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维持性血液透析患者的皮肤异常与卟啉代谢紊乱

Cutaneous abnormalities and metabolic disturbance of porphyrins in patients on maintenance haemodialysis.

作者信息

Gibson G E, McGinnity E, McGrath P, Carmody M, Walshe J, Donohoe J, O'Moore R, Murphy G M

机构信息

Department of Dermatology, Beaumont Hospital, St James's Hospital, Dublin, Ireland.

出版信息

Clin Exp Dermatol. 1997 May;22(3):124-7.

PMID:9425691
Abstract

Blistering disorders may occur in patients with chronic renal failure. Photoactive medication may account for some, and others may be attributable to porphyria cutanea tarda (PCT), but most appear idiopathic. Seventy haemodialysis patients at the National Renal Transplant Centre were therefore screened to determine the prevalence of cutaneous disease and to establish a reference range for plasma porphyrins in this population. The possible contribution of hepatitis C virus (HCV) infection to increased porphyrin levels in this group was also investigated. Ninety four percent of patients on haemodialysis had dermatoses associated with chronic uraemia, and the plasma porphyrin levels in those patients (mean +/- 2 S.D.: 19.1 +/- 13.5 nmol/L) were significantly higher than those of a normal population (n = 40; mean +/- 2 S.D.: 5.5 +/- 3.2 nmol/L) (p < 0.05). Only 2 patients (2.9%), however, had antibodies to HCV and although three others had blistering on light-exposed skin, none of these had PCT or was on photoactive medication, nor did they differ from the rest of the haemodialysis population with regard to erythropoietin or alcohol ingestion. For patients on haemodialysis, therefore, in whom urinary porphyrin estimation is impossible or unreliable, it is recommended that plasma porphyrin profiles be checked where necessary with reference to the range for a haemodialysis population, in addition to assessment of the faecal porphyrin profile. Abnormal porphyrin levels in this group may not, however, be explained by HCV infection, but the occurrence of blistering on the sun-exposed sites of 3 patients suggests that ultraviolet radiation may be implicated in those instances.

摘要

慢性肾衰竭患者可能会出现水疱性疾病。某些病例可能与光敏性药物有关,其他病例可能归因于迟发性皮肤卟啉症(PCT),但大多数病例似乎是特发性的。因此,对国家肾移植中心的70名血液透析患者进行了筛查,以确定皮肤疾病的患病率,并建立该人群血浆卟啉的参考范围。同时还研究了丙型肝炎病毒(HCV)感染对该组卟啉水平升高的可能影响。94%的血液透析患者患有与慢性尿毒症相关的皮肤病,这些患者的血浆卟啉水平(平均值±2标准差:19.1±13.5 nmol/L)显著高于正常人群(n = 40;平均值±2标准差:5.5±3.2 nmol/L)(p < 0.05)。然而,只有2名患者(2.9%)有抗HCV抗体,尽管另外3名患者在暴露于光的皮肤上有水疱,但这些患者均没有PCT或服用光敏性药物,在促红细胞生成素或酒精摄入方面也与其他血液透析人群没有差异。因此,对于无法或不可靠进行尿卟啉测定的血液透析患者,建议在必要时参照血液透析人群的范围检查血浆卟啉谱,同时评估粪便卟啉谱。然而,该组中异常的卟啉水平可能无法用HCV感染来解释,但3名患者在阳光暴露部位出现水疱表明,在这些情况下紫外线辐射可能与之有关。

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