Marsden J T, Chowdhury P, Wang J, Deacon A, Dutt N, Peters T J, Macdougall I C
Department of Clinical Biochemistry, King's College Hospital, London, UK.
Clin Nephrol. 2008 May;69(5):339-46. doi: 10.5414/cnp69339.
Despite the little known association between renal damage and the acute porphyrias, limited information is available on the characteristics and pathogenesis of renal disease in this patient group. Previous reports have focused on hypertension as the principal etiological factor. We have studied a series of 9 patients with acute intermittent porphyria (AIP) attending the Porphyria Clinic at King's College Hospital, London, UK, who were referred to the Renal Unit for investigation and treatment of their renal disease. No evidence of a glomerular lesion was found in any of the patients. In contrast, renal histology showed features of a tubulointerstitial disease, and there was evidence of impaired erythropoietin production. Hypertension and nonsteroidal antiinflammatory drug use were present in about a half of the patients. It is postulated that the nephrotoxic effects of porphyrin precursors may contribute to the etiology of this clinical syndrome.
尽管肾损伤与急性卟啉病之间的关联鲜为人知，但关于该患者群体肾脏疾病的特征和发病机制的信息有限。既往报告主要关注高血压是主要病因。我们研究了英国伦敦国王学院医院卟啉病诊所的9例急性间歇性卟啉病（AIP）患者，他们因肾脏疾病被转诊至肾脏科进行检查和治疗。所有患者均未发现肾小球病变的证据。相反，肾脏组织学显示为肾小管间质性疾病的特征，并有促红细胞生成素生成受损的证据。约一半患者存在高血压和使用非甾体类抗炎药的情况。据推测，卟啉前体的肾毒性作用可能是该临床综合征病因的一部分。
