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We report a 20 month old female patient with diploid-triploid mixoploidy (46,XX/69,XXX) syndrome with hypothyroidism and precocious puberty. The triploid cell line was only expressed in the fibroblast culture and comprised the majority (95%) of the cells. Chromosome analysis of the fetal blood sample and peripheral blood sample were normal. The patient shows typical features of full triploidy (growth and severe mental retardation, cranial and facial dysmorphism, complete syndactyly of fingers 3/4, partial syndactyly of toes 2/3) and facial but no body asymmetry. At the age of 5 months central hypothyroidism and precocious puberty were diagnosed. Thin pigmented streaks were visible on the wrists and legs of the patient at the age of 16 months. This is the first patient reported so far with 46,XX/69,XXX mixoploidy suffering from hypothyroidism and precocious puberty. 相似文献
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This report contains the experience of our centre, using the transvaginally guided puncture procedure, to reduce the number of fetuses in a multifetal pregnancy to a lower number. The aim of the procedure was to improve perinatal outcome and/or to meet the personal desires of patients and their families. We surveyed 148 multifetal pregnancy reductions. The fetus or fetuses overlying the internal os was most commonly reduced. The total uncorrected loss of the entire pregnancy was 13.4%. The corrected pregnancy loss was 11%. Of the 63 twins left after the reduction, 33 delivered preterm. Of the 36 singletons, two delivered preterm. Our conclusion was that multifetal pregnancy reduction is a safe procedure for the mother and has an acceptable loss rate of the entire pregnancy. The reduction of a fetus overlying the internal os by the transvaginal puncture procedure seems to yield results at least as good as the transabdominally performed puncture procedures for multifetal pregnancy reduction. 相似文献
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Decentralised systems have the potential to provide a viable option for long term sustainable management of household wastewater. Yet, at present, such systems hold an uncertain status and are frequently omitted from consideration. Their potential can only be realised with improved approaches to their management, and improved methods to decision-making in planning of wastewater systems. The aim of this paper is to demonstrate the value of a novel framework to guide the planning of decentralised systems so that asset management and risk management are explicitly considered. The framework was developed through a detailed synthesis of literature and practice in the area of asset management of centralised water and wastewater systems, and risk management in the context of decentralised systems. Key aspects of the framework are attention to socio-economic risks as well as engineering, public health and ecological risks, the central place of communication with multiple stakeholders and establishing a shared asset information system. A case study is used to demonstrate how the framework can guide a different approach and lead to different, more sustainable outcomes, by explicitly considering the needs and perspectives of homeowners, water authorities, relevant government agencies and society as a whole. 相似文献
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TV Martyniuk IE Chazova VP Masenko VN Volkov IuN Belenkov 《Canadian Metallurgical Quarterly》1998,70(4):33-36
AIM: Assessment of RAAS and vasopressin in patients with primary pulmonary hypertension (PPH). MATERIALS AND METHODS: Activity of plasma renin (APR), angiotensin-converting enzyme (ACE), plasma levels of angiotensin II (AII) and vasopressin (VP), serum concentration of aldosteron (AS) were measured by radioimmunoassay and enzyme immunoassay in 21 PPH patients with circulatory failure (age 34.7 +/- 2.1 years), 11 patients with NYHA functional class II-III, 10 with class IV, and 10 control subjects (age 29.8 +/- 1.5 years). RESULTS: Compared to controls, 21 PPH patients had elevated RAAS parameters: APR up to 3.52 ng/ml/h (p < 0.05), activity of ACE up to 43.13 units, AII level up to 33.93 ng/ml (p < 0.01), AS up to 468.86 ng/ml (p < 0.01), VP up to 5.26 ng/ml (p < 0.001). Circulatory failure progression resulted in activation of all the RAAS components. This and VP activation was the greatest in PPH patients with ACE > 5 ng/ml/h. PPH patients with mean pressure in the pulmonary artery higher than 60 mm Hg demonstrated higher ARP, AS, VP, AII, ACE than those who had this pressure under 60 mm Hg. CONCLUSION: PPH patients display a noticeable activation of RAAS and VP. This activation seems to be secondary as the changes increase with elevation of the pressure in the pulmonary artery and aggravation of circulatory insufficiency. Plasma renin activity determines the degree of RAAS activation as a whole. The discovered activation of RAAS in PPH gives grounds for doubts in the validity of using ACE inhibitors in the treatment of PPH. 相似文献
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A detailed comparison of experimental and theoretical SNR in an IR laser heterodyne system has been made with three different signal analyzers. Good agreement, considerably better than a factor of 1.5, is reported. Accurate allowance was made for transmission in the receiver optics, the effective quantum efficiency of the detector due to shot noise domination by the local oscillator, and for coherent speckle effects across the collection aperture. The evaluation of SNR with a surface acoustic wave spectrum analyzer and digital integrator is described in some detail. As an illustration an absolute measurement of backscattering strength in the atmosphere from an airborne equipment at altitudes up to 13.1 km is provided. 相似文献
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D-STATCOM不平衡负荷补偿电流的优化设计 总被引:7,自引:2,他引:7
在介绍不平衡负荷的电纳补偿原理的同时,分析了其不足之处,即在负荷严重不平衡的情况 下,三相补偿装置分担的负荷不均衡,限制了装置的补偿能力,也容易导致一相或两相过流;针对不 平衡负荷补偿的特殊工况,提出了补偿电流优化设计的目标,即三相电流大小均衡。文中给出了补 偿电流优化设计的理论依据和前提条件:首先,通过选择合适的电路连接方式,电流是多样可选择 的;从数学上证明了提出的优化设计目标的合理性,并给出了优化补偿电流的计算方法。通过 MATLAB计算,与传统补偿思路进行了基波电流、电容电压波动等多项指标的比较,证明优化设 计原理在各项指标上部具有明显的优势。PSCAD仿真和10 kVA样机实验结果表明,优化设计原 理可以实现且效果良好。 相似文献