CAJ | 학술논문

目的观察环孢霉素A(Cyclosporine A；CsA)治疗儿童难治性肾病综合征的临床疗效.方法应用CsA联合泼尼松治疗26例难治性肾病综合征患儿,CsA剂量为3～6 mg/(kg·d),疗程3 ～27(12.69 ±6.44)个月.治疗前后检测24h尿蛋白定量、血清胆固醇、血清尿素氮、血清肌酐、血清胱抑素(CyC)、血清N-乙酰β-氨基葡萄糖苷酶等相关生化指标,并观察药物不良反应.结果 26例患儿中激素耐药12例,激素依赖6例,频繁复发8例.治疗后完全缓解16例,占61.54％,部分缓解8例,占30.77％,未缓解2例,占7.69％,总有效率92.31％.观察指标结果:24h尿蛋白定量:治疗前3.01g/24 h,治疗后0.63g/24 h,其差异有统计学意义(P＜0.01)；血清胆固醇:治疗前(7.72 ±3.86) mmol/L,治疗后(7.15 ±3.23) mmol/L；尿素氮:治疗前(3.93±1.44) mmol/L,治疗后(4.04±1.27)mmol/L;肌酐:治疗前(33.38±13.16) μmol/L,治疗后(35.64 ±3.53) μmol/L;血清N-乙酰β-氨基葡萄糖苷酶:治疗前(18.96±4.86) u/L,治疗后(20.45 ±5.85) u/L;以上指标差异均无统计学意义(P＞0.05).激素抵抗组、激素依赖组及频繁复发组的疗效差异无统计学意义(P＞0.05).完全缓解患儿随访至6、9、12、18个月的复发率依次为37.5％、31.25％、18.75％、12.5％.8例患儿停药3～6个月,均无复发.CsA的不良反应依次为多毛、震颤、胃肠道反应等,而治疗期间肝、肾毒性并不明显.结论 CsA联合泼尼松能有效治疗小儿难治性肾病综合征,并可明显减少糖皮质激素的使用剂量,至少1年以上维持治疗疗程可明显减少病情复发.
목적 관찰배포매소A(Cyclosporine A；CsA)치료인동난치성신병종합정적림상료효.방법 응용CsA연합발니송치료26례난치성신병종합정환인,CsA제량위3～6 mg/(kg·d),료정3 ～27(12.69 ±6.44)개월.치료전후검측24h뇨단백정량、혈청담고순、혈청뇨소담、혈청기항、혈청광억소(CyC)、혈청N-을선β-안기포도당감매등상관생화지표,병관찰약물불량반응.결과 26례환인중격소내약12례,격소의뢰6례,빈번복발8례.치료후완전완해16례,점61.54％,부분완해8례,점30.77％,미완해2례,점7.69％,총유효솔92.31％.관찰지표결과:24h뇨단백정량:치료전3.01g/24 h,치료후0.63g/24 h,기차이유통계학의의(P＜0.01)；혈청담고순:치료전(7.72 ±3.86) mmol/L,치료후(7.15 ±3.23) mmol/L；뇨소담:치료전(3.93±1.44) mmol/L,치료후(4.04±1.27)mmol/L;기항:치료전(33.38±13.16) μmol/L,치료후(35.64 ±3.53) μmol/L;혈청N-을선β-안기포도당감매:치료전(18.96±4.86) u/L,치료후(20.45 ±5.85) u/L;이상지표차이균무통계학의의(P＞0.05).격소저항조、격소의뢰조급빈번복발조적료효차이무통계학의의(P＞0.05).완전완해환인수방지6、9、12、18개월적복발솔의차위37.5％、31.25％、18.75％、12.5％.8례환인정약3～6개월,균무복발.CsA적불량반응의차위다모、진전、위장도반응등,이치료기간간、신독성병불명현.결론 CsA연합발니송능유효치료소인난치성신병종합정,병가명현감소당피질격소적사용제량,지소1년이상유지치료료정가명현감소병정복발.
Objective To evaluate the efficacy of cyclosporine A plus prednisone in the treatment of refractory nephrotic syndrome in children.Methods The 26 refractory nephrotic syndrome children were treated with CsA plus prednisone,3 ～6 mg/(kg d),Po.The duration of treatment was 3 to 27 months (12.69 ± 6.44) mos.The 24 h quantitative urinary proteins,serum cholesterol,urea nitrogen,plasma creatinine,N-acetyl beta amino glycosidase enzymes cystatin C were detected before and after treatment,and the adverse drug reactions were accessed.Results Among 26 cases,12 cases of steroid-resistant NS,6 cases of steroid-dependent NS,and 8 cases of frequent relapse NS were included.16 patients (61.54％) were complete remission,8 patients (30.77％) partial remission,2 cases (7.69％) were non-remission,The total remission rate was 92.31％.The 24 hours urine protein was 3.01 g and 0.63 g before treatment and after treatment,respectively,with a statistically significant difference (P ＜0.01); serum cholesterol was (7.72 ± 3.86) mmol/L and (7.15 ± 3.23) mmol/L; nitrogen urea was (3.93 ± 1.44) mmol/L and (4.04 ± 1.27) mmol/L,creatinine (33.38 ± 13.16) μmol/L and (35.64 ± 3.53) μmol/L serum N-acetyl beta amino glycosidase enzymes was (18.96 ±4.86) u/L and (20.45 ±5.85)u/L before treatment and after treatment,respectively,without a statistically significant difference (P ＞ 0.05).The response to CsA was no significant difference in SRNS,SDNS and FRNS.Children complete remission,follow-up to 6 months,9 months,12 months and 18 months of the recurrence rate was 37.5％,31.25％,18.75％ and 12.5％.Eight cases ended treatment 3 ～ 6 months,all cases were not recurrence.The main adverse effects of CsA included hirsutism,tremble,gastrointestinal reaction and so on,and liver kidney toxicity was not obvious during the therapy course.Conclusions The treatment of CsA in combination with prednisone to children refractory nephrotic syndrome had a significant curative effect,which could obviously minimize the dosage of glucocorticoids and reduce the recurrence after at least one year of maintenance treatment of CsA.