1 IntroductionIn the traditional pulping and papermaking industry, many processes are actually done with chemical processes. But chlorine of the chloride generated by reaction with lignin and polychlorinated aromatic ...
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1 IntroductionIn the traditional pulping and papermaking industry, many processes are actually done with chemical processes. But chlorine of the chloride generated by reaction with lignin and polychlorinated aromatic compounds, is deep chromaticity and toxic, can cause cancer ,birth defects as material[1].Therefore, it will cause serious pollution to the natural environment and harms the safety of human beings. Chlorine bleaching in the international scope expand rapidly ,the development of various technologies are also very fast[2].Therefore,the introduction of biological action into the pulping and papermaking process will help to overcome the shortcomings of traditional chemical methods.
很多分析家(Alford and O’Flynn,2012;Ferlie et al,2013)认为网络是解决复杂问题(因为解决复杂问题要求对参与主体间的依存关系进行更好的管理)的最佳方法。网络可以被理解为一种涉及多个彼此没有从属关系的主体间的相互依存结构(O’T...
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很多分析家(Alford and O’Flynn,2012;Ferlie et al,2013)认为网络是解决复杂问题(因为解决复杂问题要求对参与主体间的依存关系进行更好的管理)的最佳方法。网络可以被理解为一种涉及多个彼此没有从属关系的主体间的相互依存结构(O’Toole,1997),也不排除其中权力关系的存在。
Objective: Pyruvate carboxylase (PC) deficiency is a rare metabolic disease. Recently, therapeutic possibilities have been introduced. We aimed to report the largest series of the B type of PC deficiency, focusing on ...
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Objective: Pyruvate carboxylase (PC) deficiency is a rare metabolic disease. Recently, therapeutic possibilities have been introduced. We aimed to report the largest series of the B type of PC deficiency, focusing on some neurological aspects that have not yet been documented. Methods: We retrospectively studied nine patients with the severe neonatal form of PC deficiency diagnosed in our hospital. Detailed clinical features, brain imaging, biochemical characteristics, and global outcome are reported. Results: All patients had axial hypotonia and tachypnea during the first hours of life. The initial level of consciousness was preserved in most patients. Abnormal movements (high-amplitude tremor and hypokinesia) and bizarre ocular behavior were the most common findings, whereas epilepsy was infrequent. Brain magnetic resonance imaging mostly disclosed cystic periventricular leukomalacia. Hypoglycemia, lactic acidosis, and hypercitrullinemia were invariably found. Hyperammoniemia, hypernatremia, and high proline and lysine were frequently detected. A rapid fatal outcome was observed in most patients. Interpretation: Clinical and biochemical characteristics of this deficiency are highly suggestive. Abnormal movements such as rigidity and hypokinesia (hypokinetic-rigid syndrome) are an important hallmark and may orientate to PC deficiency when associated with severe lactic acidosis.
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