Effect Of High Dose Cyclophosphamide Pulse Therapy On Refractory Nephrotic Syndrome Of 100 Cases In Children

Objective : To summarize the clinical efficacy and adverse reactions of intravenous cyclophosphamide(CTX)pulse therapy on refractory nephrotic syndrome(RNS)in children,to help clinicians treatment of refractory nephrotic syndrome in children.Methods : A retrospective longitudinal study,through collecting the application of large dose of cyclophosphamide pulse therapy refractory nephrotic syndrome the clinical data of 100 cases of children,in Pediatric of The First Hospital Affiliated to Guangxi Medical University from Aug.2006 to Aug.2016,to analysis of the clinical efficacy and adverse reactions.Results: 1.The onset age,sex and clinical classification: the age was 5 ~10 years old see more(61.8%),the ratio of male and female was 2.45:1,simplex nephropathy accounted for 58.0%,nephritis nephropathy accounted for 42.0%.2.The steroid-resistant nephrotic syndrome was the most common in the refractory nephrotic syndrome,accounted for 59.0%,the others in turn was the frequent relapse nephrotic syndrome(26.0%)? steroid-dependent nephrotic syndrome(15.0%).3.There are three of the most common pathological type:minimal-change disease(MCNS,45.5%),mesangial proliferative glomerulonephritis(MsPGN,30.3%),focal segmental glomerular sclerosis(Fs GS,18.2%),for the rest of the pathological type: membrane proliferative glomerulonephritis(MPGN,3.0%),membranous nephropathy(MN,3.0%).4.The impact of the recent curative effect after the treatment :(1)Fifty-six patients(56.0%)had achieved complete remission,twenty-one patients(21.0%)had partial remission,and the response rate was 77.0%.(2)Different clinical classification efficacy compared:the simplex kidney disease effective rate was82.8%,nephritis nephropathy effective rate was 69.0%,there was no significant difference compared to the two(P > 0.05).(3)The response rate was 79.7% in the steroid-resistant nephrotic syndrome;the frequent relapse nephrotic syndrome was 73.1%,the steroid-dependent nephrotic syndrome was 73.3%,there was no significant difference compared to the three(P > 0.05).(4)Ease the situation different pathological type:The complete remission of minimal-change disease was 60.0%,part of the response rate of 16.7%;The complete remission of focal segmental glomerular sclerosis was 50.0%,part of the response rate of33.3%;The complete remission of mesangial proliferative glomerulonephritis was 55.0%,part of the response rate of 10.0%;The complete remission of membranous nephropathy was 100.0%;The complete remission of membrane proliferative glomerulonephritis was 50.0%,part of the response rate of 50.0%.There was no significant difference of this three kinds of pathological types relieving rate in MCNS?FsGS and MsPGN(P > 0.05).5.Follow-up situation: 15 cases were cured;Urine protein has been completely relieve the 27 cases,the hormone decrement;Oral Chinese medicine in 11 cases;12cases to switch to other types of immune inhibitors;4 cases of death;Lost to 22 cases.18 cases recurrence,the recurrence rate was 32.1%.6.Adverse circumstance:gastrointestinal reaction,mild hair loss and liver function damage is common,the incidence of adverse reactions was 20.0%.Conclusions : High dose CTX veins impact in combination with small dose of hormone oral treatment children refractory nephrotic syndrome curative effect is remarkable;Starting to pay off the time for 2 period of treatment;Simplex nephropathy with nephritis nephropathy response rate was no significant difference;There was no significant difference of this three kinds of pathological types relieving rate in MCNS?FsGS and MsPGN;Low incidence of adverse reactions.