Propionic acidemia: report of a case that is successfully managed by peritoneal dialysis and sodium benzoate therapy

Propionic acidemia is a rare hereditary disease which is an autosomal recessive disorder. Defect of propionyl CoA carboxylase results in abnormal accumulation of propionate and its metabolites which interfere the pathway of glycine cleavage and the urea cycle. This organic acidemia is characterized by a wide spectrum of clinical and biochemical findings, including recurrent vomiting, difficult feeding, lethargy, hypotonia, metabolic ketoacidosis, hyperglycinemia and hyperammonemia during the acute episodes. We present a male newborn infant who sustained this disorder and was managed successfully with blood exchange transfusion, peritoneal dialysis, supplemented with sodium benzoate and sodium bicarbonate therapy. Urine gas chromatography disclosed significant elevation of propionate and its metabolites which subsided 2 days after peritoneal dialysis. Special designed formula was then given with restriction of protein intake and supplement with sodium benzoate and sodium carbonate. Prenatal genetic counseling is necessary in further pregnancy. Diagnosis can be obtained when propionyl CoA carboxylase activity is low in cultured amniotic fluid cells or chorion villi sample or when there is abnormally high methylcitrate level in amniotic fluid.     

Fatty acid profiles in response to soybean oil lipid emulsion infusions in essential fatty acid-deficient miniature swine

The ability of soybean oil lipid emulsions to affect essential fatty acid deficiency (EFAD) and plasma fatty acid distribution was studied in neonatal pigs. The test animals were maintained on a fat-free diet prior to administration of lipid emulsion. Plasma and red blood cell (RBC) membrane levels of essential [linoleic (C-18:2 omega 6) and arachidonic (C-20:4 omega 6)] and nonessential [palmitic (C-16, palmitoleic (C-16:1 omega 7), stearic (C-18), and oleic (C-18:1 omega 9)] fatty acids and the triene:tetraene ratio [5,8,11-eicosatrienoic acid (C-20:3 omega 9):arachidonic acid (C-20:4 omega 6)] were monitored to ascertain the establishment of EFAD and its correction. Nonessential fatty acids were studied, as these components of lipid therapy have received little attention. Results indicate that soybean oil emulsions are effective in reversing fatty acid profiles found in EFAD, and both essential and nonessential fatty acids are under strict metabolic control.     

严重鼻衄行颌内动脉栓塞术并发脑梗塞死亡

报道１例严重鼻衄伴高血压、动脉硬化患者，经常规方法止血无效，行患侧颌内动脉超选择性栓塞术。术后鼻出血停止，但出现脑梗塞、昏迷，经抢救无效，于术后第９天死亡。认为对有动脉硬化的患者，应不用或慎用超选择性动脉栓塞术。     

采用生活垃圾研制烧结砖

该文介绍了用好氧发酵工艺对新鲜生活垃圾进行预处理,用做制砖的原料。制砖工艺采用对辊,双轴搅拌,真空挤出,轮窑烧成等工序,垃圾掺加量为30％～50％,可完全取代内掺材料,节省90％以上的煤炭。     

重组人表皮生长因子对促进Ⅱ度烧伤愈合的作用

目的 探讨重组人表皮生长因子(rhEGF)对促进Ⅱ度烧伤创面愈合的作用。方法 采用自身对照研究方法,选取46例Ⅱ度烧伤患者观察最适治疗浓度创面局部应用的治疗效果及不良反应。结果 在浅Ⅱ度烧伤创面,观察组的愈合时间为(8.7±2.1)天,对照组为(12.2±2.4)天,而深Ⅱ度烧伤创面观察组的愈合时间是(13.4±2.6)天,对照组为(19.2±3.2)天。与对照组相比,观察组创面愈合时间均有明显缩短(P<0.01),结论 rhEGF对Ⅱ度烧伤创面均可有效地促进组织修复,加快创面愈合速度,有良好的促进愈合作用,而药物反应极轻微。     

A Bronchial Response Comparison of Exercise and Methacholine in Asthmatic Subjects

