期刊文献+
任意字段
题名或关键词
题名
关键词
文摘
作者
第一作者
机构
刊名
分类号
参考文献
作者简介
基金资助
栏目信息

尿素循环障碍病人的营养治疗 被引量：2

原文传递
导出
摘要 尿素循环又称鸟氨酸循环或krebs-hengeleit循环，主要在肝脏内完成，其功能是将有毒的氨合成无毒的尿素，从而避免高氨血症的发生。该循环包括6个步骤的系列生化反应，有6种酶参与，其中任何一种酶出现结构或功能异常都会影响尿素合成而形成高氨血症，严重者出现发作性脑病的临床表现。此为尿素循环障碍（urea cycle disorders，UCD）。
作者 刘海珍
机构地区 山东省潍坊市人民医院临床营养科
出处 《中国临床研究》 CAS 2013年第11期1251-1253,共3页 Chinese Journal of Clinical Research
关键词 尿素循环障碍 高氨血症 营养治疗
分类号 R589.3 [医药卫生—内分泌]
  • 相关文献

参考文献17

  • 1Braissant O. Current concepts in the pathogenesis of urea cycle disor- ders[J]. Mol Genet Metab,2010,100 Suppl 1 :s3 - s12.
  • 2Singh RH, Rhead WJ, Smith W, et al. Nutritional management of urea cycle disorders [ J ]. Crit Care Clin,2005,21 Suppl 4 : s27 - s35.
  • 3LiUiu F. Treatment of organic aeidurias and urea cycle disorders[ J]. J Matem Fetal Neonatal Med,2010,23 Suppl 3:73 -75.
  • 4Maillot F, Crenn P. Urea cycle disorders in adult patients [ J ]. Rev Neurol ( Paris),2007,163 (10) :897 - 903.
  • 5Nagasaka H,Tsukahara H, Yorifuji T, et al. Evaluation of endogenous nitric oxide synthesis in congenital urea cycle enzyme defects [ J ]. Metabolism,2009,58 ( 3 ) :278 - 282.
  • 6Brosnan ME, Bmsnan JT. Orotie acid excretion and arginine metabo- lism[ J]. J Nutr,2007,137(6 Suppl 2) :1656s - 1661s.
  • 7Erez A, Nagamani SC, Lee B. Argininosuccinate lyase deficiency- argininosuccinic aciduria and beyond[ J]. Am J Med Genet C Semin Med Genet,2011,157c( 1 ) :45 -53.
  • 8Puplim LB, FlakoU PJ, Ikizler TA. Protein homeostasis in chronic hemodialysis patient [ J ]. Curr Opin Clin Nutr Metab Care, 2004,7 (1) :89.
  • 9Singh RH. Nutritional management of patients with urea cycle disor- ders[ J]. J Inherit Metab Dis,2007,30(6) :880 -887.
  • 10Wilcken B. An introduction to nutritional treatment in inborn errors of disorders, different approaches [ J ]. Southeast Asian J Trop Med Public Health,2003,34 Suppl 3 : 198 - 201.

二级参考文献44

  • 1ZhangJM(张俊梅) HouL MaoJ etal.A report of a case of citrullinemia[J].中国医师进修杂志,2009,11(2):288-288.
  • 2Yin XY (尹向云) Xue XD Fu JH.Congenital urea cycle disorders-a case of neonatal citrullinemia[J].中国实用儿科杂志,2010,25(5):401-402.
  • 3Kim IS, Ki CS, Kim JW, et al. Characterization of late-onset citrullinemia 1 in a Korean patient: confirmation by argininosuccinate synthetase gene mutation analysis. J Biochem Mol Biol, 2006, 39(4):400-405.
  • 4Kara B, Albayram S, Tutar O, et al. Diffusion-weighted magnetic resonance imaging findings of a patient with neonatal citrullinemia during acute episode. Eur J Paediatr Neurol, 2009, 13(3):280-282.
  • 5Majoie CB, Mourmans JM, Akkerman EM, et al. Neonatal citrullinemia: comparison of conventional MR, diffusion-weighted, and diffusion tensor findings.Am J Neuroradiol, 2004, 25(1):32-35.
  • 6Ohtake A, Takayanagi M, Ogura N, et al. A case of transient neonatal citrullinemia. Eur J Pediatr 1983;141(1):60-61.
  • 7Nukada O, Uchiyama C, Ubuka S, et al. A case of citrullinemia with abnormal messenger RNA for argininosuccinate synthetase. Acta Paediatr Jpn, 1991, 33(5):672-677.
  • 8Sanjurjo P, Rodriguez-Soriano J, Vallo A, et al. Neonatal citrullinaemia with satisfactory mental development. Eur J Pediatr, 1991,150(10):730-731.
  • 9Thoene J, Batshaw M, Spector E, et al. Neonatal citrllinemia: treatment with keto-analogues of essential amino acids. J Pediatr, 1977, 90(2):218-224.
  • 10Clancy RR, Chung HJ. EEG changes during recovery from acute severe neonatal citrullinemia. Electroencephalogr Clin Neurophysiol, 1991, 78(3):222-227.

共引文献40

同被引文献11

引证文献2

二级引证文献4

中国临床研究
2013年 第11期

相关作者

内容加载中请稍等...

相关机构

内容加载中请稍等...

相关主题

内容加载中请稍等...

浏览历史

内容加载中请稍等...
;
使用帮助 返回顶部