Familial Juvenile Nephronophthisis in Two Siblings — Histological Findings at an Early Stage —

We present two female siblings with familial juvenile nephronophthisis (FJN) which was diagnosed at the early stage of renal failure. Diagnosis was made during the investigation of anemia in case 1 and by a subsequent family survey in case 2. Most patients with FJN are not identified until the terminal stage of renal failure and such cases have rarely been reported in Japan. Case 2 had a reduction in the maximum urinary concentration ability but no azotemia, and among the FJN patients reported in Japan so far she has the least advanced renal disease. Histological examination of the renal biopsy in case 1 showed typical findings of FJN, such as thickening and lamination of the tubular basement membrane (TBM), interstitial fibrosis, and round cell infiltration of the interstitium. In case 2, renal biopsy revealed an irregular marked thickening of the TBM with trivial interstitial changes and a normal glomerular appearance. The histology of these two cases suggests that the TBM may be the primary site affected in FJN.     

臀肌挛缩症的围手术期护理

臀肌挛缩症（gluteal muscle contracture，GMC）是由多种原因引起的臀肌及其筋膜纤维变性、挛缩，导致髋关节功能受限所表现的特有步态、体征的临床综合征。1989年陆裕朴在国内已有系统回顾后，我国病例报道逐渐增多，普及各大省市，特别是那些偏远的山区或基层农村。由于医疗卫生条件差，再加上人们的医疗卫生、保健知识缺乏，导致对本病的认识不足，该病的发病率农村明显的高于城镇。轻者造成患儿异常姿势和特殊步态。严重的可导致骨关节畸形、骨盆倾斜、跛行等。影响患者的正常发育及生活能力。     

米非司酮作用于兔输卵管收缩活动与Ca^2＋关系的研究

通过“结合化学分离与原子吸收分光光度法”对Ca^2 含量测定和离体输卵管肌条收缩记录方法，研究米非司酮（Ru486)对增加假孕（4d)兔输卵管平滑肌收缩频率，而不改变其收缩张力和振幅时与Ca^2 之间的关系，结果提示：（1）Ru486对输卵管分泌液和组织内Ca^2 的含量分布无明显影响；（2）Ru486具有降低细胞外液高Ca^2 浓度促Ca^2 内流，而使收缩增强的效应，且能协同Ca^2 通道阻断剂（Verapamil)抑制细胞外Ca^2 内流，而致收缩减弱的效应。     

核骨架/核基质结合区及其功能研究的进展

长期以来的研究只能用结合试验来证明核基质结合区(MARs)的存在,对其真正的功能了解甚少.直到最近,人们才发现了MARs的一些生物学活性,MARs参与襻环的形成和染色体的高级包装,在真核生物的转录调控和凋亡方面起到了重要作用.对MARs的生物学功能仍在进一步的研究中,本文主要就MARs的结构、在转录调控中的作用及其它一些研究进展作一综述.     

糖皮质激素依赖性皮炎的治疗进展

糖皮质激素依赖性皮炎临床报道中,中医、中西医结合的治疗方法最多,显示出了其优势所在,其中中药疗效优于西药,且在改善和治愈面部潮红、毛细血管扩张、色素变化、病情反跳等症状方面优势明显,对于面部痤疮样皮疹、痒及不适等症状,中、西药治疗疗效相近。目前糖皮质激素依赖性皮炎最好的治疗方法应该是中西医结合疗法。     

肺血管扩张在肝肺综合征发病机制中作用的研究进展

肺血管扩张是肝肺综合征的主要发病机制,然而导致HPS肺血管扩张的机制相当复杂,至今仍不清楚.目前认为肺血管内巨噬细胞聚积和雌激素升高导致的血管活性因子增多和活性增强可能与此有关,近年来这方面的研究很多,本文对此作一综述.     

Expression of neuron-specific enolase and olfactory marker protein in the developing olfactory mucosa of human fetuses]

OBJECTIVE: To study the expression of neuron-specific enolase (NSE) and olfactory marker protein (OMP) in the developing olfactory mucosa of human fetuses. METHOD: The expression of NSE and OMP in the olfactory mucosa of 6 human fetuses (12, 16, 20, 24, 28 and 34 weeks) was studied using the technique of immunohistochemistry. RESULTS: NSE immunological positive reactions were seen in all 6 fetal mucosa from gestational 12 (G12) to G34, with plenty of positive-stained dual-pole neuron cells. At G12, the positive cells aligned tightly, the cell bodies were localized in the lower portion of olfactory epithelium and the positive-stained area occupied upper 2/3 of fetal nasal mucosa. With the development, the positive cells gradually became multilayer, but the density and the relative area of positive-cells reduced. At G34, the positive cells were located only in upper 1/3 of nasal mucosa. OMP-positive reactions were localized in a few dual-pole neurons at G12, the number was much less than NSE-positive cells in the same fetus. With the development, the OMP-positive cells gradually increased with most of the cell bodies located in the upper portion of epithelium, but number still relatively less than the NSE-positive cells at the same age. CONCLUSION: At G12, there were lots of olfactory neuron in the olfactory mucosa and only a few olfactory neurons had became mature. With the development, the olfactory epithelial area reduced but the number of mature olfactory neurons increased. At the last trimester, fetal olfactory sensor was almost matured.     

Platelet-derived growth factor activates mitogen-activated protein kinase in isolated caveolae

The ability of a peptide hormone to affect many different intracellular targets is thought to be possible because of the modular organization of signal transducing molecules in the cell. Evidence for the presence of signaling modules in metazoan cells, however, is incomplete. Herein we show, with morphology and cell fractionation, that all the components of a mitogen-activated protein kinase pathway are concentrated in caveolae of unstimulated human fibroblasts. Addition of platelet-derived growth factor to either the intact cell or caveolae isolated from these cells stimulates tyrosine phosphorylation and activates mitogen-activated protein kinases in caveolae. The molecular machinery for kinase activation, therefore, is preorganized at the cell surface of quiescent cells.     

N－（4－乙氧苯基）苯甲酰胺类化合物的合成及抗炎、抗变态反应活性研究

Ｎ┐（４┐乙氧苯基）苯甲酰胺类化合物的合成及抗炎、抗变态反应活性研究周玉新１）党永红刘建飞２）徐颖刘百里（沈阳药科大学制药系，沈阳１１００１５）郑文义（东北第六制药厂，沈阳１１００４３）１９８１年，刘百里等〔１，２〕发现和研制的新药益肤酰胺，经药理实...     

肠套叠患儿行腹腔镜下空气灌肠整复术的护理

对13例难复性肠套叠患儿行腹腔镜下空气灌肠整复术.结果11例整复成功,2例改行肠切除吻合术.提出术前做好准备及心理护理;术后做好体位护理及病情观察,加强饮食、皮肤、引流管等护理和康复指导是手术成功的重要保证.