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特发性系膜毛细血管性肾小球肾炎。儿童和成人I型与II型的比较及长期预后

Idiopathic mesangiocapillary glomerulonephritis. Comparison of types I and II in children and adults and long-term prognosis.

作者信息

Cameron J S, Turner D R, Heaton J, Williams D G, Ogg C S, Chantler C, Haycock G B, Hicks J

出版信息

Am J Med. 1983 Feb;74(2):175-92. doi: 10.1016/0002-9343(83)90606-x.

Abstract

Of 104 patients with idiopathic mesangiocapillary glomerulonephritis studied for at least two years, 69 patients had type I disease and 35 had type II. Forty-five patients were children, and 59 were adults. Type II mesangiocapillary glomerulonephritis was more common in children than in adults, but no other clinical feature distinguished the two types at onset. Complement studies revealed that patients with type II had lower serum C3 concentrations and more frequently showed C3-splitting activity (C3 nephritic factor) in the serum. Children had hypertension or a lowered glomerular filtration rate less frequently at onset than did adults, but children had a higher incidence of a hematuric onset; C3 nephritic factor was also more frequent in the children. During a follow-up period of two to 21 years (mean eight years), only seven patients (five with type I and two with type II) showed clinical remission, whereas 38 percent of patients with type I and 49 percent of patients with type II died or required dialysis; a further 23 percent of patients with type I and 16 percent of patients with type II had continuing disease and reduced glomerular filtration rate. Only the presence and persistence of a nephrotic syndrome in type I predicted renal failure. In both types, the presence of sclerosis or crescents in the initial renal biopsy specimen was associated with a poorer prognosis, but no other feature was of major prognostic value.

摘要

在104例接受了至少两年研究的特发性系膜毛细血管性肾小球肾炎患者中,69例为I型疾病,35例为II型。45例为儿童,59例为成人。II型系膜毛细血管性肾小球肾炎在儿童中比在成人中更常见,但在发病时没有其他临床特征能区分这两种类型。补体研究显示,II型患者的血清C3浓度较低,且血清中更频繁地出现C3裂解活性(C3肾炎因子)。儿童在发病时出现高血压或肾小球滤过率降低的频率低于成人,但儿童血尿发作的发生率较高;C3肾炎因子在儿童中也更常见。在2至21年(平均8年)的随访期内,只有7例患者(5例I型和2例II型)出现临床缓解,而I型患者中有38%、II型患者中有49%死亡或需要透析;I型患者中另有23%、II型患者中另有16%患有持续性疾病且肾小球滤过率降低。只有I型中肾病综合征的存在和持续与肾衰竭相关。在两种类型中,初始肾活检标本中硬化或新月体的存在与预后较差相关,但没有其他特征具有主要的预后价值。

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