| ObjectiveTo explore the pathogenesis of ITP from the genetic level,as well as its impact on the diagnosis and prognosis of children;the whole children in the group were given Fuzheng Jiedu Prescription for intervention treatment,and further study whether the gene defect will affect the clinical efficacy,TCM syndrome curative effect,the increase of the count of platelet,the decrease of integral of TCM syndrome and the improvement of scale of haemorrhage grade of Fuzheng Jiedu Prescription,so as to provide reference for further study from the genetic level.This paper expounds the mechanism of Fuzheng Jiedu prescription and provides data support.Research methodFrom March 2018 to November 2019,24 children who suffer from continuous and chronic immune thrombocytopenic purpura with syndrome of qi failing to control blood admitted to Professor Yu Huiping's outpatient department of Pediatrics in Dongzhimen Hospital were selected as the research objects for gene detection.According to the results of gene report,they were divided into gene defect group and gene normal group.Statistical analysis of the positive rate of gene defects,gene defects related factors and analysis of specific gene defects that may lead to the onset of ITP,and according to the gene detection to determine the diagnosis and prognosis of children.Both groups were treated with Fuzheng Jiedu Prescription for 6 months and followed up for 1 month.By testing the platelet count of peripheral blood,recording the scale of integral of TCM syndrome and scale of haemorrhage grade,the indexes of the day of entering the group and each month of treatment were statistically analyzed to study the clinical efficacy,TCM syndrome efficacy,increase of count of platelet,decrease of the integral of TCM syndrome and improvement of haemorrhage grade of Fuzheng Jiedu prescription,and to explore whether gene defects will affect Fuzheng Jiedu prescription The therapeutic effect of Fuzheng Jiedu prescription can provide data support for further elaborating the mechanism of Fuzheng Jiedu prescription from the gene level.Research results1.General data analysis:in this study,24 children who suffer from continuous and chronic immune thrombocytopenic purpura with syndrome of qi failing to control blood were included for gene detection.According to the results of gene report,they were divided into gene defect group and gene normal group,including 13 cases in gene defect group and 8 cases in gene normal group.In the course of the study,3 children were excluded because they were definitely diagnosed as hereditary thrombocytopenia after gene testing;in addition,2 children in the gene defect group dropped off due to self withdrawal,so 11 children in the gene defect group and 8 children in the gene normal group finally completed the study.There were 8 girls and 5 boys in the gene defect group,with an average age of(6.04±3.598)years old ranging from 7 months to 13 years old;the course of disease ranged from 4 months to 48 months,with an average age of(18.92±13,690)months;two of them had a positive family history of hematological or immune system diseases,and the count of platelet count on the day of enrollment was(53.00±23.944)×109/L.In the normal gene group,there were 5 girls and 3 boys,with an average age of(4.50±2.000)years(range 2-7 years);the course of disease was(8.88±4.734)months(range 3-17 months);two of them had a positive family history of immune system diseases;the count of platelet on the day of enrollment was(42.09±19.488)X 109/L.There was no significant difference in gender,age,positive family history and platelet count on the day of admission between the two groups(P>0.05),but there was significant difference in the course of disease(P<0.05).2.Analysis of gene defect positive rate and related factors:there were 21 cases in the two groups,and 13 cases in ITP gene defect group.The gene defect positive rate was 61.90%(13/21).By multivariate binary logistic test,age,gender,course of disease,positive family history and the count of platelet on the day of enrollment were not correlated with gene defects in children with continuous and chronic ITP(P>0.05).3.Specific gene defect analysis:in the gene defect group,most of the children have different gene defects.PLA2G4A and RTEL 1 is highly correlated with ITP,and clinical reports are rare.Most of the genes that may be associated with ITP have various clinical phenotypes,such as STIM1 and BCL11B.Among the genes with unknown clinical significance,LRBA has the highest frequency.4.In terms of clinical efficacy,a total of 19 patients with continuous or chronic ITP completed the whole clinical study,including 11 patients with gene defect.The total effective rates(mainly complete response and effective)in the first,third and sixth months of treatment were 9.09%(1/11),9.09%(1/11)and 27.27%(3/11),respectively.The total effective rate(mainly complete response and effective)of normal gene group was 12.5%(1/8),12.5%(1/8)and 37.5%(3/8),separately.After 1,3 and 6 months of treatment,the clinical curative effect between the two groups have no significant difference(P>0.05).5.In terms of TCM syndrome curative effect,after 6 months of treatment,Fuzheng Jiedu prescription has significant efficacy on TCM syndrome of children with gene defect and normal gene.Among them,the TCM syndrome curative effect(mainly clinical recovery,effective and marked effect)of children with gene defect after 6 months of treatment is 90.91%,while that of children with gene normal after 6 months of treatment(including clinical recovery,marked effect and effective)is 87.5%.The difference had no significance(P>0.05).6.Fuzheng Jiedu prescription had a general effect on the improvement of the count of platelet in the two groups after 1,3 and 6 months of treatment,and the difference was not statistically significant(P>0.05).Before and after treatment,the two groups had no remarkable diffence(P>0.05).By ANOVA,the time corresponding to F=3.390,P=0.049<0.05,the count of platelet of the two groups at different time points were different,with statistical significance(P<0.05),the time*group corresponding to F=0.966,P=0.498>0.05,indicating that there was no interaction between time and grouping,the count of platelet between the two groups at different time points had no remarkable diffence.(P>0.05).In the whole course of treatment,with the passage of time,the count of platelet in the normal gene group was on the increse,and that in the gene defect group showed an ascent-descent-ascent tendency.Both groups were on the increse in the end of treatment,and in the normal gene group the increase amplitude was more evident than that in the gene defect group.7.Decrese of the integral of TCM syndrome:before treatment,there was no significant difference in the total integral of TCM syndrome and the scores of main and secondary symptoms between the two groups(P>0.05),and the two groups were comparable.Compared with before treatment,the total score of syndrome,main symptoms(purple spot bleeding,mental fatigue,pale complexion)and secondary symptoms(shortness of breath,self perspiration,less food,loose stool)of the two groups had a remarkable decrease after 6 months of treatment,and the difference had a statistically significance(P<0.05).By rank sum test,the total integral of TCM syndrome and the scores of main and secondary symptoms of the two groups had no significant difference(P>0.05).8.Improvement of haemorrhage grade:after 6 months of treatment,the effective rate(mainly recovery and marked effect)of haemorrhage grade improvement in gene defect group was 90.91%,and that in gene normal group was 87.5%.By chi square test,the two groups had no significant difference(P>0.05)9.Adverse reactions:the basic physical examination,urine routine test and biochemical function of the gene defect group and the gene normal group before and after treatment were normal,and the liver and kidney function were regularly monitored during the treatment,and there was no abnormality,and there was no obvious adverse reaction during the whole treatment.10.Follow up;the two groups were followed up by telephone or face-to-face consultation within 1 month after the completion of treatment.During the follow-up,there was no recurrence of bleeding symptoms or significant decrease of platelet count,and no other discomfort.ConclusionGene defects may be associated with ITP,age,gender,course of disease,positive family history and the count of platelet were not correlated with ITP gene defect.PLA2G4A,RTEL 1 and LRBA may be involved in ITP.Genetic testing can help clarify the diagnosis and prognosis of children with ITP.Gene defect has no significant effect on the clinical efficacy of Fuzheng Jiedu precrisption.Fuzheng Jiedu precrisption has certain clinical curative effect on ITP children with gene defect,can significantly improve TCM syndrome and reduce the risk of bleeding,and has high safety. |
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