持续性、慢性免疫性血小板减少症患儿基因特征及扶正解毒方治疗的回顾性研究

张晓倩; 余惠平; 胡淼; 何冰

doi:10.3969/j.issn.2095-6606.2022.05.001

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持续性、慢性免疫性血小板减少症患儿基因特征及扶正解毒方治疗的回顾性研究

临床研究 | 更新时间：2022-10-17

- 持续性、慢性免疫性血小板减少症患儿基因特征及扶正解毒方治疗的回顾性研究
- A retrospective study on the genetic characteristics of children with persistent and chronic ITP and the efficacy of Fuzheng Jiedu Decoction
- 现代中医临床 2022年29卷第5期页码：1-6
- 作者机构：
  
  1.北京中医药大学东直门医院　北京　100700
  2.湖北省妇幼保健院
- 作者简介：
  
  张晓倩，女，在读硕士生
  #何冰，男，硕士，主治医师，E-mail：bricebird@163.com
- 基金信息：
  
  北京市科技计划课题项目(Z171100001017224)
- DOI：10.3969/j.issn.2095-6606.2022.05.001
  中图分类号： R272.6
- 纸质出版日期：2022-09-30，
  
  收稿日期：2021-11-09，
- 稿件说明：
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张晓倩, 余惠平, 胡淼, 等. 持续性、慢性免疫性血小板减少症患儿基因特征及扶正解毒方治疗的回顾性研究[J]. 现代中医临床, 2022,29(5):1-6.

ZHANG Xiaoqian, YU Huiping, HU Miao, et al. A retrospective study on the genetic characteristics of children with persistent and chronic ITP and the efficacy of Fuzheng Jiedu Decoction[J]. Modern Chinese Clinical Medicine, 2022,29(5):1-6.
张晓倩, 余惠平, 胡淼, 等. 持续性、慢性免疫性血小板减少症患儿基因特征及扶正解毒方治疗的回顾性研究[J]. 现代中医临床, 2022,29(5):1-6. DOI： 10.3969/j.issn.2095-6606.2022.05.001.

ZHANG Xiaoqian, YU Huiping, HU Miao, et al. A retrospective study on the genetic characteristics of children with persistent and chronic ITP and the efficacy of Fuzheng Jiedu Decoction[J]. Modern Chinese Clinical Medicine, 2022,29(5):1-6. DOI： 10.3969/j.issn.2095-6606.2022.05.001.

摘要

目的

探讨持续性、慢性免疫性血小板减少症(ITP)经一线治疗效果欠佳患儿的基因特征，评价扶正解毒方对存在致病基因突变患儿的临床疗效。

方法

将40例患儿按照是否存在与血小板减少相关的致病基因突变，分为突变组17例和对照组23例。观察基因测序检查结果、临床疗效、血小板计数、出血分级、中医证候积分和中医证候疗效。

结果

40例患儿中，共有17例(占42.50%)患儿存在与血小板减少相关的致病或疑似致病基因突变，关联疾病包括遗传性血小板减少症、原发性免疫缺陷症、血管性血友病等。治疗3个月后，2组患儿临床疗效比较差异无统计学意义(

0.05)。同一时间点2组患儿的血小板计数差异无统学意义(

0.05)，不同时间点差异有统计学意义(

0.05)，随着治疗时间的增加，血小板计数也逐渐增加。治疗1个月开始，2组中医证候积分逐渐下降，不同时间点间差异有统计学意义(

0.05)；2组中医证候积分差异无统计学意义(

0.05)；组别和时间的交互作用无统计学意义(

0.05)。治疗3个月后，2组患儿出血分级改善情况、中医证候疗效比较差异均无统计学意义(

0.05)。

结论

一线治疗依赖或无效的持续性、慢性ITP患儿应积极完善基因测序检查，进行临床诊疗再评估。扶正解毒方具有较好的提升血小板计数的作用，在改善出血症状及中医证候方面有效率较高。

Abstract

Objective

To investigate the genetic characteristics of children with persistent and chronic immune thrombocytopenia (ITP) who did not respond well to first-line treatment

and to evaluate the clinical efficacy of Fuzheng Jiedu Decoction (Healthy Qi Reinforcing and Toxin Resolving Decoction) on children with pathogenic gene mutations.

Methods

The 40 children were divided into a mutation group of 17 and a control group of 23 according to their presence or absence of pathogenic gene mutations associated with thrombocytopenia. The result of gene sequencing

clinical efficacy

platelet count

bleeding grade

TCM syndrome score and TCM syndrome efficacy were observed.

Results

Among the 40 children

17 (42.50%) had pathogenic or suspected pathogenic gene mutations related to thrombocytopenia

and related diseases included HT

PID

and von Willebrand disease. After 3 months of treatment

there was no significant difference in the clinical efficacy between the two groups (

0.05). There was no statistically significant difference in platelet count between the two groups at the same time point (

0.05)

but there was a statistically significant difference at different time points (

0.05). With the increase of treatment time

the platelet count gradually increased. After 1 month of treatment

the TCM syndrome scores of the two groups gradually decreased

and the difference was statistically significant at different time points (

0.05); there was no significant difference in the TCM syndrome scores between the two groups (

0.05); the interaction between group and time was not statistically significant (

0.05). After 3 months of treatment

there was no significant difference in the improvement of bleeding grade and the efficacy of TCM syndromes between the two groups (

0.05).

Conclusions

Children with persistent and chronic ITP who have grown dependency on first-line treatment or for whom first-line treatment has proven to be ineffective should actively improve gene sequencing and re-evaluate clinical diagnosis and treatment. Fuzheng Jiedu Decoction has a good effect of increasing platelet count

and is more effective in improving bleeding symptoms and TCM syndromes.

关键词

儿童免疫性血小板减少症基因检测扶正解毒方临床疗效

Keywords

childrenimmune thrombocytopeniagenetic testingFuzheng Jiedu Decoctionclinical efficacy

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