IDIOPATHIC MESANGIOCAPILLARY GLOMERULONEPHRITIS - COMPARISON OF TYPE-I AND TYPE-II IN CHILDREN AND ADULTS AND LONG-TERM PROGNOSIS

IDIOPATHIC MESANGIOCAPILLARY GLOMERULONEPHRITIS - COMPARISON OF TYPE-I AND TYPE-II IN CHILDREN AND ADULTS AND LONG-TERM PROGNOSIS
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DOI:
10.1016/0002-9343(83)90606-x
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发表时间:
1983-01-01
影响因子:
5.9
通讯作者:
HICKS, J
HICKS, J
中科院分区:
医学2区
文献类型:
--
作者:
CAMERON, JS;TURNER, DR;HICKS, J

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在研究了至少 2 年的 104 名特发性系膜毛细血管肾炎患者中,69 名患者患有 I 型疾病,35 名患者患有 II 型疾病。患者包括 45 名儿童和 59 名成人。 II 型系膜毛细血管肾小球肾炎在儿童中比成人更常见,但在发病时没有其他临床特征区分这两种类型。补体研究显示,II 型患者的血清补体 C3 浓度较低,并且血清中更频繁地表现出 C3 分裂活性(C3 肾炎因子)。儿童发病时患有高血压或肾小球滤过率降低的频率低于成人,但儿童发病时血尿的发生率较高; C3 肾炎因子在儿童中也更常见。在2-21年(平均8年)的随访期间,只有7名患者(5名I型和2名II型)表现出临床缓解; 38%的I型患者和49%的II型患者死亡或需要透析;另有 23% 的 I 型患者和 16% 的 II 型患者患有持续性疾病且肾小球滤过率降低。只有 I 型肾病综合征的存在和持续才能预测肾衰竭。在这两种类型中,最初肾活检标本中硬化或新月体的存在与较差的预后相关,但没有其他特征具有主要的预后价值。
Of 104 patients with idiopathic mesangiocapillary glomerulonephritis studied for at least 2 years, 69 patients had type I disease and 35 had type II. Patients were 45 children and 59 adults. Type II mesangiocapillary glomerulonephritis was more common in children than in adults, but no other clinical feature distinguished the 2 types at onset. Complement studies revealed that patients with type II had lower serum complement 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 2-21 yr (mean 8 yr), only 7 patients (5 with type I and 2 with type II) showed clinical remission; 38% of patients with type I and 49% of patients with type II died or required dialysis; a further 23% of patients with type I and 16% 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.