Hosoya Mamiko, Kawasaki Yukihiko, Maeda Ryo, Sato Masatoki, Suyama Kazuhide, Hashimoto Koichi, Hosoya Mitsuaki
Department of Pediatrics, Fukushima Medical University School of Medicine.
Fukushima J Med Sci. 2018 Dec 8;64(3):142-150. doi: 10.5387/fms.2018-05. Epub 2018 Oct 27.
To clarify the predictive factors for poor outcome in pediatric C3 glomerulonephritis (C3GN), we retrospectively evaluated the relationship between the clinico-pathological findings and prognosis in cases of pediatric C3GN.
We enrolled 18 patients diagnosed with C3GN. These patients were divided into two groups, four patients in the end-stage renal disease (ESRD) group and 14 patients in non-ESRD group, based on clinical status at the last examination. Patients in the non-ESRD group were further divided into Subgroup A, consisting of 6 treatment responders, and Subgroup B, consisting of 8 non- responders. The clinical and laboratory findings, as well as the histological findings were investigated for each group.
The frequency of nephrotic syndrome at onset in the ESRD group was higher than that in the non-ESRD group. Before treatment and at 2 years after treatment, urinary protein excretion levels and serum creatinine levels in the ESRD group were higher than those in the non-ESRD group. The mean serum C3 and CH50 levels at 2 years after treatment in the ESRD group were lower than those in the non-ESRD group. The degree of renal injury, level of mesangial deposits and degree of alpha SMA staining at the time of the first renal biopsy in the ESRD group were all higher than those in the non-ESRD group.
Our results suggest that the severity of C3GN at onset and persistent complements activity are associated with poor prognosis in C3GN.
为明确儿童C3肾小球肾炎(C3GN)预后不良的预测因素,我们回顾性评估了儿童C3GN病例的临床病理表现与预后之间的关系。
我们纳入了18例诊断为C3GN的患者。根据末次检查时的临床状态,将这些患者分为两组,终末期肾病(ESRD)组4例,非ESRD组14例。非ESRD组进一步分为A亚组(6例治疗反应者)和B亚组(8例无反应者)。对每组患者的临床和实验室检查结果以及组织学检查结果进行了研究。
ESRD组起病时肾病综合征的发生率高于非ESRD组。治疗前及治疗后2年,ESRD组的尿蛋白排泄水平和血清肌酐水平均高于非ESRD组。ESRD组治疗后2年的平均血清C3和CH50水平低于非ESRD组。ESRD组首次肾活检时的肾损伤程度、系膜沉积水平和α-SMA染色程度均高于非ESRD组。
我们的结果表明,C3GN起病时的严重程度和补体活性持续存在与C3GN的预后不良有关。