Duchenne型肌营养不良症

作者综述了１０年来对Ｄｕｃｈｅｎｎｅ型肌营养不良症（ＤＭＤ）的研究概况。主要包括①ＤＭＤ的临床研究。②血清生化研究表明ＣＫ、ＬＤＨ、Ｍｂ是诊断ＤＭＤ病人和携带者的敏感指标。③心脏无创性检测和肌肉超微结构研究。④部分抗肌萎缩蛋白基因ＹＡＣ物理图谱，精细限制酶图谱和缺失热区的核苷酸顺序分析，首次发现内含子中ＡＴ富集区的同源顺序与ＤＭＤ断裂有关。⑤抗肌萎缩蛋白的缺失热区疏水肽段存在与否与ＤＭＤ发病密切相关。     

三种方案治疗小儿肺炎的药物经济学研究

目的：比较三种方案治疗小儿细菌性肺炎的效率（即费用－效果）。方法：2001年－2002年采用前瞻性随性临床试验A组50例，B组50例，C组50例作为分析模型，以深圳市二级甲等医院收费为标准，采用决策树的分析方法，进行费用一效果分析。结果：每治疗1例有效病人，A、B、C三组方案的期望成本分别为4707．45元，3052．51元，结论：同时考虑临床疗效及医疗费用，C组有最好的费用－效果，值得临床推广应用。     

Mass Screening for Neuroblastoma and Estimation of Costs

ABSTRACT. On the basis of epidemiological data and medical costs for patients with neuroblastoma, we have calculated the cost of mass screening for neuroblastoma with high performance liquid chromatography (HPLC) compared to the cost when it is not performed. If the sensitivity of the mass screening is 80 % and 22 000 infants are screened annually the cost will be 27809000 yen ($191800). If mass screening is not performed, the cost will be 28 446 000 yen ($196 200). The difference in cost (637 000 yen or $4 400) is fairly small. If the sensitivity is 75 % and 16 500 infants are screened, the difference is also small (174000 yen or $1 200). Therefore, mass screening with the HPLC method will not be an undue financial burden. But re-screening at an older age will be done with less financially favorable results, considering that the sensitivity may not be as high as that of the first screening and that mothers are somewhat reluctant about re-screening. The balance of the cost of mass screening by qualitative methods may also be less favorable, since the detection rate is low.     

Aza-peptides. III. Experimental structural analysis of aza-alanme and aza-asparagine-containing peptides

To determine the structural perturbations induced by the CαH→Nα exchange in aza-peptides, we have examined by H NMR and IR spectroscopy various derivatives of the aza-analogues of alanine, aspartic acid and asparagine in different organic solvents with increasing polarity. Their general formulas are: R'-AzXaa-NR²R³, R'-Pro-AzXaa-NR²R³ and R-AzXaa-Pro-NR²R³ (where AzXaa denotes the aza-analogue of the amino acid residue Xaa = Ala, Asp, Asn; R = Boc, Z; R², R³= H, Me, iPr). The aza-analogue of an amino acid residue appears to be a strong p-turn-inducing motif, and the AzAsn carboxamide side-chain is capable of interacting, as a proton donor, with the preceding peptide carbonyl group.     

Predicting spontaneous early neurological recovery after acute ischemic stroke

We assessed predictors of spontaneous early neurological recovery after acute ischemic stroke by means of multivariate analysis in a cohort of 1,473 consecutive patients treated at one academic center. At hospital discharge, spontaneous neurological improvement or good outcome was defined as grades 0-2 of the Rankin scale, and poor outcome (no improvement or in-hospital death) as grades 3-5. Spontaneous recovery of neurological deficit at the time of discharge from the hospital was observed in 16% of patients with cerebral infarction (n = 238). Dysarthria-clumsy hand syndrome improved in 44% of patients and was the only variable significantly associated with in-hospital functional recovery in three logistic regression models that in addition to lacunar syndromes, included demographic variables, cardiovascular risk factors, and clinical variables [odds ratio (OR) 2.56], neuroimaging findings (OR 2.48), and outcome data (OR 2.39), respectively. Clinical factors related to severity of infarction available at stroke onset have a predominant influence upon in-hospital outcome and may help clinicians to assess prognosis more accurately. Our work gives a contribution into prognostic factors after acute ischemic stroke. With regard to patterns of stroke, dysarthria-clumsy hand syndrome was a significant predictor of spontaneous in-hospital recovery in ischemic stroke patients.     

