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贝多芬的肝硬化是由血色素沉着症引起的吗?

Was Beethoven's cirrhosis due to hemochromatosis?

作者信息

Davies P J

出版信息

Ren Fail. 1995 Jan;17(1):77-86. doi: 10.3109/08860229509036379.

Abstract

Dr. Wagner's description of an advanced macronodular cirrhosis is compatible with end-stage liver disease due to a variety of causes. An alcoholic etiology seems more probable than chronic viral hepatitis since such a diagnosis might also account for the chronic pancreatitis, unless it was related to the cholelithiasis. However, Dr. Wagner's description favors a diagnosis of biliary pigment sludge related to hemolysis. Furthermore, the controversy over the extent of Beethoven's alcohol consumption and the absence of mention of pancreatic calcification weakens the case for an alcoholic etiology. On the other hand, Dr. Wagner's emphasis of bluish-green pigmentation of the liver, blackish pigmentation of the spleen, and an arteropathy of the hepatic vessels suggests the probability of hemochromatosis, which diagnosis is also in keeping with Beethoven's medical history. In this regard the composer's history of recurrent obscure abdominal pain, commencing in his third decade, is especially in keeping with hemochromatosis. As many as a third of patients present with recurrent abdominal pain, and eventually up to 40% of cases develop significant abdominal pain in the course of their disease. While some of these cases of abdominal pain have been attributed to hepatoma, ascites, pancreatitis, perisplenitis, or diabetic neuropathy, the majority remain ill-defined (32). Even so, the diagnosis of hemochromatosis remains unproved in the absence of a histological examination and measurement of hepatic iron concentration. It is proposed that the combined additive, toxic effects of alcohol and iron were the most likely cause of Beethoven's cirrhosis.

摘要

瓦格纳医生对晚期大结节性肝硬化的描述与多种病因导致的终末期肝病相符。酒精性病因似乎比慢性病毒性肝炎更有可能,因为这样的诊断也可以解释慢性胰腺炎,除非它与胆石症有关。然而,瓦格纳医生的描述倾向于诊断为与溶血相关的胆色素沉着。此外,关于贝多芬饮酒量的争议以及未提及胰腺钙化削弱了酒精性病因的可能性。另一方面,瓦格纳医生强调肝脏的蓝绿色色素沉着、脾脏的黑色色素沉着以及肝血管的动脉病变提示了血色素沉着症的可能性,这一诊断也与贝多芬的病史相符。在这方面,作曲家从三十多岁开始反复出现不明原因腹痛的病史尤其与血色素沉着症相符。多达三分之一的患者会出现反复腹痛,最终在疾病过程中高达40%的病例会出现严重腹痛。虽然这些腹痛病例中的一些归因于肝癌、腹水、胰腺炎、脾周炎或糖尿病神经病变,但大多数仍不明确(32)。即便如此,在没有组织学检查和测量肝铁浓度的情况下,血色素沉着症的诊断仍未得到证实。有人提出,酒精和铁的联合累加毒性作用是贝多芬肝硬化最可能的原因。

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