摘要
肝豆状核变性又名Wilson病(WD),患者第13号染色体铜转运P型ATPase基因突变导致肝脏铜蓝蛋白合成障碍,体内过量的铜沉积于肝脏、脑、角膜和肾脏等部位,出现肝脏、神经系统、肾功能异常和角膜环等临床表现。如能早期行排铜治疗和低铜膳食,可获长期缓解,但对晚期患者,肝移植是目前最有效的治疗方法。
出处
《中华消化杂志》
CAS
CSCD
北大核心
2007年第8期551-551,共1页
Chinese Journal of Digestion
参考文献7
-
1.肝豆状核变性[M].姚光弼.临床肝脏病学[C].上海:上海科学技术出版社,2004.590.
-
2Schilsky ML, Scheinberg IH, Sternlieb I. Liver transplantation for Wilson's disease: indications and outcome. Hepatology, 1994, 19:583-587.
-
3Brewer GJ. Recognition, diagnosis, and management of Wilson's disease. Proc Soc Exp Biol Med, 2000, 223:39-46.
-
4Dhawan A, Taylor RM, Cheeseman P, et al. Wilson's disease in children: 37-year experience and revised King's score for liver transplantation. Liver Transpl, 2005, 11:441-448.
-
5Emre S, Atillasoy EO, Ozdemir S, et al. Orthotopic liver tranplantation for Wilsons disease: a single-center experience. Transplantation, 2001, 72 : 1232-1236.
-
6Sutcliffe RP, Maguire DD, Muiesan P, et al. Liver transplantation for Wilson's disease: long-term results and quality-of-life assessment. Transplantation, 2003, 75:1003-1006.
-
7Geissler I, Heinemann K, Rohm S, et al. Liver transplantation for hepatic and neurological Wilson's disease. Transplant Proc, 2003, 35 : 1445-1446.
同被引文献23
-
1邵卓,杨广顺,杨宁,张海斌.肝移植治疗Wilson病5例分析[J].中国临床医学,2007,14(3):339-341. 被引量:2
-
2Ala A,Walker AP,Ashkan K,et al.Wilson's disease[J].Lancet,2007,369(9559):397-408.
-
3Hoogenraad TU.Paradigm shift in trentment of wilson's disease:Zinc therapy now treatment of choice[J].Brain Develop,2006,28(3):141-146.
-
4BrewerGJ,AskariF,LorinczMT,et al.Treatme nt of Wilson disease with ammonium tetrathiomolybdate[J].Arch Neurol,2006,63(4):521-527.
-
5阴瑞红,曲云东,林森.移植治疗肝豆状核变性的临床和研究进展[J].国际消化病杂志,2007,27(4):275-277. 被引量:4
-
6Dhawan A,Ferenci P,Geubel A. Genes and metals:a deadly combination[J].{H}Acta Gastroenterologica Belgica,2005,(01):26-32.
-
7Ala A,Walker AP,Ashkan K. Wilson's disease[J].{H}LANCET,2007,(9559):397-408.
-
8Geissler I,Heinemann K,Rohm S. Liver transplantation for hepatic and neurological Wilson's disease[J].{H}TRANSPLANTATION PROCEEDINGS,2003,(04):1445-1446.
-
9Sutclif e RP,Maguire DD,Muiesan P. Liver transplantation for Wilson's disease:long-term results and quality-of-life assessment[J].{H}TRANSPLANTATION,2003,(07):1003-1006.
-
10Schilsky ML. Wilson disease:genetic basis of copper toxicity and natural history[J].{H}Seminars in Liver Disease,1996,(01):83-95.
-
1丛婷婷,贾胜男,唐丽敏,潘留兰.肝豆状核变性一例[J].中国综合临床,2017,33(2):165-166.
-
2金贵凤,吴雁平,张德泉.角膜环与脑动脉硬化[J].中国疗养医学,1997,6(3):43-44.
-
3刘恩九,李中国.肯林—氟利施角膜环一例[J].吉林医学信息,1994(12):28-29.
-
4王顺祥,乔永新.肝移植治疗肝性脊髓病1例的长期疗效[J].中华肝脏病杂志,2009,17(2):149-149. 被引量:6
-
5林连捷,郑长青.Wilson病的诊断和治疗[J].世界华人消化杂志,2003,11(10):1614-1617. 被引量:4
-
6陈泽响,陆磊.帕金森病与糖尿病相关性研究进展[J].国际神经病学神经外科学杂志,2015,42(1):81-84. 被引量:4
-
7郭庆军,朱志军,郑虹,李延钧,张建军,薛芳菁,侯建存,沈中阳.肝移植治疗肝性脊髓病三例[J].中华外科杂志,2007,45(23):1650-1650. 被引量:3
-
8程东峰,彭承宏.肝移植治疗Wilson病的疗效[J].中华器官移植杂志,2005,26(5):319-320. 被引量:2
-
9彭艳,蒋莉.难治性癫痫的危险因素[J].儿科药学杂志,2010,16(4):61-63. 被引量:1
-
10崔园园,杨以良,王莎莎,华瑞.以精神障碍为首发症状的肝豆状核变性1例报告[J].临床肝胆病杂志,2016,32(11):2173-2174. 被引量:3