The authors compared the inhaled methacholine and exercise responses in 22 stable unmedicated asthmatic patients. The exercise and methacholine challenges were performed at one to three week intervals. Bronchial responsiveness to methacholine was measured in relation to the concentration of methacholine (PC₂₀M). The response to exercise was expressed as the percentage of fall in FEV₁, from the pre-exercise FEV₁ The findings showed that 21 of 22 subjects demonstrated a fall in FEV₁, of more than 20% after methacholine challenge, while only 9/22 subjects experienced a similar decrease in FEV₁. All 9 of these positive response exercise cases completed three consecutive exercise challenges prior to the methacholine challenge. Of these cases, five were refractory to the repeated exercise challenge, and the PD₂₀M at the nonexercise stage was significantly lower than the postexercise state. In fact, the methacholine challenge sensitivity actually decreased (PD₂₀ increased) after repeated exercise. The authors concluded that methacholine seems to be a more sensitive bronchial provocation test than exercise. Second, only 55.6% of the exercise test-positive subjects were refractory to the second exercise challenge. Therefore, other factors besides the release of mediators should be considered in exercise induced asthma. Third, methacholine sensitivity actually decreased (PD₂₀ increases) after repeat exercise challenge.     

A study on plasma norepinephrine and epinephrine levels in TCM liver syndromes.

The levels of plasma norepinephrine (NE) and epinephrine (E) were determined by HPLC in 220 patients with various TCM liver syndromes, and in 96 healthy subjects as controls. The plasma NE and E contents were higher in patients with liver excess syndromes, including Ganqi Yujie (GQYJ [symbol: see text]) syndrome, Ganyang Shangkang (GYSK [symbol: see text]) syndrome, Ganhuo Shangyan (GHSY [symbol: see text]) syndrome, and Ganyang Huafeng (GYHF [symbol: see text]) syndrome; while they were lower in patients with liver deficiency syndromes, including Ganqixu (GQX [symbol: see text]) syndrome, Ganxuexu (GXX [symbol: see text]) syndrome, and Ganyinxu (GYX [symbol: see text]) syndrome. Futhermore, when Ganshen Yinxu (GSYX [symbol: see text]) syndrome was turned into GYSK and then into GYHF, the plasma NE and E contents increased in order of precedence; while when GQYJ was turned into Ganyu Pixu (GYPX [symbol: see text]) syndrome, both NE and E contents decreased. The results suggest that the plasma NE and E contents are reliable objective parameters for the study of pathophysiological basis of the liver excess and liver deficiency syndromes, and the liver-kidney and liver-spleen interrelated syndromes.     

核黄素缺乏大鼠红细胞维生素E水平的变化及脂质过氧化关系的研究

对两组大鼠分别喂饲核黄素缺乏（ＲＤ）膳和核黄素添加（Ｒ８，２２ｍｇ／ｋｇ饲料）膳８周后，测定了两组大鼠的红细胞维生素Ｅ（ＲＢＬＶｅ）、红细胞超氧化物歧化酶（ＳＯＤ）和红细胞丙二醛（ＭＤＡ）的水平。结果发现：ＲＤ组ＲＢＣＶｅ水平（４．７１７３±０．７７１０ｍｇ／ｇ蛋白质）显著低于ＲＳ组（５。３８６８±１．１５３７ｍｇ／ｇ蛋白质，Ｐ＜０．０５）。而ＲＤ组的ＲＢＣＳＯＤ（７７４５．２±６１０．１ｕ／ｇ蛋白质）和ＭＤＡ（０．６８６８±０．１３７２μｇ／ｇ蛋白质）则分别显著低于和高于ＲＳ组（８２６８．５±３０１．０ｎｕ／ｇ蛋白质，０．５５４８±０．０９８０，Ｐ＜０．０５）。研究提示，核黄素缺乏引起细胞膜脂质过氧化加重可能ＲＢＣＶｅ消耗增加。     

城市公共厕所发展趋势的探讨

介绍了改革开放以来各地出现的一些新型公厕,认为前店后厕、附属式公厕、生态公厕是发展趋势,而豪华型公厕的推广目前尚不具备条件.     

Detection of mutations in the p53 gene in human head and neck carcinomas by single strand conformation polymorphism analysis.

Using the polymerase chain reaction (PCR)-based single strand conformation polymorphism (SSCP) analysis, we have examined the highly conserved regions of the p53 gene in 58 biopsy samples of head and neck tumors. Mutations were found in 13/58 (23%) tumor specimens, but not in 6 normal tissues. Ten of 13 mutations were due to single base changes and the remaining 3 were 1- or 8-base deletion mutants. These mutations were clustered in exons 5 and 7 and resulted in amino acid changes. Our results seem to indicate that mutations in the p53 gene contribute to a significant number of cases of the head and neck tumors including 20% of nasopharyngeal carcinoma biopsies. The relationship of Epstein-Barr virus or human papillomavirus and p53 gene mutations in this group of cancers was also analyzed and discussed.