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[副肿瘤性迟发性皮肤卟啉症是否存在?]

[Does paraneoplastic porphyria cutanea tarda exist?].

作者信息

Köstler E

机构信息

Hautklinik, Bezirkskrankenhauses Dresden-Friedrichstadt.

出版信息

Dtsch Z Verdau Stoffwechselkr. 1988;48(1):35-40.

PMID:3371236
Abstract

Our investigations in 134 patients showed corresponding to literature porphyria cutanea tarda (PCT) diagnosed by biochemical methods not to be a paraneoplastic dermatosis (but one possible exception acquainted). Relations between PCT and extrahepatic non-porphyrin producing tumours are improbable. Nevertheless but extremely seldom an irregular urinary porphyrin excretion associated with cutaneous changes of hepatic porphyria should lead to the presumption of a porphyrin producing hepatoma. PCT lasting for decades apparently presents a higher frequency of hepatocellular carcinoma in patients suffering from liver cirrhosis than in cirrhotics without PCT. It is supposed that this possible progredience of liver disease in PCT into hepatocellular carcinoma may be prevented by chloroquine phosphate therapy.

摘要

我们对134例患者的调查显示,与文献报道一致,经生化方法诊断的迟发性皮肤卟啉症(PCT)并非副肿瘤性皮肤病(但有一个可能的例外情况)。PCT与肝外非卟啉生成肿瘤之间的关系不太可能存在。然而,极为罕见的是,与肝性卟啉症皮肤改变相关的不规则尿卟啉排泄应引发对卟啉生成性肝癌的推测。持续数十年的PCT患者,肝硬化患者发生肝细胞癌的频率明显高于无PCT的肝硬化患者。据推测,磷酸氯喹治疗可能会阻止PCT患者肝脏疾病进展为肝细胞癌。

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