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.     

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。     

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.     

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.     

Localization of Pituitary Adenylate Cyclase-Activating Polypeptide (PACAP) in the Hypothalamus-Pituitary System in Rats: Light and Electron Microscopic Immunocytochemical Studies

The localization of pituitary adenylate cyclase-activating polypeptide (PACAP) in the hypothalamus-pituitary system in rats was examined in light and electron microscopic immunocytochemistry using a specific antiserum to synthetic PACAP 1–38 (R0831). In light microscopic study, intensely PACAP-immunostained perikarya were observed in the supraoptic and paraventricular magnocellular nucleus in the hypothalamus. In the median eminence, many immunoreactive nerve fibers were observed in the internal layer, but a few immunoreactive terminals were noticed in the external layer. In the pituitary gland, numerous immunoreactive nerve fibers were observed in the posterior lobe. In the intermediate lobe, moderately immunostained cells were observed, but in the anterior lobe no immunostained cells were noticed. In electron microscopic study, PACAP-immunoreactivity was examined by avidin-biotin peroxidase complex method. In the perikarya of the supraoptic and paraventricular magnocellular nucleus, DAB-reaction products were distributed diffusely in the cytoplasmic matrix, frequently attaching to the rough-surfaced endoplasmic reticulum. In the nerve terminals of the posterior lobe, reaction products were observed among the secretory granules, but sometimes upon them. In the cells of the intermediate lobe, reaction products were also distributed in the cytoplasmic matrix.     

永瓣藤化学成分的研究

永瓣藤化学成分的研究陈玲，谢平，戚薇，曾菊珍（药学系中药化学教研室）关键词永瓣藤，化学成分，三萜，蒜盘子甾酮永瓣藤（ＭｏｎｉｍｏｐｅｔａｌｎｍＣｈｉｎｅｎｓｅＲｅ－ｈｄ）系卫矛科永瓣藤属植物［１］，为我国特有的珍稀二类保护植物［２］，分布于江西东北部...     

Detection of circulating antigen by McAb-AST for evaluating the efficacy of anti-Leishmania chemotherapy.

We have adapted the simple and sensitive McAb-antigen spot test (AST) for evaluating the efficacy of anti-Leishmania chemotherapy. Serum samples from 37 kala-azar patients were tested by McAb-AST, and all showed definite positive reactions before treatment. After a course of antimony treatment, 20 turned negative, coupled with the disappearance of clinical symptoms; another 12 cases responded with weak positivity accompanied by an improvement of clinical manifestations; and the remaining 5 antimony-resistant patients showed strong positive reactions, with their conditions gradually worsening. Furthermore, all 6 cases in which the diagnosis was missed by the bone marrow smear method turned McAb-AST negative after chemotherapy. These results suggest that McAb-AST can be used to evaluate the efficacy of chemotherapy as well as to avoid missed diagnosis by the bone marrow smear method.     

Mitogenic, chemotactic, and synthetic responses of rat periodontal ligament fibroblastic cells to polypeptide growth factors in vitro.

The mitogenic, chemotactic, and synthetic responses of rat periodontal ligament (PDL) fibroblastic cells to epidermal growth factor (EGF), transforming growth factor-beta (TGF-beta), recombinant human platelet-derived growth factor (rhPDGF)-AB, rhPDGF-BB, natural (n) PDGF-AB, and insulin-like growth factor-I (IGF-I) were examined in vitro using PDL cells obtained from the coagulum of healing tooth sockets. PDGFs and IGF-I have potent and comparable mitogenic effects on PDL fibroblastic cells. The maximum mitogenic effect of PDGFs was observed at the concentration of 10 ng/ml, whereas that of IGF-I was seen at concentrations higher than 100 ng/ml. In contrast, EGF induced moderate, and TGF-beta inhibitory mitogenic responses. The combination of rhPDGF-AB with either EGF or TGF-beta demonstrated comparable mitogenic potency, equivalent to the level of PDGF alone regardless of the mitogenic effect of other growth factors. The combination of rhPDGF-AB and IGF-I, however, showed a synergistic effect revealing the highest mitogenic effect among all individual growth factors as well as any combinations of the growth factors tested. Similarly, PDL fibroblastic cells demonstrated strong chemotactic responses to both IGF-I and PDGFs. The maximum effect was observed by IGF-I at concentrations higher than 10 ng/ml, followed by rhPDGF-BB at 0.1 ng/ml, rhPDGF-AB and nPDGF at concentrations ranging from 0.1 to 1 ng/ml. TGF-beta revealed no, and EGF slightly increased, chemotactic effects. IGF-I slightly enhanced the synthesis of total protein, whereas other factors had no significant effect. However, both rhPDGF-AB and TGF-beta stimulated collagen synthesis. On the other hand, IGF-I showed no effect on collagen synthesis, while EGF suppressed collagen synthesis. These findings suggest that rhPDGF-BB and IGF-I stimulate proliferation and chemotaxis of PDL fibroblastic cells. In addition, the combination of these growth factors further increases the mitogenic effect. rhPDGF-AB also stimulates collagen synthesis by PDL fibroblastic cells. Thus, rhPDGF-BB and IGF-I may have important roles in promotion of PDL healing, and consequently, may be useful for clinical application in periodontal regenerative procedures.     

The Canadian Psychiatric Association practice profile survey: I. Methods and general sample characteristics.

OBJECTIVE: To describe the rationale, methodology, and general sample characteristics of the Canadian Psychiatric Association (CPA) practice profile survey, a national survey of psychiatrists and psychiatric practice. METHOD: Mail-in interviews were sent to all Canadian psychiatrists listed in their provincial registers and to all active CPA members (total = 3628). Respondents provided general information about their professional activities for one 24-hour day and detailed information for 1 randomly selected hour. Patient information--including sociodemographics, diagnostic profiles, functioning levels, risk of harm to self or others, and disposition--was elicited for 1 patient seen during the random hour as well as for the most seriously ill patient receiving clinical services that day. RESULTS: There was a 45.5% response rate. Questionnaires completed by nonpsychiatrists or with a large percentage of missing or incorrect data were eliminated (107 surveys), resulting in a final sample size of 1570. CPA members and those from Western Canada responded at a higher rate to the survey. The results suggest some cause for concern about future manpower shortages. Most psychiatrists practise eclectically, seeing patients across the life-span, and working in both community and institutional settings. The old and the young appear to be underserviced, compared with adults. CONCLUSIONS: This study represents an important step forward in evaluating the profile and activities of the profession.