Evaluation and Treatment of Children with Primary Immune Deficiency in the Emergency Department

The potential for morbidity and mortality in patients who have PID with febrile and nonfebrile illness is extremely high. Familiarity with the clinical manifestations of PID and collaboration with a pediatric immunologist are prerequisites for optimal short-term care of these complex patients. Conservative management with empiric broad-spectrum antimicrobials, early and aggressive surgical debridement of abscesses, and admission at a tertiary pediatric care center are often indicated.     

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.

     

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。     

加味麻黄附子细辛汤治疗窦性心动过缓临证体会

追溯麻黄附子细辛汤的组方背景,分析麻黄附子细辛汤的药物组成,论述麻黄附子细辛汤和窦性心动过缓之间的联系,介绍治疗窦性心动过缓的临床用药特点,指出窦性心动过缓的发病机制与心气、心阳亏虚紧密相关,明确气阳两虚、阴寒内盛的发病病机,治疗以益气温阳散寒为主要治则,用药在益气温阳药的基础上,酌加活血药、滋阴药,收效可观,并附典型病例1则。     

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...     

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。     

Changes in activity of some mechanisms of specific and nonspecific immunity in vitamin B1 deficiency

The effect of thiamine deficiency on the immune response and activity of certain mechanisms of natural immunity was studied in adult rats. Thiamine deficiency was simulated experimentally by a single injection of hydroxythiamine, a vitamin B₁ antagonist. Administration of hydroxythiamine caused a marked decrease in complement activity, phagocytic activity of the peripheral blood leukocytes, bactericidal activity of the serum, and antibody production in response to immunization with sheep's red blood cells. Conversely, lysozyme activity increased. In vitamin B₁ deficiency the intensity of incorporation of [¹⁴C]leucine into liver protein synthesis was reduced.Department of Pathological Physiology, N. I. Sechenov First Moscow Medical Institute. (Presented by Academician of the Academy of Medical Sciences of the USSR G. V. Vygodchikov.) Translated from Byulleten' Éksperimental'noi Biologii i Meditsiny, Vol. 88, No. 7, pp. 60–62, July, 1979.     

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.