Clinical characteristics of rapidly progressive leuko-araiosis

Introduction – 38 patients found to have either pure leuko-araiosis (LA) or LA combined with infarction(s) on computer tomography (CT) in 1989 were re-examined in 1992 in order to evaluate the progression of LA. The follow-up period averaged 3.2 years. Material and methods - The clinical and radiological data on patients in 1989 were collected from hospital records and re-evaluated. The patients were re-examined clinically (including 24 hour ambulatory blood pressure measurement), and neuroradiologically (CT) in 1992 for this study. Results – 11 (29%) patients were found to have significant (rapid) progression of the extent of LA on CT during the follow-up. At baseline, there was no significant difference in the mean number of brain infarctions between the groups with progressing (prLA) and non-progressing LA (nprLA) or between the number of cortical and central infarctions within these groups. At follow-up, the total number of infarctions had increased significantly in both groups, but it was mostly because of the increase in cortical infarctions in the prLA group (p = 0.043) and, conversely, the central ones in the nprLA group (p = 0.011). prLA was found to be related to heart failure (82% vs 37%, p = 0.029) and atrial fibrillation (55% vs 19%, p = 0.047), whereas nprLA was strongly associated with a sudden onset of symptoms (78% vs prLA 18%, p = 0.001) like a-true brain infarction. Other clinical factors, including mean blood pressure and heart rate, did not clearly differentiate between the groups. Conclusion - The results suggest that there are different subgroups of patients with LA associated with various vascular factors. The occurrence of LA is not related to the distribution of infarctions. The progression of LA is not related to the number of brain infarctions or to the simultaneous increase of infarctions on CT.     

预防肝硬化上消化道出血的临床研究

目的　探讨普萘洛尔和低剂量兰索拉唑长期维持治疗对预防肝硬化门脉高压消化道出血的疗效。方法　1 1 9例肝硬化门脉高压患者随机分为 3组 :Ⅰ组 :给予口服普萘洛尔加上护肝治疗。Ⅱ组 :联合给予低剂量兰索拉唑和普萘洛尔。Ⅲ组 :仅给予护肝治疗。兰索拉唑维持服药 6个月 ,普萘洛尔及一般对症治疗维持 1年。观察治疗前后各组患者所伴发溃疡、门脉高压性胃病 (PHG)、急性胃黏膜病变 (AGML)情况 (发生率 ) ,各组消化道出血的复发率、门静脉直径 (PVD)、脾静脉直径 (SVD)的变化。结果　经 1年观察 ,结果显示 ,普萘洛尔组、联合治疗组、对照组出血的复发率分别为 1 5 0 %、2 2 %、48 5 % ,组Ⅰ、组Ⅱ复发率显著低于组Ⅲ ,同时 ,组Ⅰ与组Ⅱ间的差异有显著性意义。治疗可见组Ⅰ、组Ⅱ患者所伴发的溃疡、PHG、AGML明显改善 ,PVD、SVD缩小。结论　普萘洛尔组联合抑制酸维持治疗 ,可预防引起消化道出血多种病因 ,较单用普萘洛尔的疗效好     

Quality of life research in patients with rectal cancer: traditional approaches versus a problem-solving oriented perspective

The present paper critically appraises two recent overviews of the literature on rectal cancer and quality of life (QL). These reviews focus on the Anglo-American literature, largely neglect research from other countries, and provide little stimulus regarding future research directions. As an alternative perspective we propose the concept of problem-solving oriented QL research. The major theme is that the QL concept must be integrated into the clinical arena. To begin with, QL researchers must make themselves understandable. We outline several ways in which this can be achieved: (a) placing QL in a broader concept together with outcomes that are more familiar to clinicians; (b) depicting individual patients in the form of QL profiles; (c) clarifying the psychosocial/clinical correlates of particular QL scores of interest; and (d) conducting studies with a definitive practical goal in mind and integrating practitioners and patients into the study group. We illustrate the feasibility of such a research program by performance data from our Marburg-Biedenkopf field trial. Pursuing an ambitious research strategy that integrates experimental and applied research, the QL movement will have the chance to show that it is not merely l'art pour l'art, but indeed is beneficial to society. Received: 28 September 1998 / Accepted: 14 October 1998     

嗅沟脑膜瘤术式选择及长期临床预后随访

目的分析嗅沟脑膜瘤的手术治疗和随访结果。方法收集20例嗅沟脑膜瘤手术病例,并分析其临床表现及随访结果。结果嗅沟脑膜瘤大多以精神症状、视觉障碍和嗅觉减退为首发症状,9例行冠状切口,4例行半冠状切口,分块切除肿瘤,5例翼点入路,1例纵裂入路。1例眶上入路,SimpsonI级切除6例。SimpsonⅡ级切除13例,SimpsonⅢ级切除1例,预后良好,1例复发。随访时间1~6年(平均3.1年),术前术后均保留嗅觉的为2例,精神状态改善18(91%)和视力障碍改善的15(75%)。1例(5%)复发再次手术。结论术式的选择取决诸多因素,预后取决于手术中的组织保护。     

Treatment of craniopharyngiomas—the stereotactic approach in a ten to twenty-three years' perspective

Summary Fourty-two consecutive patients with craniopharyngioma were treated by stereotactic approach, i.e. preferentially stereotactic puncture and installation of colloid isotope into cystic tumours and external stereotactic single dose irradiation to solid tumour parts. In a minority of cases, such treatment was less suitable, and surgical removal and/or radiotherapy was used. There was no peroperative mortality. A long-term follow up (observation time 10–23 years) of the 31 patients alive indicated that they were socially well adapted with a high rate of fulltime work and a low rate of intercurrent disease. In spite of substitution therapy for pituitary insufficiency in most cases, the patients were subjectively seldom disturbed by their disease.Our results support a change in the choice of therapy for craniopharyngioma patients, from open neurosurgery to the less invasive stereotactic techniques.     

