疗效显著,值得推广应用。     

Acute leukaemia in a defined geographic area – Incidence,clinical history and prognosis

A consecutive series of patients (1978–1981) comprising all patients with acute leukaemia from a population of 475000 inhabitants was reviewed. Thus, 94 patients were diagnosed as having acute leukaemia. No patients were lost from follow-up. The incidence figures of ALL and AML differed significantly from those of Sweden as a whole. 9 patients were < 15 years old. The median age of adult patients was 64 years, 60.8% being ≥ 60 years old. Of adult patients with AML, 20% had a preleukaemic history (chronic myeloproliferative disorders, myelodysplastic syndromes and others). None of 6 patients with leukaemia as a metamorphosis of a chronic myeloproliferative disorder achieved a complete remission. The overall remission rate of the remaining adult patients was 25%. Treated patients, 15–39 years old, with AML without any preleukaemic history, had a complete remission rate of 80% compared to 12% for patients ≥ 60 years old with the same diagnosis. Of 60 patients with ‘primary’ AML, 14 were not treated, mainly because of advanced age and complicating diseases. Most of these patients died within a week of admission.     

Widespread serum amyloid P immunoreactivity in cortical amyloid deposits and the neurofibrillary pathology of Alzheimer's disease and other degenerative disorders

Amyloid P (AP) component is present in all types of systemic amyloid deposits. Recently, it has been shown to be also present in cerebral amyloid lesions of Alzheimer's disease (AD). In this study, we used immunocytochemical methods to extend these findings at the electron microscope level and characterize the spectrum of AP immunoreactivity in neurofibrillary pathology (NFP) of AD and other neurodegenerative disorders including Down's syndrome (DS), Creutzfeldt-Jakob, Parkinson's, Pick's and diffuse Lewy body diseases and progressive supranuclear palsy. In AD and DS, AP immunoreaction product was evident in all the classical amyloid lesions and NFP in a large sample of all cortical areas examined. The distribution and relative intensity of immunostaining was similar to that of thioflavin S staining in serial sections. In many cases, however, plaques and vessels stained by anti-AP serum were not apparent with thioflavin S. Serial sections immunostained with antiserum to amyloid A, C-reactive protein or to other proteins involved in systemic amyloidoses and the acute phase response showed no evidence of staining in any of the cerebral lesions. Electron microscopy confirmed that AP immunoreactivity was associated with the abnormal filaments characteristic of NFP as well as amyloid fibrils found in plaques and vessels showing congophilic amyloid angiopathy. Plaques of Creutzfeldt-Jakob disease, Pick bodies of Pick's disease, tangles and Lewy bodies in Parkinson's disease and a subpopulation of Lewy bodies in the diffuse Lewy body disease coexistent with AD were also stained. With the exception of vessels in two of the five cases, AP was not detected in age-matched controls. Our observations indicate AP to be a consistent feature of cerebral NFP and amyloid deposits.     

布一加综合征的诊断和治疗(附48例报告)

目的：探讨布一加综合征的诊治经验。方法：回顾分析本院自1993年－2002年，采用超声介入及手术方法治疗48例布加综合征的疗法结果。超声引导下支架术治疗布一加综合征38例，其中内支架术联脾肾分流术治疗5例，支架术联合肠腔分流术治疗8例；腔房人工血管转流术治疗4例，肠腔转流术治疗2例，肠颈转流术治疗2例，经右心房联合破膜2例。结果：随访1个月－9年，平均4．68年，37例获显疗效，2例改善，6例行二次手术治疗，3例死亡。结论：应重视早期诊断和治疗。腔内超声介入方法治疗布一加综合征，简便、准确、安全、疗效肯定；联合分流术解除肝静脉梗阻，可获得满意疗效。     

儿童难治性肾病综合征的探讨95例临床分析

本文对95例肾病综合征进行分析,探讨了性别、年龄、发病迁延时间、浮肿、蛋白尿、血尿、血压、尿素氮、血浆蛋白、血浆胆固醇、免疫球蛋白、补体C_3与激素反应及分型的关系。认为下列综合分析可做为判定难治性肾病综合征的参考。难治性肾病多分布在7岁以上,激素治疗4～8周血浆蛋白尚未恢复,尿镜检反复出现红细胞及颗粒管型,血清r—球蛋白不低,而补体C_3降低,提示难治性肾病。各种感染常常是造成肾病综合心难以控制,甚至死亡的重要因素。     

