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威尔逊病患者血清碱性磷酸酶活性降低。

Low serum alkaline phosphatase activity in Wilson's disease.

作者信息

Shaver W A, Bhatt H, Combes B

出版信息

Hepatology. 1986 Sep-Oct;6(5):859-63. doi: 10.1002/hep.1840060509.

DOI:10.1002/hep.1840060509
PMID:3758940
Abstract

Low values for serum alkaline phosphatase activity were observed early in the course of two patients with Wilson's disease presenting with the combination of severe liver disease and Coombs' negative acute hemolytic anemia. A review of other cases of Wilson's disease revealed that 11 of 12 patients presenting with hemolytic anemia had values for serum alkaline phosphatase less than their respective sex- and age-adjusted mean values; in eight, serum alkaline phosphatase activity was less than the lower value for the normal range of the test. Low values for serum alkaline phosphatase were much less common in Wilson's disease patients with more chronic forms of presentation. Copper added in high concentration to serum in vitro did not have an important effect on serum alkaline phosphatase activity. The mechanism responsible for the decrease in serum alkaline phosphatase activity in patients is uncertain.

摘要

在两名患有威尔逊氏病的患者病程早期,观察到血清碱性磷酸酶活性值较低,这两名患者同时伴有严重肝病和库姆斯试验阴性的急性溶血性贫血。对威尔逊氏病其他病例的回顾显示,12例出现溶血性贫血的患者中有11例血清碱性磷酸酶值低于其各自经性别和年龄校正的平均值;其中8例血清碱性磷酸酶活性低于该检测正常范围的下限值。血清碱性磷酸酶值较低在呈现更慢性形式的威尔逊氏病患者中不太常见。体外向血清中添加高浓度铜对血清碱性磷酸酶活性没有重要影响。导致患者血清碱性磷酸酶活性降低的机制尚不确定。

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1
Low serum alkaline phosphatase activity in Wilson's disease.威尔逊病患者血清碱性磷酸酶活性降低。
Hepatology. 1986 Sep-Oct;6(5):859-63. doi: 10.1002/hep.1840060509.
2
Failure of simple biochemical indexes to reliably differentiate fulminant Wilson's disease from other causes of fulminant liver failure.单纯生化指标无法可靠地区分暴发性威尔逊病与其他暴发性肝衰竭病因。
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Acute hemolytic crisis with fulminant hepatic failure as the first manifestation of Wilson's disease: a case report.以急性溶血性危机伴暴发性肝衰竭为首发表现的威尔逊病:一例报告
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Hemolytic anemia in Wilson's disease.威尔逊病中的溶血性贫血。
N Engl J Med. 1967 Feb 23;276(8):439-44. doi: 10.1056/NEJM196702232760804.
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D-Penicillamine improved laparoscopic and histological findings of the liver in a patient with Wilson's disease: 3-year follow-up after diagnosis of Coombs-negative hemolytic anemia of Wilson's disease.青霉胺改善了一名威尔逊病患者肝脏的腹腔镜检查和组织学结果:威尔逊病库姆斯阴性溶血性贫血诊断后3年随访。
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Diagnosis of Wilson's disease presenting as fulminant hepatic failure.以暴发性肝衰竭为表现的威尔逊病的诊断
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Clinical differentiation of fulminant Wilsonian hepatitis from other causes of hepatic failure.暴发性威尔逊氏病肝炎与其他肝衰竭病因的临床鉴别
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Inverse correlation of serum bilirubin and alkaline phosphatase in fulminant Wilson's disease.暴发性威尔逊病中血清胆红素与碱性磷酸酶的负相关
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Low serum alkaline phosphatase activity associated with severe Wilson's disease. Is the breakdown of alkaline phosphatase molecules caused by reactive oxygen species?低血清碱性磷酸酶活性与严重威尔逊病相关。碱性磷酸酶分子的分解是由活性氧引起的吗?
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[Hemolytic anemia in Wilson's disease (hepato-lenticular degeneration)].[威尔逊病(肝豆状核变性)中的溶血性贫血]
Klin Wochenschr. 1972 Nov 1;50(21):995-1002. doi: 10.1007/BF01486994.

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