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迟发性皮肤卟啉病的临床管理:更新。

The clinical management of porphyria cutanea tarda: An update.

机构信息

Photobiology and Cutaneous Porphyria Unit, St. John's Institute of Dermatology, London, UK.

Division of Hematology, University of Utah, Salt Lake City, Utah, USA.

出版信息

Liver Int. 2024 Sep;44(9):2191-2196. doi: 10.1111/liv.15980. Epub 2024 May 30.

DOI:10.1111/liv.15980
PMID:38813949
Abstract

Porphyria cutanea tarda (PCT) is the commonest of the porphyrias (Semin Liver Dis 1998;18:67). It often occurs secondary to an underlying internal disorder, has significant impacts on liver health and longevity, and is a treatable disease. Thus, for the clinician, recognising the disease to make the correct diagnosis, identifying causative underlying diseases, and treating the porphyria and its complications, are crucial. Although reviews on the management of PCT have been written, there have recently been significant advances in the understanding of the factors predisposing to the disease, and of its wider health impacts. This review aims to help the clinician to diagnose and manage patients with PCT, with an emphasis on the impact of recent advances on clinical management.

摘要

迟发性皮肤卟啉病(PCT)是最常见的卟啉病(Semin Liver Dis 1998;18:67)。它通常继发于潜在的内部疾病,对肝脏健康和寿命有重大影响,并且是一种可治疗的疾病。因此,对于临床医生来说,识别疾病以做出正确诊断、识别潜在病因疾病以及治疗卟啉病及其并发症至关重要。尽管已经写了关于 PCT 管理的综述,但最近对导致疾病的因素及其更广泛的健康影响有了重大的认识进展。本综述旨在帮助临床医生诊断和管理 PCT 患者,重点是最近进展对临床管理的影响。

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