Infantile chronic tubulo-interstitial nephritis with cortical microcysts: variant of nephronophthisis or new disease entity?

Over a 15-year period we observed seven children (four girls, three boys) who presented within the first months of life with severe renal failure and acidosis, associated with hypertension in five patients and polyuria in four. In addition, one patient had a severe cholestatic liver disease. In two families, a similarly affected sibling had died previously. Four patients were referred with the clinical diagnosis of polycystic kidney disease because of moderate enlargement of kidneys, but renal imaging (intravenous pyelography and ultrasonography) did not confirm this diagnosis. A renal biopsy, performed in all patients, showed similar features characterized by a diffuse chronic tubulo-interstitial nephritis (TIN) and particularly by the presence of microcystic dilatation of proximal tubules and Bowman's space. Liver pathology was normal in two patients, including one with hepatomegaly. However, in the patient with cholestasis there was inflammatory portal fibrosis with mild duct proliferation. Progression of the renal disease was extremely rapid and all patients reached end-stage renal failure (ESRF) before the age of 2 years (11–22 months). Two children had successful renal transplants. Although this chronic TIN shares some features with nephronophthisis, we suggest that it represents a distinct entity both on clinical and morphological grounds. The specific clinical features of this disease are its early onset and rapid progression to ESRF. Pathologically, it differs from nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts.     

健脾止泻饮治疗糖尿病腹泻32例观察

目的:观察自拟健脾止泻饮治疗糖尿病腹泻的临床疗效。方法:治疗组32例用健脾止泻饮治疗,对照组30例用维生素B1、复方苯乙呱啶、盐酸黄连素治疗。结果:治疗组总有效率93.8%,对照组总有效率56.7%,两组比较有极显著性差异(P<0.01)。结论:健脾止泻饮治疗糖尿病腹泻有较好疗效,且无不良反应和毒副作用。     

Infantile fibrosarcoma: radiological and clinical features

Two cases of infantile fibrosarcoma are described. This rare childhood malignancy of mesodermal origin usually affects the lower limbs, as it did in both of our cases. Previously, the only treatment option available involved some form of radical and often mutilating surgery. More recently, combination chemotherapy has given good results, with the effect that various imaging modalities have become important in assessing both the initial extent of disease and the response to treatment. Computed tomography has the advantage of demonstrating the amount of osseous involvement, but at the expense of a considerable dose of ionizing radiation. On the other hand, magnetic resonance imaging, with its multiplanar capacity, gives superior demonstration of breaching of tissue planes, which has important implications for planning of surgery. However, as in other soft tissue tumours, changes in signal characteristics with treatment have proved less specific than was originally anticipated.     

Plasticity of human motion processing mechanisms following surgery for infantile esotropia

Monocular oscillatory-motion visual evoked potentials (VEPs) were measured in prospective and retrospective groups of infantile esotropia patients who had been aligned surgically at different ages. A nasalward-temporal response bias that is present prior to surgery was reduced below pre-surgery levels in the prospective group. Patients in the retrospective group who had been aligned before 2 yr of age showed lower levels of response asymmetry than those who were aligned after age 2. The data imply that binocular motion processing mechanisms in infantile esotropia patients are capable of some degree of recovery, and that this plasticity is restricted to a critical period of visual development.     

腹泻型肠易激综合征患者结肠黏膜组织异常表达蛋白质的筛选与鉴定

目的 筛选及鉴定腹泻型肠易激综合征(D-IBS)患者与正常人结肠黏膜组织中的差异表达蛋白.方法 D-IBS患者及健康志愿者各4例,分别为D-IBS组和正常对照组.通过结肠镜取回盲部及乙状结肠黏膜进行活检,用含0.1% PMSF的冰盐水将标本洗净后立即置于液氮中保存.提取蛋白质,采用双向凝胶电泳法筛选差异表达蛋白,并采用质谱法对变化最明显的蛋白点进行鉴定.结果 利用双向凝胶电泳技术成功得到人结肠黏膜组织的蛋白质组图谱.正常对照组图谱平均得到蛋白点336个,组内图像平均匹配率92%,D-IBS组图谱平均得到蛋白点426个,组内图像平均匹配率95%.D-IBS组与正常对照组之间进行平均胶图谱的比较,两组间匹配率为74%.Volume值变化大于2倍以上的点共24个,其中21个点表达上调,3个点表达下调.表达上调最明显的2个蛋白点经鉴定分别为免疫球蛋白J链(Ig-j)和热休克蛋白27(HSP27),表达下调最明显的2个蛋白点则分别为血红蛋白β亚基和果糖二磷酸醛缩酶A.结论 采用双向凝胶电泳法可以成功得到人结肠黏膜组织蛋白质组表达图谱,D-IBS患者与正常人结肠黏膜组织蛋白质表达存在差异,已经鉴定出来的4个蛋白可能在D-IBS的发病机制中起一定作用.     

