Diagnosis and prenatal diagnosis of epidermolysis bullosa herpetiformis (Dowling-Meara) in a mother,two affected children,and an affected fetus

In utero skin biopsy was performed on a fetus at risk of an uncertain form of epidermolysis bullosa (EB). The mother had produced two affected offspring diagnosed variously as having junctional or dystrophic EB. The two offspring and the fetus were products of different fathers. The mother claimed to have no disease and on clinical examination was without blisters. Examination of the fetal skin biopsy by light and electron microscopy revealed separation of the epidermal sheet from the majority of the biopsy sample, although occasional remnants of basal cells remained associated with the basement membrane. Aggregations of keratin filaments were observed within basal cells of the detached epidermis and in the attached basal cell remnants. The diagnosis was thus suggested to be epidermolysis bullosa Dowling-Meara. Re-review of the clinical and laboratory data from the affected infants revealed a clinical and histological pattern consistent with this diagnosis. Further discussion with the mother revealed that her skin had blistered as a child and that she presently had hyperkeratotic palms and soles. This history is consistent with the autosomal dominantly inherited epidermolysis bullosa herpetiformis (Dowling-Meara). This is the first reported prenatal diagnosis of EB Dowling—Meara. The morphological criteria of intraepidermal blistering and clumped keratin filaments within basal and immediately suprabasal cells characteristic of an affected individual postnatally also identified an affected fetus. There is, however, insufficient experience to be certain that these findings will hold from region to region in the body or among all affected fetuses, and thus prenatal diagnosis on a morphological basis should still be made with caution.     

天津市节能减排绩效及经济效益协调性研究

利用节能绩效-减排绩效关系图,以及节能绩效、减排绩效与经济效益协调关系三角图,研究了2006-2008年天津市17个区县及滨海新区的节能减排绩效关系及其经济协调性状态和趋势.结果如下:①2006-2008年,天津市17个区县及滨海新区的节能减排绩效整体上较差,河西区、西青区、津南区、北辰区、宝坻区、静海县和蓟县的节能减排绩效呈现变差趋势,东丽区和滨海新区(塘沽区、汉沽区和大港区)节能减排绩效很差且无明显变好趋势;其他6个区县的节能减排绩效呈现变好趋势.②2008年,北辰区、武清区、静海县3个区县的经济协调性很强,河西区、河北区、汉沽区、蓟县处于强不经济协调性状态,滨海新区处于弱不协调性状态;2006年,大部分区县(包括滨海新区)处于弱不经济协调性状态,且按强协调趋势发展,其协调性增强主要来自经济效益驱动.③天津市17个区县及滨海新区的节能减排绩效及经济协调性评估权重选择较为合理,能准确地反映节能减排现状.     

天津市工业节能减排绩效及其与经济效益协调性评估

利用节能绩效-减排绩效关系图,以及节能绩效、减排绩效与经济效益协调关系三角图,研究了2006─2008年天津市18个行业及滨海新区8个企业的节能减排绩效关系及其经济协调性状态和趋势. 结果表明:天津市18个行业节能减排绩效(节能绩效和减排绩效)主要由减排绩效贡献;冶金及相关产业的节能减排绩效很差,化工及相关产业节能减排绩效较差,其他行业节能减排绩效处于一般～好的状态,各行业节能减排绩效无变好的趋势;滨海新区大部分企业的节能减排绩效主要由减排绩效贡献. 2008年,天津市大部分行业节能减排绩效与经济效益协调性较好,仅有化学原料及化学制品制造业、非金属矿物制造业和电力煤气及水的生产和供应业等3个行业处于较不协调状态; 天津市大部分行业按弱协调性趋势发展;滨海新区企业的经济协调性状况堪忧,仅有2个企业处于很强或强协调状态.