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直接作用抗病毒药物清除丙型肝炎后亚临床型皮肤卟啉病的消退。

Resolution of subclinical porphyria cutanea tarda after hepatitis C eradication with direct-acting anti-virals.

机构信息

Liver Unit, Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS) and Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBEREHD), University of Barcelona, Barcelona, Spain.

Biochemistry and Molecular Genetics Unit, Hospital Clínic de Barcelona, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.

出版信息

Aliment Pharmacol Ther. 2020 May;51(10):968-973. doi: 10.1111/apt.15703. Epub 2020 Apr 15.

Abstract

BACKGROUND

Hepatitis C virus (HCV) is a risk factor for porphyria cutanea tarda (PCT), a rare disease originating in the liver characterised by overproduction of porphyrins. Although hepatitis C infection is highly prevalent among patients with porphyria, only a minority of hepatitis C patients develop PCT.

AIMS

To explore the presence of porphyrin abnormalities in a cohort of asymptomatic hepatitis C-infected patients and the impact of anti-viral therapy.

METHODS

Eighty-four consecutive patients with HCV infection treated with direct-acting antivirals after 1 January 2018 were longitudinally evaluated for the presence of porphyrin abnormalities. Those patients with biochemical abnormalities at baseline were additionally evaluated at follow-up. Porphyrins in urine were screened by fluorometry and isomer separation was performed by liquid chromatography.

RESULTS

In five patients, all of them asymptomatic, porphyrin profile abnormalities were detected: three presented significant increased urinary porphyrins with a typical PCT profile, and two showed normal levels of urinary porphyrins, but abnormal porphyria-like profiles. Urine evaluation after hepatitis C cure showed complete normalisation of the urinary porphyrins in all patients, confirming the biochemical cure of the disease.

CONCLUSIONS

We document the existence of rare cases of hepatitis C-infected patients with significant uroporphyrinuria in the absence of dermatological manifestations. Anti-viral therapy normalises the biochemical disorder, preventing patients from presenting PCT associated complications.

摘要

背景

丙型肝炎病毒(HCV)是迟发性皮肤卟啉病(PCT)的一个风险因素,PCT 是一种罕见的肝脏起源疾病,其特征是卟啉产量过多。尽管丙型肝炎感染在卟啉症患者中非常普遍,但只有少数丙型肝炎患者会发展为 PCT。

目的

探索丙型肝炎感染患者无症状队列中卟啉异常的存在情况以及抗病毒治疗的影响。

方法

对 2018 年 1 月 1 日后接受直接作用抗病毒药物治疗的 84 例连续 HCV 感染患者进行纵向评估,以确定是否存在卟啉异常。对基线时存在生化异常的患者进行随访评估。通过荧光法筛查尿液中的卟啉,通过液相色谱法进行异构体分离。

结果

在五名患者中均发现卟啉图谱异常,这些患者均无症状:其中三人出现显著的尿卟啉增加,具有典型的 PCT 图谱;两人表现为正常的尿卟啉水平,但出现异常的卟啉样图谱。丙型肝炎治愈后对尿液进行评估,所有患者的尿液卟啉均完全正常,证实了疾病的生化治愈。

结论

我们记录了在无皮肤表现的情况下,丙型肝炎感染患者存在显著的尿卟啉症的罕见病例。抗病毒治疗可使生化紊乱正常化,防止患者出现与 PCT 相关的并发症。

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