Post-mortem analysis for two prevalent β-oxidation mutations in sudden infant death

BACKGROUND: Fatty acid oxidation disorders may cause sudden and unexpected infant death and are associated with the histological hallmark of hepatic steatosis. The goal of the present study was to assess the value of post-mortem molecular analysis for medium-chain acyl-coenzyme A dehydrogenase (MCAD) and mitochondrial trifunctional protein (MTP) defects in unexplained sudden infant death (SID) associated with fatty infiltration of the liver. MCAD catalyzes the first step of medium-chain fatty acid oxidation while MTP catalyzes the last three steps of long-chain fatty acid oxidation. METHODS: In a retrospective study, 220 consecutive cases of sudden and unexplained infant death certified by medical examiners at Wake Forest University Medical Center were assessed for hepatic steatosis. Subjects with evidence of hepatic steatosis were screened for mutations in MCAD and MTPalpha-subunit using DNA isolated from paraffin-embedded liver tissue, single-strand conformation variance, and nucleotide sequence analyses. RESULTS: Sixteen cases (7.3%) were associated with diffuse micro-vesicular or mixed micro- and macro-vesicular hepatic steatosis. Two of these 16 cases (12.5%) had disease-causing mutations. One was homozygous for the prevalent MCAD A985G mutation. The second was a compound heterozygous for the prevalent MTP G1528C mutation and a novel 1 bp deletion in exon 18 of the MTPalpha-subunit gene. CONCLUSIONS: A significant proportion (7.3%) of SID is associated with hepatic steatosis. The present data support post-mortem molecular analysis for the MCAD A985G and MTP G1528C prevalent mutations in cases of sudden and unexplained infant death associated with hepatic steatosis.     

哒嗪与苯、庚氧基取代苯共聚物的合成

1，3-双(二苯基瞵)-丙烷基-二氯化镍存在下，3，6-二氯哒嗪与1，4-二溴苯格氏试剂共聚，庚氧基取代苯与3，6-二氯哒嗪格氏试剂共聚得到了哒嗪类新型共轭共聚物。并用FTIR、NMR对中间体和聚合物的结构进行了表征。采用XRD测试对共聚物结晶性进行了讨论。该合成方法所得的共聚物收率分别为75％和67％。其中含有烷氧基苯单元的共聚物在DMF、DMSO等极性溶剂和有机酸中具有良好的溶解性。共聚物的紫外-可见光谱中，分别在310nm、345nm等处出现最大吸收峰。XRD结果表示共聚物具有一定的结晶性。     

Long-Term Results of Hybrid Therapy in Patients with Atrial Fibrillation Who Develop Atrial Flutter During Flecainide Infusion

The flecainide infusion test has been proposed to screen candidates for hybrid pharmacological and ablation therapy. We report the long-term follow-up of 154 consecutive patients with paroxysmal or persistent atrial fibrillation (AF) who developed atrial flutter (AFL) during flecainide infusion (IC AFL), treated with inferior vena cava-tricuspid annulus isthmus catheter ablation and oral flecainide (hybrid therapy). Over a mean of 54.1 ± 13.1 months 82 patients (53%) remained free of AF and AFL. Flecainide was discontinued because of adverse effects in 6 patients (4%). A history of persistent AF, and the documentation of ≥1 spontaneous AFL episode before the flecainide test were independent predictors of successful hybrid therapy. In patients with paroxysmal AF without documented spontaneous AFL, the long-term efficacy of hybrid therapy was 38.5% (P = 0.03). The flecainide infusion test reliably detects candidates for hybrid therapy. The efficacy of this therapy is maintained over the long-term with a high patient compliance.     

Percutaneous Mitral Commissurotomy in Acute Pulmonary Edema

From January 1994 to July 1998, percutaneous mitral commissurotomy was performed in 520 patients. Of these patients, 7 (4 men and 3 women aged 31 ± 5.6 years) were dilated in an emergency situation of intractable pulmonary edema caused by severe mitral stenosis. Three patients required mechanical ventilatory support. Percutaneous mitral commissurotomy was performed with the Inoue balloon. The dilatation of the valve was undertaken even though the echocardiographic score of the valve was high. Percutaneous mitral commissurotomy resulted in an increase in the mitral valve area from 0.72 ± 0.18 cm² to 1.95 ± 0.18 cm² (P = 0.011) with a concomitant reduction in pulmonary artery systolic pressure from 82.5 ± 16.4 mmHg to 46.7 ± 11.6 mmHg (P = 0.018). One patient died (he had two cardiac arrests before the dilatation). During follow-up (mean 18 months), one patient presented with a restenosis, one an aggravation of mitral insuflciency grade, and four were in NYHA functional Class II. Thus, percutaneous mitral commissurotomy can be considered as a treatment of choice in patients with intractable pulmonary edema caused by severe mitral stenosis.     