Plasma Concentrations of Mycophenolic Acid Acyl Glucuronide Are Not Associated with Diarrhea in Renal Transplant Recipients

The aim of this study was to determine whether plasma concentrations of the acyl (AcMPAG) and phenolic (MPAG) glucuronide metabolites of mycophenolic acid (MPA) were related to diarrhoea in renal transplant patients on mycophenolate mofetil (MMF) with cyclosporine (CsA) or tacrolimus (TCL). Blood samples (0, 30, 120 min) were taken at days 3, 10, week 4, months 3, 6 and 12 for determination of MPA, MPAG and AcMPAG. MPA-AUC was estimated using validated algorithms. Two hour AUCs were calculated for MPAG and AcMPAG. Immunosuppressive therapy consisted of CsA/MMF (n= 110) and of TCL/MMF (n= 180). In 70/290 (24%) patients 86 episodes of diarrhoea were recorded during 12 months. Significantly more patients on TCL (31.1%) suffered from diarrhea compared to CsA (12.7%). MMF dose, MPA-AUC and the 2 h AUCs of MPAG and AcMPAG did not differ between patients with and without diarrhoea. Plasma AcMPAG and MPAG concentrations were substantially higher in patients on CsA compared with TCL, while MPA-AUC was lower in the former group. These data support the concept that CsA inhibits the biliary excretion of MPAG and AcMPAG, thereby potentially reducing the risk of intestinal injury through enterohepatic recycling of MPA and its metabolites.     

婴幼儿血管瘤病理结构变化与临床演变过程的联系

目的探讨婴幼儿血管瘤患者临床演变过程和病理结构变化的内在联系。方法选取52例婴幼儿血管瘤标本,年龄2个月至11岁。采用HE染色观察各阶段血管瘤的病理特点,使用图像分析系统分析血管瘤组织中的细胞总数量、微血管总数量和总面积。结果按患儿出生后时间:1~6个月,血管瘤中细胞增殖速度快、排列紧密,微血管数量迅速增加;7~12个月,血管瘤中细胞数量迅速减少,微血管数量减少,但总面积继续扩大;1~3岁,大部分血管瘤组织呈微血管团样,微血管面积最大;3~5岁,微血管数量和总面积均迅速减少;5岁之后,大部分血管瘤消退完成,被纤维脂肪组织代替。结论婴幼儿血管瘤患者临床演变过程和病理结构变化有密切联系,病理结构变化主导临床演变过程。     

583例小儿肺炎证候病机学关联规则分析

目的：通过数理分析探讨小儿肺炎的证候病机学。方法：运用关联规则分析583例小儿肺炎发病治疗前症状体征。结果：获得668项频繁项集，其中支持度大于80％者4项：肺部呼吸音粗糙、无恶寒、舌质红、咳嗽每咳数声，共获得关联规则6888条，最高支持度80％，最高置信度98％。结论：小儿肺炎48h内的主要症状体征为：咳嗽每咳数声、咳嗽阵作、气促、发热、多痰、舌质红、肺部呼吸音粗糙、无恶寒、无紫绀、无恶心呕吐等，并且咳嗽每咳数声与咳嗽阵作及发热、无恶寒、气促、多痰、舌质红等症状体征常常关联出现。在证候病机上表现：肺气郁阻是小儿肺炎最主要的病机病理。热邪入里，肺热壅盛，肺气郁阻，津蓄为痰为常见的病机病理改变，且热与郁与痰的病机密切关联。     

小儿感染性腹泻病原与肿瘤坏死因子水平的关系

肿瘤坏死因子是非常重要的炎症介质。本文为观察小儿感染性腹泻时ＴＮＦ水平与病原的关系。对１９９５年６月￣１９９６年８月收治的４６例细菌感染性腹泻，３５例病毒感染性腹泻患儿进行检测，于入院第一天测定ＴＮＦ水平记录当天的最高体温及腹泻次数，同时进行粪便病原学检查，结果表明在两组具有可比性的情况下，细菌性腹泻组较病毒性腹泻组的ＴＮＦ水平显著增高。     

云南省路南彝族自治县小儿急性腹泻病流行病学调查

1986—1987年对云南省路南县5岁以下儿童随机抽样1016例,每月调查急性腹泻病发病情况。结果表明年发病率为1.43±0.07次/年。6月至1岁组发病率最高。4、5、6月为发病高峰季节,病原学检查以产肠毒素埃希氏大肠杆菌为主占39.1％,其次为轮状病毒,占26％。两年定群研究得出小儿急性腹泻病发病危险因素为饮用溏水沟水,随地大小便,多子女家庭与体弱多病儿童及人畜共居等。因此,改善供水,培养卫生习惯,加强计划生育等措施是该县防治本病主导措施。