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一位迟发性皮肤卟啉病患者因胆结石导致光敏性短暂恶化。

Transient Worsening of Photosensitivity due to Cholelithiasis in a Variegate Porphyria Patient.

作者信息

Susa Shinji, Sato-Monma Fumiko, Ishii Kouta, Hada Yurika, Takase Kaoru, Tada Kyoko, Wada Kiriko, Kameda Wataru, Watanabe Kentaro, Oizumi Toshihide, Suzuki Tamio, Daimon Makoto, Kato Takeo

机构信息

Department of Neurology, Hematology, Metabolism, Endocrinology and Diabetology, Yamagata University Faculty of Medicine, Japan.

出版信息

Intern Med. 2016;55(20):2965-2969. doi: 10.2169/internalmedicine.55.7108. Epub 2016 Oct 15.

Abstract

Variegate porphyria (VP) is an autosomal dominant disease caused by mutations of the protoporphyrinogen oxidase (PPOX) gene. This porphyria has unique characteristics which can induce acute neurovisceral attacks and cutaneous lesions that may occur separately or together. We herin report a 58-years-old VP patient complicated with cholelithiasis. A sequencing analysis indicated a novel c.40G>C mutation (p.G14R) in the PPOX gene. His cutaneous photosensitivity had been worsening for 3 years before the emergence of cholecystitis and it then gradually improved after cholecystectomy and ursodeoxycholic acid treatment with a slight decline in the porphyrin levels in his blood, urine and stool. In VP patients, a worsening of photosensitivity can thus be induced due to complications associated with some other disease, thereby affecting their porphyrin-heme biosynthesis.

摘要

混合型卟啉病(VP)是一种由原卟啉原氧化酶(PPOX)基因突变引起的常染色体显性疾病。这种卟啉病具有独特的特征,可诱发急性神经内脏发作和皮肤病变,这些病变可能单独出现或同时出现。我们在此报告一名58岁的VP患者并发胆结石。测序分析表明PPOX基因存在一种新的c.40G>C突变(p.G14R)。在胆囊炎出现前,他的皮肤光敏性已经恶化了3年,胆囊切除术后以及用熊去氧胆酸治疗后逐渐改善,其血液、尿液和粪便中的卟啉水平略有下降。因此,在VP患者中,与其他一些疾病相关的并发症可诱发光敏性恶化,从而影响其卟啉-血红素生物合成。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fc73/5109563/5ec3ddcc081f/1349-7235-55-2965-g001.jpg

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