遗传性凝血因子Ⅶ缺陷症家系基因型与表型分析

目的研究遗传性凝血因子Ⅶ缺陷症家系基因型与表型关系。方法对一个遗传性凝血因子缺陷症家系成员的因子Ⅶ(FⅦ)和其他凝血因子活性进行检测,用PCR及DNA序列检测因子Ⅶ基因缺陷,并分析临床出血状况。结果家系中先证者FⅦ基因中位于第8号外显子上发现一个纯合的碱基突变:10827C→T,导致Arg(CGG)304Trp(TGG)氨基酸替换。其弟弟妹妹均有此纯合的碱基突变,且均伴有因子Ⅹ活性(FⅩ:C)升高。先证者和其弟弟妹妹临床均无出血症状。结论先天性因子Ⅶ缺乏是否发生临床出血可能与FX:C水平有关。     

电子病案首页质量缺陷分析与对策

我院病案室在实施电子病案管理的一年多时间中,通过建立网上首页质量控制,从中发现电子病案首页的质量缺陷问题,分析了问题的原因所在,制定了六项主动控制对策,并通过实践取得了较好的电子病案首页管理成效。     

Efficacy of consensus interferon in the treatment of chronic hepatitis C

BACKGROUND AND AIMS: Consensus interferon (CIFN) is a newly developed type I interferon. The aim of this study was to investigate the safety and efficacy of CIFN in the treatment of patients with chronic hepatitis C and to determine the predictors for sustained response. METHODS: Patients were randomized to receive 3 micrograms or 9 micrograms CIFN three times a week for 24 weeks, followed by 24 weeks of observation. Efficacy was assessed by normalization of serum transaminase levels and disappearance of serum hepatitis C virus (HCV)-RNA at the end of treatment and at 24 weeks after stopping treatment. Histologic response was defined as a decrease of at least two points in the Knodell necroinflammatory score at week 48 and was compared with baseline. RESULTS: There were no serious adverse effects related to CIFN therapy. Overall, 44% of patients receiving 3 micrograms and 48% of patients receiving 9 micrograms had normalization of serum transaminase levels and disappearance of HCV viremia at the end of treatment. At 24 weeks after stopping treatment, 16% of patients in receiving 9 micrograms and 12% of patients receiving 3 micrograms had sustained responses. The histologic responses in patients receiving 9 micrograms and those receiving 3 micrograms were 60% and 36%, respectively. The necroinflammatory score was significantly reduced from baseline to week 48 in both groups. In addition, bodyweight < 60 kg and pretreatment serum HCV-RNA level < 0.5 MEq/mL can serve as predictors for sustained response to CIFN treatment. CONCLUSIONS: These findings suggest that 9 micrograms CIFN is safe and effective in the treatment of patients with chronic hepatitis C.     

Gastrointestinal metastasis in hepatocellular carcinoma: radiological and endoscopic studies of 11 cases

BACKGROUND: From October 1990 to January 1999, 11 of 2237 hepatoma patients at our hospital had gastrointestinal tract metastasis. We describe the radiological and endoscopic features, clinical course and prognoses of those patients. METHODS: All patients were male. Six patients were hepatitis B carriers, and cirrhosis was noted in eight patients on admission. We reviewed all available radiological, endoscopic and pathological features. RESULTS: The commonest clinical presentation was frank gastrointestinal bleeding. Histological proof of gastrointestinal involvement was seen in six patients. Endoscopic features included ulcerative tumours mimicking advanced gastric carcinoma (43%) and submucosal tumours (29%). The sites of organ involvement were stomach (five), duodenum (two), colon (three) and duodenum and colon (one). Direct invasion by a contiguous neoplasm was the major route of gastrointestinal tract metastasis. Portal vein thrombosis may be the key point of haematogenous spread to other sites. CONCLUSIONS: The prognosis in these patients was extremely poor. Almost all patients died within 5 months if no further aggressive management was performed. Surgical intervention may be the optimal choice for palliative treatment of HCC with gastrointestinal tract involvement.     

民国中医期刊的史学与文献价值(续完)——广州现存广东期刊调查

民国中医期刊保存了许多没有以单行本出版的论著和医案集。它们是中医文库和中医学术研究中被忽略的一部分。在民国广东中医期刊所刊载的3246篇文献中，共有240种、1005篇文献为长篇或短篇连载。这些文献是研究民国时期医学的宝贵资料。     

