C1q nephropathy in children: clinical characteristics and outcome
C1q nephropathy in children: clinical characteristics and outcome
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DOI：
10.1007/s00467-013-2692-4
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发表时间：
2014-03-01
影响因子：
3
通讯作者：
Tullus, Kjell
中科院分区：
文献类型：
--
作者：
Gunasekara, Vindya N.;Sebire, Neil J.;Tullus, Kjell
C1q肾病被认为是一种独立的疾病实体。C1q阳性也被描述为与肾病综合征（NS）相关，是预后不良的潜在标志物。本研究的目的是描述35例肾组织学显示主要系膜C1q沉积的儿童的临床特征、实验室参数和结果，并调查我们机构的经验是否支持上述假设。将患有微小病变型肾病综合征（MCNS）和基于C1q染色的主要系膜C1q沉积的儿童的结果与没有这种免疫染色的MCNS儿童的结果进行比较。患者队列的中位年龄为4.5岁（范围6个月至16岁），69%为男孩，88%表现为肾病综合征（NS）。C1q染色和MCNS的儿童比免疫染色阴性的儿童有更多的复发（p = 0.001）和更短的无复发期（p = 0.033），但两组的长期结局相似。有MCNS和系膜C1q沉积（染色）的儿童表现出更多的复发，但在长期肾脏预后方面没有差异。
C1q nephropathy has been suggested as a separate disease entity. C1q positivity has also been described in association with nephrotic syndrome (NS) as a potential marker for worse outcome. The aims of this study were to describe the clinical characteristics, laboratory parameters and outcomes of 35 children whose renal histology revealed predominant mesangial C1q deposition and to investigate if the experience at our institution supports the above hypothesis.Clinical and pathological characteristics of all children whose kidney biopsies showed positive C1q staining were retrospectively recorded. The outcome of children with minimal change nephrotic syndrome (MCNS) and predominant mesangial C1q deposition based on C1q staining was compared with that of a concurrent group of children with MCNS with no such immune staining.The median age of the patient cohort was 4.5 years (range 6 months to 16 years), 69 % were boys and 88 % presented with nephrotic syndrome (NS). Children with C1q staining and MCNS had more relapses (p = 0.001) and shorter relapse-free periods (p = 0.033) than those with negative immunostaining, but the long-term outcomes were similar in both groups.Our data do not support C1q nephropathy as a separate diagnostic category. Children with MCNS and mesangial C1q deposition (staining) showed more relapses but no difference in long term renal outcome.