Valvular injury in a patient with PR3-ANCA-associated glomerulonephritis
Valvular injury in a patient with PR3-ANCA-associated glomerulonephritis
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PR3-ANCA 相关性肾小球肾炎患者的瓣膜损伤
DOI：
10.1038/ncpneph0943
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
E. Imai
中科院分区：
文献类型：
--
作者：
H. Iwatani;Y. Nagasawa;K. Oka;Y. Isaka;E. Imai
1例11岁男孩在常规体检中出现血尿，后来被诊断为与抗中性粒细胞胞浆抗体(ANCA)相关的肾小球肾炎。尽管接受了皮质类固醇和免疫抑制剂的治疗，但他继续发展为终末期肾脏疾病。患者在16岁时接受了肾移植，但移植肾发生了三次PR3-ANCA相关性肾炎复发。每次复发都得到了皮质类固醇和免疫抑制剂的成功治疗。19岁时的超声心动图显示中度至重度的主动脉瓣返流。患者在24岁时死于肺炎。尸检发现移植肾的非冠状瓣膜穿孔和新月体肾炎的复发。检查：物理检查，尿液和血液分析，肾脏活检，超声心动图和尸检。诊断PR3-ANCA相关性肾小球肾炎，移植物的新月体肾炎复发，主动脉瓣非冠状体尖的主动脉返流和穿孔。免疫抑制剂和皮质类固醇的处理。
BackgroundAn 11-year-old boy who had hematuria at a routine health check-up was later diagnosed with proteinase 3 (PR3) antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. Despite treatment with corticosteroids and immunosuppressants, he went on to develop end-stage renal disease. The patient received a renal transplant at the age of 16 years, but relapse of PR3-ANCA-related nephritis to the graft occurred three times. Each relapse was successfully treated with corticosteroids and immunosuppressants. An echocardiogram at the age of 19 years revealed moderate-to-severe aortic regurgitation. The patient died of pneumonia when he was 24 years old. Autopsy revealed a perforation in the noncoronary cusp of the aortic valve and recurrence of crescentic glomerulonephritis in the transplanted kidney.InvestigationsPhysical examinations, urine and blood analyses, renal biopsies, echocardiograms and autopsy.DiagnosisPR3-ANCA-associated glomerulonephritis, recurrence of crescentic glomerulonephritis to the graft, aortic regurgitation and perforation in the noncoronary cusp of the aortic valve.ManagementImmunosuppressants and corticosteroids.