Incidence of cancer in children born after in-vitro fertilization

Evaluation of the long-term health of children born using in-vitro fertilization (IVF) provides important information to clinicians and consumers. Until very recently, there have been no published data on the incidence of cancer in children conceived as a result of IVF, despite a number of case reports of neuroblastoma in children conceived using fertility drugs. This study used a record-linkage cohort design to investigate the incidence of cancer in children born after IVF. The study included all conceptions using assisted reproductive technologies between 1979 and 1995 at two clinics in Victoria, Australia that resulted in a live birth. Data on births were linked with a population-based cancer registry to determine the number of cases of cancer that occurred. The standardized incidence ratio (SIR) was calculated by comparing the observed number of cases to the expected number of cases. The final cohort included 5249 births. The median length of follow-up was 3 years, 9 months (range 0-15 years). In all, 4.33 cases of cancer were expected and six were observed, giving a SIR of 1.39 (95% CI 0.62-3.09). This study found that children conceived using IVF and related procedures did not have a significantly increased incidence of cancer in comparison to the general population.     

Pregnancy outcome and neonatal data of children born after ICSI using testicular sperm in obstructive and non-obstructive azoospermia

BACKGROUND: Registries on outcome of ICSI pregnancies obtained with testicular sperm do not differentiate between obstructive (OA) and non-obstructive azoospermia (NOA). We evaluated the pregnancy outcome and neonatal data on children born after ICSI using testicular sperm of men with histologically proven OA or NOA. METHODS: Pregnancies obtained after ICSI using testicular sperm of men with defined NOA (n = 70) were compared with those of men with OA (n = 204). RESULTS: Multiple birth rates in NOA and OA couples, respectively, were 21 versus 27% (P = NS), overall preterm delivery rates were 38 versus 26% (NS), and prematurity rates were 24 versus 13% for singletons (NS) and 86 versus 54% for twins (relative risk 1.59, 95% confidence interval 1.04-2.42). Median gestational age for singletons was 38.3 versus 39.3 weeks, respectively (P < 0.05). The low birth weight rates were 34 versus 31%, respectively (NS). The early perinatal mortality rate was 66 versus 15 per 1000 births, respectively, (NS). Major congenital malformations were observed in 4 versus 3%, respectively, of the live born babies (NS). Prenatal karyotypes showed 7% de-novo abnormalities in the NOA group versus 1% in the OA group (NS). CONCLUSIONS: Our data do not show differences between NOA and OA pregnancies except for a strong tendency towards a lower gestational age in singletons and a higher percentage of premature twins in the NOA group. Although our data are based on a limited sample, the differences observed call for further analysis. Given the low pregnancy rates after ICSI with NOA, a multicentre study, differentiating NOA and OA patients, would be recommended.     

Reproductive planning after genetic counselling: a perspective from the last decade

Studies from the last decade on factors influencing reproductive planning after genetic counselling were reviewed. Increased possibilities of DNA-analysis and prenatal diagnosis might have brought about a shift in the paramountcy of factors influencing reproductive planning after genetic counselling. In contrast to the literature in the seventies, the magnitude of the genetic risk was no longer found to be one of the decisive factors in postcounselling reproductive planning. Instead, the interpretation of the risk as high or low and the desire to have children appeared to be paramount. The impact of new developments in DNA-analysis in prenatal diagnosis and presymptomatic testing will be an important subject for future studies on factors influencing reproductive planning.     

Evaluation of genetic counselling: recall of information, post-counselling reproduction, and attitude of the counsellees

Of the families who had received genetic counselling between 1972 and 1981, 791 replied to a questionnaire which covered recall of information, post-counselling reproduction and attitudes towards counselling and prenatal diagnosis. Eighty percent had adequate knowledge of mode of inheritance and 74% of recurrence risk. Knowledge of mode of inheritance was poorest in multifactorial transmission (63%) and knowledge of recurrence risk in X-chromosomal disorders (61%). Forty-five per cent of the families had started a pregnancy after the counselling. 25%). Early lethality of the disorder and feasibility of a prenatal study contributed to positive reproductive decisions. Nine per cent of the children born after the counselling were affected by the disorder in question. The observed risks tended to match well with the expected ones. Sixty-two per cent of the respondents felt that the counselling had had a great or moderate impact on their reproductive plans. Forty-two per cent expressed a wish to hear the counsellor's opinion in addition to the facts. This was more common when the disorder was severe. Although most couples (53%) wished to have a prenatal study, if feasible, and abort an affected foetus, 16% were against abortion in such a case and 31% wished to have the study but were ambiguous about an abortion.     

Sputum microbiome profiles identify severe asthma phenotypes of relative stability at 12 to 18 months

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