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孤立性C3系膜增生性肾小球肾炎患者的长期演变

Long-term evolution of patients with isolated C3 mesangial glomerulonephritis.

作者信息

Calls Ginesta J, Almirall J, Torras A, Darnell A, Revert L

机构信息

Nephrology Service, Hospital Clínic i Provincial, Barcelona, Spain.

出版信息

Clin Nephrol. 1995 Apr;43(4):221-5.

PMID:7606875
Abstract

Isolated mesangial C3 proliferative glomerulonephritis is a nephropathy poorly defined among glomerular diseases. Clinical picture is characterized by episodes of gross hematuria and/or persistent or recurrent microhematuria and/or proteinuria. Short-term prognosis (less than 3 years) is considered to be benign, although not much information is available in reference to long-term follow-up. We reviewed all renal biopsies performed in our center between 1978 and 1992 (n = 2,200) in order to study clinical course of these patients. Isolated mesangial C3 deposition was found in 11 cases (0.5%). Isolated proteinuria (> 0.5 g/d) was present in 3 patients and nephrotic syndrome in 2. Hematuria with proteinuria was detected in 5 patients. In one patient hematuria was the only clinical finding. Arterial hypertension was observed in 4 cases. At the time of renal biopsy, renal function was accurately normal in all but one patient. In our series, renal function showed no changes during the first 3 years, accordingly to data referred to in the literature. After 7 years of follow-up, however, renal function was declining in 4 of 5 patients who developed terminal renal failure up to requiring hemodialysis. Therefore, initial benign prognosis ascribed to this glomerulonephritis would not be confirmed in a long-term follow-up.

摘要

孤立性系膜C3增殖性肾小球肾炎是一种在肾小球疾病中定义不明确的肾病。临床表现以肉眼血尿发作和/或持续性或复发性镜下血尿和/或蛋白尿为特征。短期预后(小于3年)被认为是良性的,尽管关于长期随访的信息不多。我们回顾了1978年至1992年在我们中心进行的所有肾活检(n = 2200),以研究这些患者的临床病程。11例(0.5%)发现孤立性系膜C3沉积。3例患者存在孤立性蛋白尿(>0.5g/d),2例为肾病综合征。5例患者检测到血尿伴蛋白尿。1例患者血尿是唯一的临床表现。4例观察到动脉高血压。在肾活检时,除1例患者外,所有患者的肾功能均完全正常。在我们的系列研究中,根据文献报道的数据,肾功能在最初3年内没有变化。然而,经过7年的随访,5例发展为终末期肾衰竭直至需要血液透析的患者中有4例肾功能在下降。因此,这种肾小球肾炎最初被认为的良性预后在长期随访中并未得到证实。

相似文献

1
Long-term evolution of patients with isolated C3 mesangial glomerulonephritis.孤立性C3系膜增生性肾小球肾炎患者的长期演变
Clin Nephrol. 1995 Apr;43(4):221-5.
2
IgG-associated primary glomerulonephritis in children.儿童IgG相关原发性肾小球肾炎
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Isolated C3 deposition in patients without systemic disease.
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Mesangial isolated C3 deposition in patients with recurrent or persistent hematuria.复发性或持续性血尿患者的系膜区孤立性C3沉积。
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[Isolated hematurias: histologic and immunologic study].[孤立性血尿:组织学与免疫学研究]
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[Mesangial glomerulonephritis and intermediate uveitis].[系膜增生性肾小球肾炎与中间葡萄膜炎]
Nefrologia. 2004;24(5):489-92.
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Berger's disease in children. Natural history and outcome.儿童伯杰氏病。自然病史与转归。
Medicine (Baltimore). 1985 May;64(3):157-80.
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Pediatr Nephrol. 2012 Aug;27(8):1221-31. doi: 10.1007/s00467-011-1935-5. Epub 2011 Jun 19.
2
Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees.家族性 C3 肾小球病伴 CFHR5 突变:16 个家系中 91 例患者的临床特征。
Clin J Am Soc Nephrol. 2011 Jun;6(6):1436-46. doi: 10.2215/CJN.09541010. Epub 2011 May 12.
3
Clinicopathologic features, outcome, and therapeutic interventions in four children with isolated C3 mesangial proliferative glomerulonephritis.
四名孤立性C3系膜增生性肾小球肾炎患儿的临床病理特征、预后及治疗干预措施
Pediatr Nephrol. 2005 Sep;20(9):1273-8. doi: 10.1007/s00467-005-1919-4. Epub 2005 Jun 10.