Complement analysis in children with idiopathic membranoproliferative glomerulonephritis:: A long-term follow-up
Complement analysis in children with idiopathic membranoproliferative glomerulonephritis:: A long-term follow-up
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DOI：
10.1034/j.1399-3038.2001.012003166.x
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发表时间：
2001-06-01
影响因子：
4.4
通讯作者：
Kirschfink, M
中科院分区：
文献类型：
--
作者：
Schwertz, R;Rother, U;Kirschfink, M
50例特发性膜增生性肾小球肾炎（MPGN）患儿，年龄2-14岁，发病明显，监测血清中C3肾病因子（C3 NeF）的存在和补体活化的迹象。此外，对32例患者进行C3同种异体分型。观察时间2 ~ 20年（中位11年）。60%的患者中至少检测到一次C3 NeF活性（96例I型患者中有11例，17例II型患者中有15例，7例III型患者中有4例）。C3 nef阳性患者CH50和C3水平显著降低，C3dg/C3d水平升高。随访期间，62%的MPGN I型患者C3水平持续正常，43%的MPGN III型患者C3水平持续正常，但只有18%的MPGN II型患者C3水平持续正常。C3异型频率与健康对照中发现的不同，C3 nef阳性患者的C3F/C3FS变异显著转变。C3b(Bb)P作为替代通路激活的标志物在C3 nef阳性患者中没有增加。尽管存在C3 NeF活性，但在整个观察期间，6例患者的C3水平保持正常。6例患者无法检测到C3 NeF。而7人在随访期间出现C3 NeF活动。有或没有C3 NeF活性的患者肾脏存活率无显著差异。无论是C3变异还是持续的低C3或低CH50水平对临床结果都没有任何预后价值。未发现因子H缺乏。
Fifty children with idiopathic membranoproliferative glomerulonephritis (MPGN), aged 2-14 years at apparent onset, were monitored for the presence of C3 nephritic factor (C3 NeF) and signs of complement activation in serum. In addition, C3 allotyping was performed in 32 patients. Observation time ranged from 2 to 20 (median 11) years. C3 NeF activity was detected at least once in 60%, of the patients (in 11 of 96 with type I, in 15 of 17 with type II, and in four of seven with type III). C3 NeF-positive patients had significantly reduced levels of CH50 and C3 and elevated levels of C3dg/C3d. During follow-up, C3 levels were persistently normal in 62% of the patients with MPGN type I and in 43% with type III but in only 18%, with type II. C3 allotype frequencies differed from those found in healthy controls with a significant shift to the C3F/C3FS variants in C3 NeF-positive patients. C3b(Bb)P as a marker for alternative pathway activation was not increased in C3 NeF-positive patients. Despite the presence of C3 NeF activity, C3 levels remained normal in six patients throughout the observation period. C3 NeF became undetectable in six patients. whereas seven developed C3 NeF activity during follow-up. There was no significant difference in renal survival probability in patients with or without C3 NeF activity. Neither C3 variants nor continous low C3 or low CH50 levels had any prognostic value for the clinical outcome. No factor H deficiency was detected.