AN APPROACH FOR HIGH SENSITIVITY DETECTION OF PROSTATE CANCER BY ANALYSIS OF CHANGES IN STRUCTUREDNESS OF THE CYTOPLASMIC MATRIX OF LYMPHOCYTES SPECIFICALLY INDUCED BY PSA-ACT

PURPOSE: An alternative procedure for detection of prostate cancer was examined based on the observation that cells reexposed in vitro to antigenic or mitogenic stimulation will change their intracellular structuredness as measured by polarization of fluorescent light emitted by labeled cells (SCM test). MATERIALS AND METHODS: Lymphocytes derived from patients bearing a nonmalignant prostate tumor and healthy individuals were exposed to PSA-ACT, PHA, and MUC-1. RESULTS: Of sixty-five patients with prostate carcinoma (CaP), sixty-two were correctly diagnosed by the test. Of the eighty males in the control group, five were incorrectly diagnosed as having the disease and seventy-five were correctly diagnosed as healthy subjects. The sensitivity of the test was 96.8%. The specificity was 91.1%. The BPH (Benign Prostatic Hyperplasia) control group exhibited a sensitivity of 9.38%, but the specificity was 91.1%. Similar percentages for specificity and sensitivity were observed in the NRT (Non-Relevant Tumor) control group. CONCLUSIONS: The results shown here indicate the possibility of a different use of PSA-ACT for detection of prostate cancer with high specificity and sensitivity.     

CUTANEOUS LEISHMANIASIS: SCHEDULES FOR INTRALESIONAL TREATMENT WITH SODIUM STIBOGLUCONATE

Background. Various therapeutic modalities have been used for treating cutaneous leishmaniasis. Intralesional pentavalent antimonial injection is one of the effective therapeutic modalities. In this study, the efficacy of three different intralesional schedules with sodium stibogluconate have been investigated. Methods. Ninety-six patients with 129 lesions were entered into the study, all with confirmed diagnosis of cutaneous leishmaniasis. The lesions were treated with three different schedules, including daily, alternate day, and weekly interlesional injections of sodium stibogluconate. The clinical response was then compared to arrive at the most effective schedule. Results. The clinical responses (complete and partial cure) were 67%, 97%, and 91% for the daily, alternate-day, and weekly schedules, respectively. Lesions with either partial cure or failure of treatment were injected weekly with the same medication until complete cure had been achieved. The final overall success rate of complete cure was 99.2%. Conclusions. Alternate day or weekly intralesional treatment of cutaneous leishmaniasis with sodium stibogluconate are more effective than daily treatments.     

Left Ventricular Perforation During Percutaneous Balloon Mitral Valvuloplasty With Emergency Surgery

Two cases of left ventricular (LV) perforation by balloon catheter following successful balloon mitral valvuloplasty are presented. The first patient was dilated first with a single straight-tip balloon catheter and after with a double balloon catheter. The second patient underwent a valvuloplasty with a double balloon catheter only. The subsequent tamponade was treated by immediate aspiration but without hemodynamic improvement. Both patients required emergency surgery with surgical repair of the LV apex, and both of them could be weaned. Both patients are well 18 months after the procedure. We discuss the mechanism of this serious complication and technical considerations to prevent it.     

2002～2004年乌鲁木齐市天山区结核病控制效果评价

目的评价2002～2004年乌鲁木齐市天山区世界银行贷款/英国政府增款中国结核病控制项目(简称卫X项目)实施效果.方法根据天山区卫X项目实施规划、年度计划、项目季报表及相关资料,分析结核病人登记管理情况.结果 3年共发现涂阳肺结核病人331例,完成3年病人发现计划的108.9%,233例初治涂阳病人治愈率为97.9%,61例复治涂阳病人治愈率为90.2%(2004年第4季度涂阳病人治愈率未作统计),均达到涂阳病人治愈率达85%的项目要求.结论乌鲁木齐市天山区卫X项目取得显著成效,发现和治愈了大量肺结核病人,为天山区结核病控制工作可持续发展提供了宝贵经验.     

In vivo expression of cytokine mRNA in rats infected with Schistosoma mansoni

As an animal model, rat schistosomiasis mansoni has provided considerable knowledge of immune mechanisms involved in the expulsion of worms and in a subsequent development of immunity to reinfection. Although it is clear that ADCC mechanisms participate in immunity to reinfection; the nature of the cytokines involved in immunity is unknown. To analyse the pattern of cytokines involved, the mRNA levels of different cytokines were assessed by RT-PCR as they occur within tissues during the course of infection. In spleens from infected rats, a significant elevation in IL-2 and IL-5 mRNA was observed during the early phase of infection (day 7). Analysis of pulmonary cytokine responses showed a dramatic increase in IL-4 and IL-5 on day 7. This was accompanied with a low but significant increase in IL-2 (day 11) and IL-12 (day 7) in the absence of augmented IFN-γ expression. The cytokine expression patterns of draining lymph nodes (LN) from infected rats showed a significant increase of IL-2, IL-4 and IL-5 on day 21. Analysis of IL-10 expression showed exclusively a significant increase in LN on day 11. IFN-γ mRNA was not detected in any tissue sample. Thus, rats develop a predominately Th2-type cytokine response during a primary infection which may be involved at least in part, in the expression of immunity against Schistosoma mansoni infection .