CAJ | 학술논문

目的探讨小儿原发性局灶节段性肾小球硬化(FSGS)的临床表现、肾脏病理和治疗的疗效特点,以减少或延缓终末期肾病(ESRD).方法对近10年病理确诊的原发性FSGS患儿212例进行了回顾性分析,依据2004年D'Agati原发FSGS组织病理学新分型标准,将患儿分为非特异型、门部型、细胞型、顶部型、塌陷型共5个亚型,比较不同病理分型与其临床表现、实验室指标及治疗的疗效、分析评估病理类型与预后的关系.结果原发FSGS临床诊断以肾病综合征最多178例(83.9%)[其中单纯型97例(45.8%),肾炎型81例(38.2%)];肾病水平蛋白尿14例(6.6%);蛋白尿或伴镜下血尿20例(9.4%).肾组织病理学分型以非特异型最多86例(40.6%),门部型25例(11.8%),细胞型58例(27.4%),顶部型31例(14.6%),塌陷型12例(5.6%).其临床病理的相关性表明,顶部型多呈单纯型肾病,塌陷型多呈肾炎型肾病,此两种亚型之间差异有统计学意义(P＜0.05),细胞型多为小婴儿,临床类型介于两者之间.本组患儿对激素的反应较差,顶部型及非特异型激素初治可敏感,但后多为频复发、依赖或转为耐药的病例;塌陷型、门部型和细胞型多为原发耐药的病例;顶部型与塌陷型两者差异有统计学意义(P＜0.05).激素联合免疫抑制剂治疗,50%以上获得完全缓解.塌陷型2年肾存活率为67%,3年肾存活率为41%.结论原发FSGS病理分5种亚型,临床主要表现为不同程度的蛋白尿、部分伴有血尿和肾功能不全;顶部型治疗反应较好,塌陷型治疗反应及预后较差.
목적 탐토소인원발성국조절단성신소구경화(FSGS)적림상표현、신장병리화치료적료효특점,이감소혹연완종말기신병(ESRD).방법 대근10년병리학진적원발성FSGS환인212례진행료회고성분석,의거2004년D'Agati원발FSGS조직병이학신분형표준,장환인분위비특이형、문부형、세포형、정부형、탑함형공5개아형,비교불동병리분형여기림상표현、실험실지표급치료적료효、분석평고병리류형여예후적관계.결과 원발FSGS림상진단이신병종합정최다178례(83.9%)[기중단순형97례(45.8%),신염형81례(38.2%)];신병수평단백뇨14례(6.6%);단백뇨혹반경하혈뇨20례(9.4%).신조직병이학분형이비특이형최다86례(40.6%),문부형25례(11.8%),세포형58례(27.4%),정부형31례(14.6%),탑함형12례(5.6%).기림상병리적상관성표명,정부형다정단순형신병,탑함형다정신염형신병,차량충아형지간차이유통계학의의(P＜0.05),세포형다위소영인,림상류형개우량자지간.본조환인대격소적반응교차,정부형급비특이형격소초치가민감,단후다위빈복발、의뢰혹전위내약적병례;탑함형、문부형화세포형다위원발내약적병례;정부형여탑함형량자차이유통계학의의(P＜0.05).격소연합면역억제제치료,50%이상획득완전완해.탑함형2년신존활솔위67%,3년신존활솔위41%.결론 원발FSGS병리분5충아형,림상주요표현위불동정도적단백뇨、부분반유혈뇨화신공능불전;정부형치료반응교호,탑함형치료반응급예후교차.
Objective To evaluate the correlation between clinico-pathological features and outcome of children with primary focal segmental glomerular sclerosis (FSGS). Method A total of 212 pediatric patients with D'Agati (2004) primary FSGS were included in this study between 1997 and 2008. According to FSGS histologic classification criteria, 5 pathologic variants were recognized: collapsing ( COLL), cellular (CELL), glomerular tip lesion (GTL), perihilar, and not otherwise specified (NOS). Retrospective analysis of the therapeutic response, the relationship between the clinical efficacy and pathology and the outcome of the patients was made. Results Of the 212 patients, 178 (83. 9% ) had nephritic syndrome (NS), 97 (45.8%) had simple NS , 81 (38.2%) had nephritis-type NS, GTL variants were mostly appeared to be nephritic syndrome (n = 28) and COLL variants were the fewest ( n = 11 ). The difference between the two variants had statistical significance ( P ＜ 0. 05 ). Fourteen cases ( 6. 6% ) had nephrotic proteinuria, 20 cases (9. 4% ) had proteinuria with micro-hematuria. According to histologic classification,NOS ( n = 86, 40. 6% ) was the most common type; perihilar type was seen in 25 cases ( 11.8% ); CELL was seen in 58 cases (27.4%), COLL in 12 cases (5.6%), GTL in 31 cases ( 14. 6% ). Chronic tubular injury was present in most cases. GEL variants were mostly found in the early infancy. GTL and NOS variants initially appeared to be responsive to steroids, but subsequently became resistant or frequently recurrent; CELL and COLL appeared to be primarily steroid resistant, GTL and COLL variants had statistically significant differences (P ＜ 0. 05 ). The patients were followed-up for 5 months to 10 years. A response to therapy was observed in 50%, COLL FSGS had the highest rate of ESRD; 2 years renal survival rates were 67%, 3 years were 41%. Conclusions FSGS is defined as a clinicopathologic syndrome manifesting proteinuria and focal and segmental glomenular sclerosis with foot process effacement. The location of the sclerosis within the glomeruli proved to have prognostic significance. Collapsing glome rulopathy is the most aggressive variant of FSGS. Compared with other variants, GTL variant may be the best type. Different histologic variants of FSGS have substantial differences in clinical features at the time of biopsy diagnosis and substantial differences in renal outcomes. Prolonged treatment of FSGS-NS with corticosteroids and immune suppressive agents may have some effects in achieving sustained remission and improve prognosis in children.