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共济失调毛细血管扩张症中的出血性膀胱炎和多瘤病毒JC感染。

Haemorrhagic cystitis and polyomavirus JC infection in ataxia telangiectasia.

作者信息

Christmann Martin, Heitkamp Sonja, Lambrecht Evelyn, Doerries Kristina, Schubert Ralf, Zielen Stefan

机构信息

Department of Pediatrics, Johann Wolfgang Goethe University, Theodor-Stern-Kai 7, Frankfurt/Main 60590, Germany.

出版信息

J Pediatr Urol. 2009 Aug;5(4):324-6. doi: 10.1016/j.jpurol.2009.02.198. Epub 2009 Mar 21.

Abstract

We report the case of a young adolescent with ataxia telangiectasia (AT) and life-threatening haemorrhage from the bladder due to a combination of bladder wall telangiectasis, immunosuppressive therapy and an infection with polyomavirus JC. BK and JC are both members of the polyomavirus family. BK virus is a known cause of haemorrhagic cystitis in bone-marrow and nephropathy in kidney transplant patients, whereas JC virus is mainly associated with progressive multifocal leukoencephalopathy and only rarely found in haemorrhagic cystitis. Although opportunistic infections are uncommon in AT and virus replication was described as being down-regulated in ATM (AT mutated protein)-deficient cells, clinicians should be aware that severe haematuria in a patient with AT and undergoing immunosuppressive therapy is suggestive for polyomavirus JC-induced haemorrhagic cystitis.

摘要

我们报告了一例患有共济失调毛细血管扩张症(AT)的青少年病例,该患者因膀胱壁毛细血管扩张、免疫抑制治疗以及感染多瘤病毒JC而出现危及生命的膀胱出血。BK病毒和JC病毒均为多瘤病毒家族成员。BK病毒是骨髓移植患者出血性膀胱炎和肾移植患者肾病的已知病因,而JC病毒主要与进行性多灶性白质脑病相关,在出血性膀胱炎中很少见。尽管机会性感染在AT中并不常见,且病毒复制在ATM(AT突变蛋白)缺陷细胞中被描述为下调,但临床医生应意识到,患有AT且正在接受免疫抑制治疗的患者出现严重血尿提示可能是多瘤病毒JC引起的出血性膀胱炎。

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