Membranoproliferative glomerulonephritis type II (dense deposit disease):: An update
Membranoproliferative glomerulonephritis type II (dense deposit disease):: An update
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DOI：
10.1681/asn.2005010078
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发表时间：
2005-05-01
影响因子：
13.6
通讯作者：
Zipfel, PF
中科院分区：
文献类型：
--
作者：
Appel, GB;Cook, HT;Zipfel, PF
II 型膜增生性肾小球肾炎 (MPGN II) 是一种罕见疾病，其特征是肾脏肾小球基底膜内以及眼睛的布鲁赫膜内沉积异常电子致密物质。大多数患者的诊断年龄在 5 至 15 岁之间，在 10 年内，大约一半进展为终末期肾病，偶尔会出现视力障碍的晚期合并症。 MPGN II 的病理生理学基础与补体级联旁路途径 (AP) 的不受控制的全身激活有关。在大多数患者中，补体调节丧失是由 C3 肾炎因子引起的，C3 肾炎因子是一种针对 AP 的 C3 转化酶的自身抗体，但在某些患者中，已发现 H 因子基因突变。对于后者患者，血浆替代疗法可以预防肾衰竭，但对于大多数患者来说，尚无经过证实的有效治疗方法。这种疾病几乎在所有同种异体肾移植物中都会复发，其中很大一部分最终会失败。分子诊断工具和新疗法的开发旨在控制肾脏局部或全身水平的补体级联 AP，可能会导致 MPGN II 的有效治疗。
Membranoproliferative glomerulonephritis type II (MPGN II) is a rare disease characterized by the deposition of abnormal electron-dense material within the glomerular basement membrane of the kidney and often within Bruch's membrane in the eye. The diagnosis is made in most patients between the ages of 5 and 15 yr, and within 10 yr, approximately half progress to end-stage renal disease, occasionally with the late comorbidity of visual impairment. The pathophysiologic basis of MPGN II is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade. In most patients, loss of complement regulation is caused by C3 nephritic factor, an autoantibody directed against the C3 convertase of the AP, but in some patients, mutations in the factor H gene have been identified. For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment. The disease recurs in virtually all renal allografts, and a high percentage of these ultimately fail. The development of molecular diagnostic tools and new therapies directed at controlling the AP of the complement cascade either locally in the kidney or at the systemic level may lead to effective treatments for MPGN II.