河南食管癌高、低发区无症状人群食管固有膜血管乳头的变化

目的：探讨食管癌高、低发区无症状居民食管上皮固有膜血管乳头（简称乳头）增生特征（乳头密度和高度）及其与病变分布的关系，加深对食管癌变早期形态学变化特征的了解。方法：2480例无症状人群食管粘膜活检组织，采用食管癌高低发区食管纤维内镜检查，粘膜活检，组织病理学检查和形态学测量技术对食管乳头的分布特征及其与病变的关系进行分析。结果：食管癌高低发区居民食管中、下段乳头升高（≥上皮厚度的1／2）发生率之间差异并不明显（P＞0．05），但是，高发区居民食管中段乳头密度（乳头数目／mm)明显高于下段，并高于低发区居民食管中段的乳头密度（P＜0．05）；高发区居民食管上皮乳头升高伴基底细胞过度增生患者明显高于正常人（P＜0．05），而低发区未观察到类似情况。结论：乳头增生表现为乳头数目增多和乳头升高是食管癌高发区人群食管上皮特征性形态学变化，高分区居民上皮乳头升高伴明显基底细胞过度增生，提示乳头增生可能是食管癌变极早期阶段的重要形态学变化，反映了上皮细胞的增生状态。     

335例术后全身炎症反应综合征的临床分析

【目的】了解手术创伤对术后全身炎症反应综合征 (SIRS)的影响。【方法】搜集外科重症监护室 (SICU) 335例患者的术后资料 ,分析不同手术组SIRS发病率 ;手术时间、失血量与SIRS持续时间的关系 ;SIRS持续时间与术后并发症的关系。【结果】术后SIRS发病率为 75 8% ,大手术高达 92 4 % ;无并发症患者失血量与SIRS持续时间呈正相关 (r1=0 783,P<0 0 1) ,手术时间与SIRS持续时间呈正相关 (r2 =0 398,P <0 0 1) ;随着SIRS持续时间延长 ,并发症发病率显著增高 (P<0 0 5 )。【结论】术后SIRS发生、发展与手术创伤密切相关 ;监测SIRS进程有助于及早发现并发症     

建立筋膜间室综合征模型的新方法

目的：建立一种更符合临床的筋膜间室综合征动物模型，以便于基础及临床研究。方法：用宽度和犬小腿肌腹长度相当的袖带包绕小腿，然后充气使实验肢体全长受压、缺血。12只犬随机分为两组，I组：袖带充气至40kPa，压迫8h；Ⅱ组：袖带充气至80kPa，压迫8h。结果：动态监测组织压并对神经、肌肉行组织学检查，发现实验动物肢体胫前筋膜间室内组织压远远超过4.0kPa，神经、肌肉发生不可逆变性、坏死。结论：用充气袖带压迫法建立的动物模型是成功的，各项指标均达到筋膜间室综合征的诊断标准。     

染色内镜和放大内镜技术是提高早期大肠癌诊治水平的重要手段

应用常规内镜技术难以发现大肠平坦型病变和凹陷型病变。近年来染色内镜和放大内镜技术已经发展成熟,在国外已获广泛应用,可以发现大肠微小病变和早期大肠癌。应用腺管开口分型方法可以预测肿瘤病变的组织学类型及肿瘤的浸润深度,据此可确定行内镜下粘膜剥离术或分片粘膜剥离术将肿瘤切除,抑或行外科手术治疗。在当前我国的胃肠内镜医疗界,应广泛开展染色内镜和放大内镜的临床应用,以早期发现大肠病变,提高我国大肠癌的内镜诊治水平。     

Efficacy of cyclosporin in difficult-to-treat idiopathic membranous nephropathy

SUMMARY: Poor tolerance and the potential long-term toxicity have limited the widespread use of corticosteroids and cytotoxic drugs in the treatment of idiopathic membranous nephropathy (IMN). Cyclosporin A (CyA) has been proven to be a less toxic alternative, but its efficacy needs further confirmation. Cyclosporin A (2–3mg/kg per day) in combination with low-dose methylprednisolone (4mg/day) was given to 28 nephrotic patients with IMN who had failed to respond, or tolerate, or to complete treatments with steroids and/or cytotoxic drugs. the mean duration of treatment was 11 ± 7 months. Seven patients (25%) showed a complete remission of proteinuria, 17 (60%) a partial one, and four (15%) did not respond at all. the average time to achieve optimal remission was 4.2 ± 1.4 weeks following the initiation of therapy. In those who responded completely or partially, plasma creatinine (Per) did not change significantly from pre CyA levels during follow up (1.0 ± 0.3 vs 1.2 ± 0.3mg/dL, P =NS). the remaining four patients who had renal insufficiency already before CyA (mean Per: 2.1 ± 0.8mg/dL), showed a rapid deterioration of renal function after the initiation of CyA (mean Per: 3.1 ± 1.5 mg/dL, P <0.01), and as a consequence, the drug was discontinued. A mul-tivariate analysis on the clinical and histological features demonstrated that the degree of renal function impairment ( P <0.02), the percentage of obsolete glomeruli ( P <0.01), and the severity of interstitial fibrosis ( P <0.005) independently predicted the response to therapy. Low dose CyA is an effective and safe alternative treatment for patients with IMN and normal renal function. However, the drug should be given with caution to patients with established renal insufficiency.