Andersson C, Wikberg A, Stegmayr B, Lithner F
Department of Family Practice, University Hospital, Umeå, Sweden.
J Intern Med. 2000 Oct;248(4):319-25. doi: 10.1046/j.1365-2796.2000.00743.x.
Can renal insufficiency in subjects with acute intermittent porphyria (AIP) be due solely to
A population-based study.
Subjects with AIP > or = 18 years of age (n = 386) in the four most northerly counties of Sweden.
Screening with creatinine clearance at 24 h. Patients below the lower reference level underwent a repeat clearance test and, if still low, also chromEDTA clearance.
286 (74%) subjects performed the creatinine clearance test and in 57 clearance was low; the second clearance proved normal in 23 who were then excluded. Eighteen subjects with other possible medical reasons for renal insufficiency, ethical reasons or refusing further examinations were also excluded. The 16 remaining subjects with no explanation for their renal insufficiency other than AIP were then studied in detail. All 14 women, mean age 52 years, and two uraemic men, 58 and 67 years, had manifest AIP. Twelve patients had hypertension (HT) and four were normotensive in spite of renal insufficiency. Histological findings of renal biopsies revealed diffuse glomerulosclerotic and interstitial changes with additional ischaemic lesions.
Protracted vasospasm in attacks of AIP may be a cause of renal lesions. This is discussed.
急性间歇性卟啉病（AIP）患者的肾功能不全是否仅由……引起
一项基于人群的研究。
瑞典最北部四个县年龄≥18岁的AIP患者（n = 386）。
进行24小时肌酐清除率筛查。低于参考下限的患者进行重复清除率测试，若结果仍低，则进行铬标记乙二胺四乙酸清除率测试。
286名（74%）患者进行了肌酐清除率测试，其中57人清除率较低；23人第二次清除率测试结果正常，随后被排除。另外18名因其他可能导致肾功能不全的医学原因、伦理原因或拒绝进一步检查的患者也被排除。对其余16名除AIP外无法解释肾功能不全原因的患者进行了详细研究。所有14名女性（平均年龄52岁）和2名男性尿毒症患者（58岁和67岁）均有明显的AIP。12例患者患有高血压（HT），4例尽管肾功能不全但血压正常。肾活检的组织学结果显示弥漫性肾小球硬化和间质改变，并伴有缺血性病变。
AIP发作时的持续性血管痉挛可能是肾损害的一个原因。对此进行了讨论。
