CAJ | 학술논문

目的对8例经重复肾活检确诊为原发性局灶节段性肾小球硬化症(PFSGS)的患儿进行研究,探讨临床-病理相关性,两次肾活检病理类型的联系以及重复肾活检的指征.方法回顾分析近10年在北京大学第一医院儿科肾活检的病例,对其中8例重复肾活检、并最终确诊为PFSGS的病例进行分析,总结其临床特点、重复肾活检的指征、前后两次肾活检病理类型的不同,以及治疗反应.其中局灶节段性肾小球硬化症(FSGS)分型依据2004年D'Agati提出的最新分型标准.结果 8例患儿首次发病年龄在1 ～12岁,临床诊断均为肾病综合征.首次肾穿年龄1.1 ～15.0岁,随访时间10个月～14年.重复肾活检的原因为治疗反应差,持续大量蛋白尿不缓解,伴或不伴肾功能下降.3例患儿两次肾活检均在北京大学第一医院完成,第1次病理类型分别为:系膜增生、FSGS细胞型(CELL)及FSGS顶端型(GTL).第2次肾穿后,分别加用或更换免疫抑制剂,3例出现肾功能下降或终末期肾病,起病年龄均在1岁左右；2例FSGS塌陷型(COLL)者,1例重复肾活检发现伴随亚急性小管间质肾炎.结论 PFSGS是一组临床病理综合征,临床表现以肾病综合征多见.在病程中出现治疗反应差,病情持续不缓解时,通常提示病理转型.系膜增生可以转化为FSGS,FSGS各亚型也可发生转换.塌陷型及发病年龄小者预后差.
목적 대8례경중복신활검학진위원발성국조절단성신소구경화증(PFSGS)적환인진행연구,탐토림상-병리상관성,량차신활검병리류형적련계이급중복신활검적지정.방법 회고분석근10년재북경대학제일의원인과신활검적병례,대기중8례중복신활검、병최종학진위PFSGS적병례진행분석,총결기림상특점、중복신활검적지정、전후량차신활검병리류형적불동,이급치료반응.기중국조절단성신소구경화증(FSGS)분형의거2004년D'Agati제출적최신분형표준.결과 8례환인수차발병년령재1 ～12세,림상진단균위신병종합정.수차신천년령1.1 ～15.0세,수방시간10개월～14년.중복신활검적원인위치료반응차,지속대량단백뇨불완해,반혹불반신공능하강.3례환인량차신활검균재북경대학제일의원완성,제1차병리류형분별위:계막증생、FSGS세포형(CELL)급FSGS정단형(GTL).제2차신천후,분별가용혹경환면역억제제,3례출현신공능하강혹종말기신병,기병년령균재1세좌우；2례FSGS탑함형(COLL)자,1례중복신활검발현반수아급성소관간질신염.결론 PFSGS시일조림상병리종합정,림상표현이신병종합정다견.재병정중출현치료반응차,병정지속불완해시,통상제시병리전형.계막증생가이전화위FSGS,FSGS각아형야가발생전환.탑함형급발병년령소자예후차.
Objective To analyze the characteristics of repeated renal biopsy-proven primary focal segmental glomerulosclerosis (PFSGS) in 8 children,and to reveal the relationship between clinical features and pathology,between the two times of renal biopsy pathology,and the indications for repeated renal biopsy.Method The records of cases who ever experienced renal biopsy in this hospital were reviewed,of whom 8 cases of repeated renal biopsy-proven PFSGS were enrolled.The clinical manifestations,the reason why they had renal biopsy again,the difference in renal pathological findings,between the two biopsies and their therapeutic response.The classification of focal segmental glomerulosclerosis (FSGS) was based on the new criteria suggested by D'Agati in 2004.Result Of the 8 cases,age of onset ranged from 1 to 12 years,all were diagnosed as nephrotic syndrome ( NS),the age of first biopsy ranged from 1.1 to 15.0 years,and the follow-up period was 10 months to 14 years. The reason for repeated biopsy was poor therapeutic response,continuous heavy proteinuria,or the progressive renal dysfunction. Four cases had the both biopsies in this hospital,and the first renal pathology showed minimal change disease (MCD),mesangial proliferation,FSGS CELL type and FSGS GTL type.After the second biopsy,they were additionally treated with immunosuppressive agents or switched to another one,2 cases with FSGS COLL type presented renal dysfunction or end stage renal disease (ESRD),1 case who developed the disease at 1.4 years of age,presented renal dysfunction at 10 months follow-up.The remaining 5 cases acquired complete remission.Conclusion FSGS is a clinicopathological syndrome,NS predominates clinically. It often indicates pathologic transformation when the patients show poor therapeutic response or continuous heavy proteinuria without remission.Mesangial proliferation can convert into FSGS,and the subtype of FSGS can shift.FSGS COLL type and onset at young age may suggest poor prognosis.