Length of hospital stay and associated costs for olanzapine vs. haloperidol in the treatment of schizophrenia relapse in Germany

We examined the number of days spent in hospital due to a relapse of schizophrenia and the associated costs for patients treated with olanzapine or haloperidol. Twenty-one German psychiatric hospitals participated in this retrospective study. Data on the last hospitalisation following a relapse of schizophrenia were documented for equal numbers of patients on olanzapine and haloperidol. Matching for time since diagnosis and severity of symptoms was performed. Data were collected on 136 matched pairs. Total length of time spent in hospital was the same on average for patients in both groups (median about 5 weeks), but olanzapine patients spent nearly 1 week less in the in-patient setting than haloperidol patients, resulting in a saving of Euro 411 per patient. Our findings are consistent with those of randomised clinical trials in concluding that olanzapine is preferable to haloperidol in terms of the direct cost of treating schizophrenia. Andrea Spannheimer Kendle GmbH & Co. GMI KG, Stefan-George-Ring 6, 81929 Munich, Germany, e-mail: spannheimer.andrea@kendle.com     

上腹器官簇移植术实验中血流动力学及血生化的变化

目的:观察上腹器官簇移植术实验中血流动力学及血生化变化。方法:杂种猪32只。随机分为供体组和受体组,无肝期行体外静脉转流。测定术中的血流动力学和血生化指标。结果:(1)术中血流动力学在无肝期初期及开放后早期时间内有明显的波动,心输出量、血压、肺动脉压和肺毛细血管楔压下降,心率加快;(2)转流在一定程度上稳定血流动力学;(3)生化变化:开放前行肝冲洗的8例,血钾仅轻度升高;未作冲洗的6例,则血钾明显升高;(4)开放后均有不同程度的代谢性酸中毒。结论:上腹器官簇移植术,机体血流动力学及血生化均有改变,特别是在肝移植阶段明显。其它器官移植期处理也应考虑肝移植后续作用的影响。     

The weighted rank pairwise correlation statistic for linkage analysis: Simulation study and application to Alzheimer's disease

The weighted rank pairwise correlation (WRPC) statistic has been proposed as a robust test of genetic linkage, particularly adapted to the analysis of large and complex pedigrees and for age-dependent and heterogeneous diseases. In this paper a simulation study is presented. Validity and power of the WRPC test are studied and compared to the Haseman-Elston sibpair method for various types of problems. The power of the WRPC test is slightly lower than the Haseman-Elston method for analyzing a large number of small randomly chosen pedigrees. It is higher however in presence of genetic heterogeneity or for analyzing large individual pedigrees. Recently, evidence of linkage of Alzheimer's disease with a locus on chromosome 14, D14s43, has been obtained by the Lod-score method. We reanalyze these data using the WRPC test, essentially confirming the results of the Lod-score method. The WRPC test statistic is higher than the equivalent Lod-score statistic for the two pedigrees which show strong evidence of linkage with the two methods. The global WRPC test statistic is slightly lower than the Lod-score test statistic. The WRPC test, however, makes no hypothesis of a specific genetic transmission model and can be computed very quickly; in addition, an exact P-value can be computed by simulation for individual pedigrees. © 1994 Wiley-Liss, Inc.     

Clinical experience with minimal flow xenon anesthesia

Xenon is a more potent anesthetic than nitrous oxide, and gives more profound analgesia. This investigation was performed to assess the potential of xenon for becoming an anesthetic inspite of its high manufacturing cost. Seven ASA I—-II patients undergoing cholecystectomy (n = 4), hernia repair (n = 2), or mammoplasty (n=l) were studied. Denitrogenation by 15–20 min of oxygen breathing under propofol anesthesia was followed by fentanyl–supplemented xenon anesthesia administered via an automatic minimal flow system which held the oxygen concentration at 30%. Xenon anesthesia lasted 76–228 min and 8–14 1 of xenon (ATPD) was used, of which 5.6–8.1 1 was expended during the first 15 min. Anesthesia appeared to be satisfactory, and the patients woke up rapidly after xenon was discontinued. The automatic system made minimal flow xenon anesthesia easy to administer, but nitrogen accumulation is still a problem. Assuming a xenon price of 10 US $ per litre, the average cost for xenon was about 65 US $ for the first 15 min and then about 25 USS for each subsequent hour of anesthesia.     

亚胺培南/西司他丁与头孢他啶治疗中、重度细菌感染疗效费用分析比较研究

对我院４５例确诊为中、重度细菌感染住院患者进行了亚胺培南／西司他丁与头孢他啶疗效费用分析比较研究。结果表明：２组病例有效率、死亡率无显著性差异；头孢他啶组较亚胺培南／西司他丁组疗程明显延长。亚胺培南／西司他丁每日所需费用明显高于头孢他啶；治疗结束时，前者全部费用并未超过后者；全部住院费用无明显差异。作者认为：决定２种药物全部费用的因素，除与药物单价和每日费用有关，还与药物疗程密切相关。选用药物抗菌作用越强，用药时间即相应缩短，住院时间必然缩短；最终患者住院费用降低