Characteristics of prolidase and prolinase in prolidase-deficient patients with some preliminary studies of their role in skin

• 1) The prolidase (Pd) and prolinase (Pn) activities of cultured skin fibroblasts derived from two prolidase-deficient sisters, the elder with typical clinical manifestations [symptom (+)] and the younger with only slight clinical manifestations [symptom (–)] were examined biochemically. Pd activity against several substrates other than Gly-Pro were present to some degree in both sisters. There were no detectable differences in Pd activity between the symptom (+) patient and the symptom (–) sister. Pn activity seemed to be increased in both. The lower Pn activity found against Pro-Gly as compared with those against other substrates indicates that Pro-Gly, which has been used for Pn assays in most previous reports, may not be the best substrate for this test. Pd derived from control fibroblasts was activated by Mn²⁺ against all substrates tested in this experiment. Cu²⁺, Hg²⁺, Cd²⁺ and Zn²⁺ remarkably inhibited enzyme activity, Co²⁺ slightly inhibited it, and neither Mg²⁺ nor Fe²⁺ had any remarkable effect. The Pd derived from the prolidase-deficient patients was also activated by Mn²⁺. This Pd seemed to be more inhibited by Co²⁺ than was the control. However, we found no remarkable differences between the two patients.
• 2) We also studied Pd and Pn activities in rat skin and blood during wound healing. Pd and Pn activities adjacent to the wound increased in parallel with fibroblast proliferation. Pd activity was also detected in an extract of newborn mouse epidermis.

     

Pattern electroretinogram can be more than the sum of local luminance responses

It is generally accepted that the pattern electroretinogram for very large spatial elements is the result of local luminance stimulation. Responses due to the luminance differences between elements may be assumed to be relatively unimportant because in the case of large elements only few retinal units are stimulated by gradients. With decreasing pattern element size one wonders to what extent the electroretinogram continues to be based on the local luminance stimulation. We investigated this question using 8 Hz checkerboard reversal and compared the pattern recordings with the recordings resulting from the same stimulus field modulated homogeneously (focal electroretinogram). A 100% modulated checkerboard at retinal level may be considerably less modulated because of imperfect optics of the eye. So the pattern electroretinogram should be compared with homogeneous field stimulation of correspondingly lower modulation depth. On the basis of the optical transfer properties of the eye we compared by subtracting the proper focal electroretinogram from the pattern electroretinogram. The difference response was virtually zero for check sizes larger than 120. For checks from 60 down the difference response was of the same order of magnitude as the adjusted focal recording. This difference response for eyes with normal optics is largest around 30; its wave form was found to be rather invariant with check size.     

肉桂挥发油对“虚证”模型环核苷酸系统反应性的影响

本实验用纯系CFW小鼠与Wistar大鼠建立“虚证”模型。以环核苷酸系统反应性为指标观察了肉桂挥发油的作用。通过气相色谱及气质联用等方法研究表明,本实验所提取的挥发油中桂皮醛含量约为92％。肉桂挥发油可显著降低“阳虚”——甲状腺素缺乏(甲减)模型升高了的cGMP系统反应性而使其降低了的耗O_2率升高;这是一种治疗作用。肉桂挥发油可显著升高“阴虚”——甲状腺素过多(甲亢)模型已升高了的cAMP系统反应性并使其升高了的耗O_2率进一步升高,这是一种恶化作用。这两种作用与中医对症治疗的原则是一致的。     

不同生长激素分泌状态的矮身材儿童血脂水平的研究

目的 探讨不同生长激素分泌状态下矮身材儿童血脂水平的差异,为生长激素缺乏对儿童体脂代谢的影响提供理论依据。方法 收集矮身材儿童188例,依据生长激素药物激发试验峰值分为生长激素完全缺乏(cGHD)组、生长激素部分缺乏(pGHD)组、非生长激素缺乏性(nGHD)组,研究对象均禁食禁水10 h后空腹测定总胆固醇(TC)、三酰甘油(TG)、低密度脂蛋白胆固醇(LDL)和高密度脂蛋白胆固醇(HDL)4项血脂水平。结果 3组儿童HDL水平比较差异无统计学意义(P>0.05);3组儿童TC、TG、LDL、non-HDL水平比较差异均有统计学意义(P<0.05);组间两两比较,cGHD组TC、LDL、non-HDL水平较其他组明显升高(P<0.05),cGHD组TG水平较nGHD明显升高(P<0.05),与pGHD组比较差异无统计学意义(P>0.05)。pGHD、nGHD组高TC、高LDL及高non-HDL的发生率明显低于cGHD组(P<0.05),nGHD组临界高LDL的发生率明显低于cGHD组(P<0.05)。而3组间TG、HDL的异常发生率及TC、TG...     

New metrics for analysis of dendritic branching patterns demonstrating similarities and differences in ON and ON-OFF directionally selective retinal ganglion cells.

