ABSTRACT
Biomass resources are strategically vital for the sustainable development of a country.The United States has recently made a variety of plans or roadmaps and taken significant actions in order to promote the R&D of biomass resources.These plans and actions for the key issues in the development of biomass resources were analysed.
ABSTRACT
The production technology for the first generation of biofuels has been made great achievements and some countries, such as USA, EU countries, and Brazil, have formed successful biofuel industrial chains. Cellulose ethanol, a representative of the second generation of biofuels and a kind of more promising alternative fuels, has not yet got enough key technological breakthroughs and its large scale commercial production still needs rather some time to realize. Now it is in the early stage of transition to the second generation of biofuels. Taking the development of second generation of biofuels as their national policies, many countries have developed long-term development plans and objectives, and provided a good policy environment and strong support for it. Thus institutes and enterprises are engaging and successing in addressing key issues relating to the development of biofuels. At the mean time, some important issues relating to the sustainable development of biofuels have attracted people's attention.
ABSTRACT
<p><b>OBJECTIVE</b>To establish a specific procedure for the high-risk screening and diagnosis of organic acidurias and other inherited metabolic diseases in China.</p><p><b>METHODS</b>A nation-wide network for the high-risk screening and diagnosis of genetic metabolic diseases was formed to facilitate the collaboration. Urine samples were collected using filter paper from patients with clinical symptoms suspicious of inherited metabolic diseases. The samples were eluted with distilled water and internal standards were added. Samples were treated with hydroxylamine hydrochloride to form oximes to improve the recoveries of 2-ketoacids. Urinary organic acids were extracted with ethyl acetate and diethyl ether under acidic condition. After dehydration, the combined organic phase was evaporated to dryness with nitrogen. The residues were added with BSTFA + 1%TMCS and heat incubated to form the trimethylsilyl derivatives, and then were analyzed on an Agilent 5890/5973N gas chromatography-mass spectrometer (GC-MS), with a 7683 series auto-sampler. The peaks were identified by reference to a mass spectral library.</p><p><b>RESULTS</b>Totally 352 samples were collected from the network collaborating hospitals since 2001. Thirty-four (9.66%) cases of various inherited metabolic diseases were diagnosed with an age range of 2 days to 14 years. The disease profile was consisted of methylmalonic acidemias (6), alpha-keto-glutaric aciduria (5), tyrosinemia type I (4), dicarboxylic aciduria (4), multiple carboxylase deficiency (3), phenylketonuria (3), lactic acidemia (3), propionic acidemia (2), ornithine transcarbamoylase deficiency (1), ethylmalonic-adipic aciduria (1), glutaric aciduria type II (1) and 3-methylcrotyl CoA carboxylase deficiency (1). The most common clinical symptoms and signs included mental and developmental retardation, convulsion, musculotonic abnormality and jaundice. Routine laboratory tests often revealed metabolic acidosis, hypoglycemia and hyperammonemia, etc.</p><p><b>CONCLUSION</b>Urine organic acids analysis by GC-MS remains to be the most important technique for the high-risk screening and diagnosis of inherited metabolic diseases. Use of urine filter paper for sample collection and analysis in advanced genetic metabolic centers is a practical approach to extend the diagnostic capacity and improve the management of such diseases in China. Collaborative network played a critical role in the success of the program.</p>