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D-青霉胺在以急性肝衰竭伴溶血为表现的威尔逊病中的应用

D-penicillamine in Wilson's disease presenting as acute liver failure with hemolysis.

作者信息

Vielhauer W, Eckardt V, Holtermüller K H, Lüth J B, Schulte B, Prellwitz W, Sonntag W

出版信息

Dig Dis Sci. 1982 Dec;27(12):1126-9. doi: 10.1007/BF01391452.

DOI:10.1007/BF01391452
PMID:7172963
Abstract

Wilson's disease in a young woman presenting with an acute course is described. The clinical manifestations were fulminant hepatic failure associated with marked intravascular hemolysis. Immediate D-penicillamine and high-dose steroid therapy did not influence the course of the disease. Necropsy revealed an increased hepatic copper content and cirrhosis with extensive necrosis of the liver.

摘要

本文描述了一名年轻女性患威尔逊氏病且病程呈急性的情况。临床表现为暴发性肝衰竭并伴有明显的血管内溶血。立即给予青霉胺和大剂量类固醇治疗并未影响疾病进程。尸检显示肝脏铜含量增加以及伴有广泛肝坏死的肝硬化。

相似文献

1
D-penicillamine in Wilson's disease presenting as acute liver failure with hemolysis.D-青霉胺在以急性肝衰竭伴溶血为表现的威尔逊病中的应用
Dig Dis Sci. 1982 Dec;27(12):1126-9. doi: 10.1007/BF01391452.
2
[Hemolytic anemia associated with severe liver insufficiency as an initial symptom of Wilson's disease].[以溶血性贫血合并严重肝功能不全为初始症状的威尔逊病]
Ned Tijdschr Geneeskd. 1982 Nov 20;126(47):2142-5.
3
Wilson's disease with hepatic presentation in childhood.儿童期以肝脏表现为主的威尔逊病。
Mymensingh Med J. 2007 Jan;16(1):29-32. doi: 10.3329/mmj.v16i1.244.
4
[True hepatic Wilson's disease].[真性肝豆状核变性]
Pediatr Med Chir. 1983 Mar-Apr;5(1-2):7-10.
5
Penicillamine-induced normalization of clinical signs, and liver morphology and histochemistry in a case of Wilson's disease.青霉胺治疗威尔逊病1例,临床症状、肝脏形态及组织化学恢复正常。
Pediatrics. 1970 Feb;45(2):260-8.
6
[2 cases of Wilson's disease diagnosed at the infra-clinical stage. Prolonged, early treatment using penicillamine. Result].2例亚临床期诊断的威尔逊病。使用青霉胺进行长期、早期治疗。结果
Tunis Med. 1974 Jan-Feb;52(1):29-31.
7
[Wilson's disease: a review apropos of a clinical experience in 16 patients].[威尔逊氏病:基于16例临床经验的综述]
Rev Med Chil. 1995 Sep;123(9):1098-107.
8
[Morbus Wilson--pathogenesis, diagnosis, therapy, and course (author's transl)].
Monatsschr Kinderheilkd (1902). 1977 Dec;125(12):947-52.
9
[Wilson's disease or liver cirrhosis of a different etiology].
MMW Munch Med Wochenschr. 1979 Nov 16;121(46):50.
10
Chronic hepatitis as a first manifestation of Wilson's disease.慢性肝炎作为威尔逊病的首发表现。
Ann Intern Med. 1972 Jan;76(1):59-64. doi: 10.7326/0003-4819-76-1-59.

引用本文的文献

1
Research progress in stem cell therapy for Wilson disease.肝豆状核变性的干细胞治疗研究进展
Regen Ther. 2024 Mar 15;27:73-82. doi: 10.1016/j.reth.2024.03.005. eCollection 2024 Dec.
2
Antemortem diagnosis and short-term survival of a patient with Wilson's disease presenting as fulminant hepatic failure.一名表现为暴发性肝衰竭的威尔逊病患者的生前诊断及短期生存情况
Dig Dis Sci. 1984 Sep;29(9):862-4. doi: 10.1007/BF01318433.

本文引用的文献

1
Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, by S. A. Kinnier Wilson, (From the National Hospital, and the Laboratory of the National Hospital, Queen Square, London) Brain 1912: 34; 295-509.进行性豆状核变性:一种与肝硬化相关的家族性神经系统疾病,作者S. A. 金尼尔·威尔逊,(来自伦敦女王广场国立医院及国立医院实验室)《大脑》1912年:第34卷;第295 - 509页
Brain. 2009 Aug;132(Pt 8):1997-2001. doi: 10.1093/brain/awp193.
2
The anemia of liver disease: observations on its mechanism.肝病性贫血:其机制的观察
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3
INFECTIOUS HEPATITIS; A GENERALIZED DISEASE. A STUDY OF RENAL, GASTROINTESTINAL AND HEMATOLOGIC ABNORMALITIES.
传染性肝炎;一种全身性疾病。对肾脏、胃肠道及血液学异常的研究。
Am J Med. 1964 Nov;37:789-801. doi: 10.1016/0002-9343(64)90027-0.
4
Effects of fat-soluble compounds on lysosomes in vitro.脂溶性化合物在体外对溶酶体的影响。
Biochem Pharmacol. 1962 Aug;9:97-116. doi: 10.1016/0006-2952(62)90015-1.
5
The liver in juvenile Wilson's disease.青少年威尔逊氏病中的肝脏
Pediatrics. 1962 Sep;30:402-13.
6
Acute case of Wilson's disease (hepatolenticular degeneration).
Lancet. 1958 Jun 21;1(7034):1309-11. doi: 10.1016/s0140-6736(58)92065-8.
7
Hepatolenticular degeneration (Wilson's disease) as a form of idiopathic cirrhosis.肝豆状核变性(威尔逊病)作为一种特发性肝硬化的形式。
N Engl J Med. 1957 Feb 7;256(6):235-42. doi: 10.1056/NEJM195702072560601.
8
Penicillamine, a new oral therapy for Wilson's disease.青霉胺,一种治疗威尔逊氏病的新型口服疗法。
Am J Med. 1956 Oct;21(4):487-95. doi: 10.1016/0002-9343(56)90066-3.
9
Histochemical localization of copper with rubeanic acid.用赤藓红酸进行铜的组织化学定位。
Lab Invest. 1956 May-Jun;5(3):299-305.
10
Studies on copper metabolism. XIII. Hepatolenticular degeneration.铜代谢研究。十三。肝豆状核变性。
J Clin Invest. 1954 Nov;33(11):1487-501. doi: 10.1172/JCI103027.