妇康片治疗子宫内膜单纯性增生的临床分析

目的观察子宫内膜单纯性增生患者服用妇康片前后子宫内膜雌激素受体(estrogen receptor,ER)、孕激素受体(progesterone receptor,PR)与增殖细胞核抗原(proliferative cell nuclear antigen,PCNA)表达的变化,为临床治疗提供理论依据。方法病理诊断子宫内膜单纯性增生患者30例,分段诊刮术后第5天及下两次月经周期第5天开始口服妇康片,5mg/d,连服22d,共3个月经周期,于第3个月经周期的第25~28天取子宫内膜组织。用免疫组织化学染色法观察治疗前后雌激素受体、孕激素受体、增殖细胞核抗原的表达变化。结果口服妇康片治疗3个月经周期后,患者月经周期缩短,经量减少。子宫内膜单纯性增生治疗前ER、PR与PCNA的表达分别为3.33±1.92、4.13±1.55和4.40±1.54,经口服妇康片治疗3个周期后ER、PR和PCNA的表达分别为1.97±1.87、1.80±1.75和1.83±1.00。与治疗前ER、PR和PCNA的表达比较,差异有统计学意义(P<0.05)。结论妇康片可抑制子宫内膜细胞增殖活性,降低ER、PR和PCNA表达,可有效治疗子宫内膜单纯性增生。     

Adjuvant chemotherapy after radical hysterectomy for cervical carcinoma

Three hundred and sixty-eight cases of invasive cervical cancer (stage IB through early stage IIB) were treated with radical abdominal hysterectomy and bilateral pelvic lymphadenectomy at Chang Gung Memorial Hospital. Of these patients, 172 were classified postoperatively as a high-risk group after surgical-pathological assessment of tumor extent. Among these high-risk patients, 40 received adjuvant chemotherapy with cisplatin, vinblastine, and bleomycin (PVB), 38 received adjuvant radiotherapy, and 79 refused adjuvant treatment. The 3-year cumulative disease-free survival rate was 91.6% for the low-risk group and 59.7% for those at high risk. Among patients in the high-risk group, the 3-year survival rate was 75.0% for patients treated with adjuvant chemotherapy and 46.8% for those not treated with adjuvant therapy (P less than 0.05). The preliminary results of this pilot study showed a significant activity of adjuvant chemotherapy, which warrants further investigation of its role in the treatment of cervical cancer.     

土鳖虫对大鼠血液流变学的影响

土鳖虫提取液连续给大鼠灌胃１０ｄ，可使血球压积，全血高切粘度，全血低切粘度，ＲＢＣ聚集指数，ＲＢＣ刚性指数均明显降低。使ＲＢＣ沉降率，血沉方程常数明显升高。对血浆粘度，纤维蛋白原含量无影响。说明土鳖虫主要是通过影响ＲＢＣ发挥降低血液粘度作用的。     

Transabdominal chorionic villus sampling: fetal loss rate in relation to maternal and gestational age.

In this paper we report the fetal loss rate in relation to both maternal and gestational age in 1764 pregnant women who underwent transabdominal chorionic villus sampling (TA-CVS) between January 1986 and August 1990. The fetal loss rate, considered as a proportion of continuing pregnancies, decreased with advancing gestational age at sampling from 4.3 per cent before 9 weeks to 0.4 per cent at or after 13 weeks, the difference being statistically significant (p < 0.025). The fetal loss rate increased from 1.6 per cent in women under 30 to 2.4 per cent in women of 40 years or over, but the difference was not statistically significant. Considering that the total fetal loss rate before 28 weeks' gestation was on average 1.91 per cent (1.3 per cent under 35 years and 2.8 per cent in women of 35 or over), we believe that TA-CVS is a safe and effective technique for prenatal diagnosis of genetic diseases.     

In utero intussusception producing ileal atresia and meconium peritonitis with and without free air

Two cases of intestinal atresia due to in utero intussusception are reported. Meconium peritonitis occurred in both cases and in one free air in the peritoneal cavity was also found. Both were managed successfully by resection and end-to-end anastomosis. The intussusceptions were discovered on gross and microscopic examination of the distal ileal segment. These two cases and 33 from the literature are reviewed to determine major features of this seemingly unusual occurrence. The atresia was limited to the ileum and jejunum in all cases and involved the ileum in 77%. The atresia was limited to the gap and fibrous-connecting-cord types in all cases, with the gap type slightly more frequent (57%). In all but 1 of the 35 cases (97%), the intussusceptum was found in the small-bowel segment distal to the atresia; in only 2 were free air and calcium deposits identified at birth on X-ray studies of the abdomen. Prematurity was encountered in only 14% of the 35 cases, suggesting that intussusceptum-induced midgut atresia occurs late in pregnancy. The occurrence of two cases in 2 years in Torrance, California, and three cases in 3 years in Helsinki, Finland, suggest that this occurrence must be more frequent than the sparse incidence reported in the literature would indicate. It is suggested that careful gross and microscopic examination of the small-bowel segment just distal to the atresia be carried out in all cases of jejunoileal atresia of gap or cord types to determine the true incidence and clinical nature of this type of in utero intussusception-induced jejunoileal atresia.