Zhang Pei, Yang Xiao, Gao Chun-Lin, Wu Wei, Xia Zheng-Kun
Department of Pediatrics, Jinling Hospital, Affiliated Hospital of Medical School, Nanjing University, Nanjing, China.
Department of Pediatrics, Longgang District Center Hospital of Shenzhen, Shenzhen, China.
Front Pediatr. 2023 Aug 25;11:1206168. doi: 10.3389/fped.2023.1206168. eCollection 2023.
Crescentic glomerulonephritis (CrGN) is a relatively rare but severe condition in childhood with the clinical feature of rapidly progressive glomerulonephritis (RPGN). The aim of this study is to investigate the clinicopathological features and prognosis of CrGN in children.
We retrospectively analyzed the clinical and laboratory data, renal pathological results, treatment, and outcome of 147 CrGN in two Chinese pediatric nephrology centers.
Among the 147 children, there were 22 cases of type I (15.0%), 69 cases of type II (46.9%), and 56 cases of type III (38.1%). The mean percentages of crescents in CrGN I, II, and III were 85.3%, 68.7%, and 73.6%, respectively. The children with type I CrGN presented with more severe clinical manifestations and pathological lesions. The 3-month cumulative renal survival rates of types I, II, and III CrGN were 66.3%, 93.6%, and 75.6%, respectively. The 1-year cumulative renal survival rates of types I, II, and III CrGN were 56.9%, 85.3%, and 73.1%, respectively, and the 5-year cumulative renal survival rates of types I, II, and III CrGN were 33.8%, 73.5%, and 47.1%, respectively. The Kappa Consistency Test between the 3-month and 1-year total renal survival (82.1% vs. 74.7%) of the children was 0.683 ( < 0.001), and between the 1-year and 5-year total renal-free survival (78.3% vs. 69.1%) of the children was 0.476 ( < 0.001). The Bowman's Capsule Rupture (BCR), crescent, interstitial inflammation, and interstitial fibrosis/tubular atrophy (IF/TA) score were predictors of end-stage kidney disease (ESKD) risk but BCR showed better predictive value for ESKD than interstitial inflammation score ( = 0.027) and IF/TA score ( = 0.047).
Patients with type I tended to have the worst renal survival rates. The three-month renal prognosis could partially reflect the 1-year renal prognosis, and the 1-year mortality rate could partially reflect the 5-year mortality rate of children with CrGN.
新月体性肾小球肾炎（CrGN）是儿童期相对罕见但严重的疾病，具有快速进展性肾小球肾炎（RPGN）的临床特征。本研究旨在探讨儿童CrGN的临床病理特征及预后。
我们回顾性分析了两个中国儿科肾脏病中心147例CrGN患儿的临床和实验室数据、肾脏病理结果、治疗情况及预后。
147例患儿中，I型22例（15.0%），II型69例（46.9%），III型56例（38.1%）。CrGN I型、II型和III型新月体的平均比例分别为85.3%、68.7%和73.6%。I型CrGN患儿临床表现和病理损害更严重。I型、II型和III型CrGN患儿3个月累积肾脏生存率分别为66.3%、93.6%和75.6%。I型、II型和III型CrGN患儿1年累积肾脏生存率分别为56.9%、85.3%和73.1%，I型、II型和III型CrGN患儿5年累积肾脏生存率分别为33.8%、73.5%和47.1%。患儿3个月和1年总肾脏生存率（82.1%对74.7%）之间的Kappa一致性检验为0.683（<0.001），患儿1年和5年总无肾生存率（78.3%对69.1%）之间的Kappa一致性检验为0.476（<0.001）。肾小球囊破裂（BCR）、新月体、间质炎症和间质纤维化/肾小管萎缩（IF/TA）评分是终末期肾病（ESKD）风险的预测指标，但BCR对ESKD的预测价值优于间质炎症评分（=0.027）和IF/TA评分（=0.047）。
I型患者的肾脏生存率往往最差。3个月肾脏预后可部分反映1年肾脏预后，1年死亡率可部分反映CrGN患儿5年死亡率。
