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Effects of anticopper therapy on hepatocellular mitochondria in patients with Wilson's disease: an ultrastructural and stereological study.

作者信息

Sternlieb I, Feldmann G

出版信息

Gastroenterology. 1976 Sep;71(3):457-61.

PMID:950097
Abstract

Liver biopsy specimens from 7 patients with Wilson's disease (hepatolenticular degeneration), obtained before and after 3 to 5 years of D-penicillamine therapy, were studied by electron microscopy and stereology. The characteristic mitochondrial abnormalities encountered in the hepatocytes of untreated patients were less pronounced or disappeared after treatment in 5 of the 7 patients. Simultaneously, relative mitochondrial volume, surface density of the external mitochondrial membranes, and the number of these profiles per unit area increased, whereas abnormal elevations SGOT and SGPT returned to normal levels.

摘要

相似文献

1
Effects of anticopper therapy on hepatocellular mitochondria in patients with Wilson's disease: an ultrastructural and stereological study.
Gastroenterology. 1976 Sep;71(3):457-61.
2
Dermal alterations in patients with Wilson's disease treated with D-penicillamine.用D-青霉胺治疗的威尔逊病患者的皮肤改变
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Isr J Med Sci. 1979 Jun;15(6):485-9.
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[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
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[On hepatolenticular degeneration (Wilson's disease). 3. Hepatic localization of Cu64: scintigraphic and dynamic study before and after treatment with BAL and D-penicillamine].[关于肝豆状核变性(威尔逊病)。3. 64Cu在肝脏的定位:用二巯丙醇(BAL)和青霉胺治疗前后的闪烁扫描及动态研究]
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Syndromic variability of Wilson's disease in children. Clinical study of 44 cases.儿童威尔逊病的综合征变异性。44例临床研究。
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Mitochondrial and fatty changes in hepatocytes of patients with Wilson's disease.
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Iron accumulation in the liver of male patients with Wilson's disease.威尔逊氏病男性患者肝脏中的铁蓄积。
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引用本文的文献

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Wilson disease and the differential diagnosis of its hepatic manifestations: a narrative review of clinical, laboratory, and liver histological features.威尔逊病及其肝脏表现的鉴别诊断:临床、实验室及肝脏组织学特征的叙述性综述
Ann Transl Med. 2021 Sep;9(17):1394. doi: 10.21037/atm-21-2264.
2
mtDNA depletion-like syndrome in Wilson disease.威尔逊病中的线粒体DNA耗竭样综合征
Liver Int. 2020 Nov;40(11):2776-2787. doi: 10.1111/liv.14646. Epub 2020 Sep 30.
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Mitochondria and Reactive Oxygen Species in Aging and Age-Related Diseases.
线粒体与衰老及衰老相关疾病中的活性氧
Int Rev Cell Mol Biol. 2018;340:209-344. doi: 10.1016/bs.ircmb.2018.05.006. Epub 2018 Jun 22.
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Wilson disease: histopathological correlations with treatment on follow-up liver biopsies.威尔逊病:治疗随访肝活检的组织病理学相关性。
World J Gastroenterol. 2010 Mar 28;16(12):1487-94. doi: 10.3748/wjg.v16.i12.1487.
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Megamitochondria formation - physiology and pathology.巨型线粒体的形成——生理学与病理学
J Cell Mol Med. 2002 Oct-Dec;6(4):497-538. doi: 10.1111/j.1582-4934.2002.tb00452.x.
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Oral zinc sulphate for Wilson's disease.口服硫酸锌治疗威尔逊氏病。
Arch Dis Child. 1985 Jul;60(7):656-9. doi: 10.1136/adc.60.7.656.
7
Microwave energy fixation for electron microscopy.用于电子显微镜的微波能量固定法。
Am J Pathol. 1985 Aug;120(2):230-43.