The morphology and dendritic branching patterns of retinal ganglion cells have been studied in Golgi-impregnated, whole-mount preparations of rabbit retina. Among a large number of morphological types identified, two have been found that correspond to the morphology of ON and ON-OFF directionally selective (DS) ganglion cells identified in other studies. These two kinds of DS ganglion cell are compared with each other, as well as with examples of class I, class II, and class III cells, defined here with reference to our previous studies. Cell body, dendritic field size and branching pattern are analyzed in this paper and levels of dendritic stratification are examined in the following paper. ON DS ganglion cells are about 10% larger in soma size and about 5 times the dendritic field area of ON-OFF DS ganglion cells, when compared at the same retinal location. These two morphological types of ganglion cell can be said to define the upper and lower bounds of an intermediate range of cell body and dendritic field sizes within the whole population of ganglion cells. Nevertheless, in previous physiological studies receptive field sizes of the two types were shown to be similar. This discrepancy between morphological and physiological evidence is considered in the Discussion in terms of a model of the excitatory receptive field of ON-OFF DS ganglion cells incorporating starburst amacrine cells. A new set of metrics is introduced here for the quantitative analysis and characterization of the branching pattern of neuronal arborizations. This method compares the lengths of terminal and preterminal dendritic branches (treated separately), as a function of the distances of their origins from the soma, viewed graphically in a two-dimensional scatter plot. These values are derived from computer-aided 3D logging of the dendritic trees, and distance from the soma is measured as the shortest distance tracked along the dendritic branches. From these metrics of the "branch length distributions," scale-independent branching statistics are derived. These make use of mean branch lengths and distances, slopes of lines fitted to the distributions, and elliptical indices of scatter in the distributions. By these measures, ON and ON-OFF DS ganglion cells have similar branching patterns, which they share to varying degrees with functionally unrelated class III.1 ganglion cells. The scale of the branching patterns of ON and ON-OFF DS cells and their degree of uniformity are different, however.(ABSTRACT TRUNCATED AT 400 WORDS)     

Debrancher deficiency neuromuscular disorder with pseudohypertrophy in two brothers

Summary A neuromuscular disorder is reported in two brothers, aged 28 and 38 years, with glycogenosis type III. Both patients had proximal weakness, pseudohypertrophy of sternocleidomastoid. trapezius and quadriceps muscles, mild distal wasting and myopathic EMG changes. Pseudohypertrophy was more evident in the younger brother, whereas weakness was prominent in the older one. In the former, muscle biopsy revealed vacuolar myopathy and virtual absence of amylo-1,6-glucosidase enzyme. Few familial cases of debrancher deficiency neuromuscular disorder have been reported. Distal wasting has been considered a quite characteristic manifestation of the disease. It is also suggested that this particular kind of pseudohypertrophy may represent a distinctive feature of glycogenosis type III.     

呼出气氢测定试验对飞行人员乳糖酶缺乏症的研究

对66名汉族飞行人员进行乳糖呼出气氢测定试验,乳糖吸收不良的发生率为83.3％,其中乳糖不耐受者占34.6％;与一般汉族人群无明显差别。对10名确定为中度以上乳糖吸收不良的飞行人员进行250ml鲜牛奶的试验结果,有50％呼出气氢含量在正常范围,并无一例出现胃肠道症状。提示较长期食用牛奶未能使乳糖酶缺乏状态发生改变,但每日食用适量牛奶属合理营养。     

Measure of association between selenium status and sepsis in cattle

Odds ratio was used to evaluate relationship between sepsis in cattle less than 1 year old and mastitis in cows and erythrocyte glutathione peroxides (GSH-PX) activity as an indicator of selenium status. Data were from 178 blood samples collected from referred cases to Urmia University, Veterinary Teaching Hospital. Low activity of GSH-PX was significantly associated with higher odds of developing sepsis (P=0.005) in young cattle and mastitis (P=0.044) in adult cows. The odds ratios for the low activity of GSH-PX on incidence of sepsis and mastitis were 4.74 and 3.95, respectively. Selenium deficiency was associated with sepsis in young cattle and mastitis in cows, i.e., cattle with low activity of erythrocyte GSH-PX were at increased risk of sepsis and mastitis.     

Successful in-vitro fertilization pregnancy with spermatozoa from a patient with Kartagener's syndrome: case report

This paper reports on the successful treatment by in-vitro fertilization (IVF) of a couple in whom the male partner had Kartagener's syndrome. His spermatozoa were severely asthenozoospermic with deficient dynein arms and disordered microtubular configuration. On computer-assisted sperm analysis (CASA) motile spermatozoa displayed straight non-progressive motility with minimal amplitude of lateral head displacement and none were hyperactivated. This is the first case report in which spermatozoa with axonemal disruption in a man with immotile cilia syndrome (ICS) have been shown to be able to penetrate the zona pellucida and fertilize oocytes. IVF may be a suitable treatment for certain variants of ICS.     

新生儿G6PD缺陷病和晚发性维生素K缺乏症的特点与预防

目的 探讨新生儿C6PD缺陷病和晚发性维生素K缺乏症的危害与预防措施。方法 回顾分析1995-2000年儿内科住院的1周-2月(不含2月)的婴儿3104例次，其中病死56例。结果 1周-2月的小婴儿占住院患儿的19．34％，其中新生儿G6PD缺陷病239例，占7．70％；晚发性维生素K缺乏症92例，占2．96％。死因的第2、3位分别是晚发性维生素K缺乏症(13例，占23．21％)和新生儿C6PD缺陷病(12例，占21．43％)，两者的病死率分别为14．13％和5．02％，极显著高于(x^2＝17．59，P＜0．01)或相近于(x^2＝0．88，P＞0．05)肺炎的3．57％。新生儿G6PD缺陷病合并感染占38．49％、低氧血症占23．35％、低血糖占19．25％、酸中毒占15．90％，继发胆红素脑病占13．81％。晚发性维生素K缺乏症出现抽搐占90．22％、胃肠、注射部位出血占60．89％；CT证实颅内出血占98．91％。结论 1周-2月的小婴儿约占住院患儿的两成，新生儿G6PD缺陷病和晚发性维生素K缺乏症的病死率均很高，两者是除肺炎外最主要的死因。提议制定并推广预防这2种疾病的常规措施，并参照国内外相应的现状拟出其具体内容。