Renal symptomatology in patients with acute intermittent porphyria. A population-based study
Renal symptomatology in patients with acute intermittent porphyria. A population-based study
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DOI：
10.1046/j.1365-2796.2000.00743.x
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发表时间：
2000-10-01
影响因子：
11.1
通讯作者：
Lithner, F
中科院分区：
文献类型：
--
作者：
Andersson, C;Wikberg, A;Lithner, F
目标。急性间歇性卟啉(AIP)受试者的肾功能不全是否完全由AIP引起？一项以人口为基础的研究。瑞典最北部四个县的AIP大于或等于18岁的受试者(n=386)。在24小时进行肌酐清除筛查。低于较低参考水平的患者接受了重复清除试验，如果仍然较低，还进行了EDTA铬清除。286名受试者(74%)进行了内生肌酐清除试验，57名受试者的肌酐清除率较低；23名被排除的受试者第二次肌酐清除正常。18名因肾功能不全、道德原因或拒绝进一步检查而可能有其他医学原因的受试者也被排除在外。剩下的16名受试者，除了AIP外，对他们的肾功能不全没有其他解释，然后进行了详细的研究。所有14名女性，平均年龄52岁，以及两名尿毒症男性，58岁和67岁，都有明显的AIP。12例患者有高血压(HT)，4例血压正常，但肾功能不全。肾活检的组织学结果显示弥漫性肾小球硬化和间质改变，并伴有额外的缺血损害。AIP发作时的持续性血管痉挛可能是导致肾脏损害的原因之一。对此进行了讨论。
Objective. Can renal insufficiency in subjects with acute intermittent porphyria (AIP) be due solely to AIP?Design. A population-based study.Subjects. Subjects with AIP greater than or equal to 18 years of age (n = 386) in the four most northerly counties of Sweden.Interventions. 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.Results. 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.Conclusion. Protracted vasospasm in attacks of AIP may be a cause of renal lesions. This is discussed.