摘要
目的 研究伴新月体形成和（或）表现为急进性肾炎（RPGN）的原发性IgA肾病患儿的临床病理表现、治疗方法和预后.方法 以2000-2014年北京大学第一医院儿科肾穿刺活检确诊的IgA肾病,肾活检时年龄≤18岁患儿作为纳人标准,并排除继发于系统性红斑狼疮、过敏性紫癜或肝脏疾病的IgA肾病患儿.根据临床表现分为RPGN组和非RPGN组,根据肾活检病理分为有新月体组和无新月体组以及新月体性IgA肾病组和非新月体性IgA肾病组.分别进行组间临床表现、病理特点、治疗和预后的比较.结果 共纳入265例患儿,RPGN组10例（3.8％）,其余为非RPGN组;有新月体组151例,无新月体组114例;新月体性IgA肾病组19例,非新月体性IgA肾病组132例.RPGN组与非RPGN组相比,肾活检时肉眼血尿发生率（70.0％比29.4％）、血肌酐水平、校正肌酐清除率水平、病理为新月体性IgA肾病发生率（50.0％比5.5％）、随访肉眼血尿发生率（30.0％比4.3％）、随访血肌酐水平、随访校正肌酐清除率水平差异有统计学意义（P＜0.05）,RPGN组采用甲泼尼龙冲击、血液净化治疗的例数比非RPGN组更多（P＜0.05）;有新月体组和无新月体组相比,肾活检时肉眼血尿发生率（37.1％比22.8％）、血肌酐水平、校正肌酐清除率水平、24 h尿蛋白定量水平、随访肉眼血尿发生率（7.9％比1.8％）,差异有统计学意义（P＜0.05）,有新月体组应用甲泼尼龙冲击、口服激素、环磷酰胺冲击治疗的例数较无新月体组更多（P＜0.05）;新月体性IgA肾病组和非新月体性IgA肾病组相比,肾活检时RPGN发生率（26.3％比1.5％）、血肌酐水平、24 h尿蛋白定量水平、随访血肌酐水平差异有统计学意义（P＜0.05）,新月体性IgA肾病组应用甲泼尼龙冲击、血液净化治疗的例数较无新月体组更多（P＜0.05）.随访时,临床表现为RPGN且病理为新月体性IgA肾病者有20.0％肾功能恢复正常,病理表现为新月体性IgA肾病但不伴RPGN者有71.4％肾功能恢复正常,临床表现为RPGN伴非新月体性IgA肾病者有80.0％肾功能恢复正常,病理有新月体形成但尚未达到新月体性IgA肾病者有87.5％肾功能恢复正常.结论 在伴有新月体形成和（或）表现为急进性肾炎的原发性IgA肾病患儿中,临床表现为RPGN且病理表现为新月体性IgA肾病者临床表现最重,预后最差;病理表现为新月体形成,但尚未达新月体性IgA肾病者临床表现最轻,预后最佳.
Objective IgA nephropathy is the most common type of glomerulonephritis in the world.Its clinical and pathological manifestations vary.A few of the patients with IgA nephropathy present with rapidly progressive glomerulonephritis （RPGN） and/or crescent formation.Their conditions are serious and acute,but there are few reports on their characteristics,treatment and outcome.This study aimed to analyze the clinicalopathological features,treatment and prognosis of primary IgA nephropathy in children,to provide a reference for clinical diagnosis and treatment.Method A retrospective study was conducted in children with primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis admitted to our department from 2000 to 2014.The patients meeting the inclusion and exclusion criteria were included.Patients were divided into RPGN group and non-RPGN group according to the clinical manifestations,crescent formation group and non-crescent group,crescentic IgA nephropathy group and noncrescentic IgA nephropathy group according to renal biopsy.Their clinical manifestations and pathological features,treatment and prognosis were compared.Result A total of 265 patients were recruited,10 patients （3.8%） had RPGN,151 patients （57.0%） had crescent formation,19 cases （7.2%） showed crescentic IgA nephropathy.Compared with non-RPGN group,RPGN group showed more gross hematuria,higher serum creatinine,lower creatinine clearance correction at biopsy and follow-up,and more crescentic IgA nephropathy （P 〈 0.05）.The percent of patients who received methylprednisolone pulse and blood purification therapy in RPGN group is higher than that of non-RPGN group （P 〈 0.05）.Compared with noncrescent group,crescent formation group showed more gross hematuria at biopsy and follow-up,higher serum creatinine at biopsy,lower creatinine clearance correction,more 24-hour urinary protein at biopsy and higher serum creatinine at follow-up （P 〈 0.05）.The percentage of patients received more methylprednisolone pulse,oral steroids,cyclophosphamide pulse in crescent formation group was higher than that of noncrescent group （P 〈 0.05）.Compared with non-crescentic IgA nephropathy group,crescentic IgA nephropathy group showed more RPGN percent,higher serum creatinine,more 24-hour urinary protein at biopsy （P 〈0.05）.The percentage of patients who received more methylprednisolone pulse and blood purification therapy in crescentic IgA nephropathy group was more than non-crescentic IgA nephropathy group （P 〈 0.05）.At follow-up,20.0% of the patients with RPGN and crescent nephritis returned to normal renal function and the percent of crescent glomerulonephritis but not RPGN was 71.4%,RPGN but not crescent glomerulonephritis was 80.0%,crescent formation but not crescent nephritis was 87.5%.Conclusion In primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis,the patients with both RPGN and crescentic IgA nephropathy showed the worst clinical manifestations,its prognosis was worst while the patients with crescent formation showed the mildest clinical manifestations and best prognosis.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2015年第9期670-675,共6页
Chinese Journal of Pediatrics
