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In recent years, an increasing number of inherited diseases in man have been recognized in which there is an impairment in the peroxisomal β-oxidation of very-long-chain fatty acids. In general, these disorders are associated with severe neurological and physical abnormalities and death within the first years of life. In this paper we describe our experience with regard to the prenatal diagnosis of a number of different inborn errors of peroxisomal β-oxidation. Eleven pregnancies at risk were monitored by measuring very-long-chain fatty acid levels as well as very-long-chain fatty acid β-oxidation in cultured chorionic villous fibroblasts and/or amniotic fluid cells. Five affected fetuses were identified. It is concluded that prenatal diagnosis in this group of diseases can be done reliably using cultured chorionic villous fibroblasts or amniotic fluid cells.  相似文献   
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Online searching in publically available patent files opens up interesting possibilities to provide a rapid response to critical questions. A computerized analysis of all patents of leading German pharmaceutical companies over the last decade in important indication areas is described. Supported by subsequent manual processing of individual patents it is shown that duplicate experiments on animals practically never occur.  相似文献   
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Pesticide use in agriculture can cause undesirable effects on humans and the natural environment. One of the objectives of integrated agriculture is the elimination or reduction of possible sources of environmental pollution such as pesticides. To achieve this objective, farmers need a method to assist them in estimating the environmental impact of pesticide use. This paper addresses a two-part question: what factors should be taken into consideration to assess pesticide environmental impact, and how can impact be quantified? As the environmental impact of a pesticide depends on its dispersion in the environment and on its toxicological properties, the literature on these topics is reviewed to address the first part of the question. To address the second part of the question, six recent approaches to assess the impact of pesticides on the environment are compared regarding choice, transformation and aggregation of input parameters. The use of simulation models to assess environmental impact is discussed.  相似文献   
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A fetus with multiple structural defects was seen at prenatal ultrasound examination. After termination of the pregnancy a bilateral cleft lip, alveolus, and palate; micrognathia; and webbed joints were seen. Fetal tissues showed indications of infection, intranuclear inclusion bodies, chronic stress, haemolysis, arterial wall damage, and profuse haemorrhage. Parvovirus B19 DNA was detected in fetal tissues by dot hybridization after polymerase chain reaction. The possibility of parvovirus B19 infection leading to congenital malformations is discussed.  相似文献   
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Two types of fermented organic waste (trade and industry waste and fruit and vegetable waste) were successfully used as a sole carbon source to produce poly-3-hydroxybutyrate-co-valerate (PHBV) by Ralstonia eutrophus (formerly Alcaligenes eutrophus) via oxygen limitation.The production of PHBV could be optimized by optimizing the oxygen transfer through the fermentor. Thereby, a peak concentration of 1.1 g PHBV per liter cell suspension, 40 w% of cell dry weight, was obtained at an aeration rate of 0.24 mol O2/h·kg biomass. The yield of PHBV on the fatty acid concentration in the organic waste was 0.16 g polymer/g volatile organic matter. The process obtained, compares well with the commercial production process of PHBV based on glucose.  相似文献   
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