Objective:To analyze the neurodevelopmental characteristics of the children with methylmalonic acidemia and investigate its possible influencing factors.Methods:The clinical questionnaire was used to collect clinical data of children with methylmalonic acidemia at the First Affiliated Hospital of Henan University of Chinese Medicine from February 2016 to February 2018, and Gesell development scal was used to evaluate the neurodevelopment.Results:Among the 86 patients, 45 cases were boys and 41 cases were girls.They were 2.4 to 62.5 months, and the minimum age was 8.3 months.Thirty-three cases were detected by neonatal screening, 53 cases were diagnosed after onset.The energy zone in this children were mild to severe developmental defects.Twenty-eight cases were 0 to 6 months old, and fine exercise, the large exercise and adaptability developmental defects were significant of them; 24 cases were >6 to 12 months old, and the language, adaptability and personal-social developmental defects were significant of them; 23 cases were >12 to 36 months old, and the language, adaptability and large movements developmental defects were significant of them; 11 cases aged >36 to 72 months had significant developmental defects in language, personal-social contact and adaptability.The developmental quotients of fine movements, big movements, adaptability, personal-social and language of the children diagnosed by newborn screening were much better than those diagnosed after onset in each energy zone(all P<0.05). Conclusions:Although the treatment is actively regulated, most children with methylmalonic acidemia still have neurodevelopmental abnormalities with varying degrees of severity.The children before the age of 6 months have significant defects in motor movement and adaptability development.The patients after the age of 6 months usually have language, adaptability and social contact defects.Newborn screening is important and nervous system damage is milder in the children diagnosed by newborn screening.

为了培养规范化培训医师在儿科工作中良好的岗位胜任力,结合中医药大学中西医结合儿科学科特点及自身带教经验,通过培养本学科的专业理论知识、临床诊断思维能力、临床实践能力、职业素养等方面进一步提高规范化医师的临床能力,成长为更优秀的临床儿科医生,使其毕业后在岗位上能快速适应各用人单位的需求.

良好的医患沟通是实现和谐医患关系的基础,加强住院规培医师医患沟通能力的培养,对改善医患关系有着重要的意义,有利于医患双方相互正确理解对方、协调关系,是保证诊疗活动顺利进行的前提.文章结合儿科肾脏病的临床特点及自身带教经验,探讨中医药大学中规培医师在医患沟通能力培养方面存在的问题及解决措施,以期建立更加和谐的医疗环境,使规培医师成长为更优秀全面的临床医生.

目的:观察升清降浊制动颗粒对多发性抽动症(tourette syndrome,TS)模型大鼠纹状体内多巴胺(dopamine,DA)水平及多巴胺转运蛋白(dopamine transporter,DAT)、多巴胺D2受体(dopamine DZ receptor,DRD2)mRNA表达的影响.方法:将70只雄性SD大鼠随机分为空白对照组,模型组,氟哌啶醇组,升清降浊制动颗粒高剂量组、低剂量组,每组14只.采用3,3'-亚氨基二丙腈(3,3'-Iminodipropionitrile,IDPN)制备TS模型大鼠.从造模完成次日开始,空白对照组、模型组给予等体积生理盐水灌胃,氟哌啶醇组、升清降浊制动颗粒高剂量组、升清降浊制动颗粒低剂量组分别给予氟哌啶醇稀释液[200 μg·kg-1]、升清降浊制动颗粒高剂量悬液[2.58 mg·g-1]、升清降浊制动颗粒低剂量悬液[1.29 mg·g-1]治疗,第21天结束后取材.采用高效液相色谱-四级杆离子阱串联质谱仪(HPLC-MS/MS Q-TRAP)检测纹状体中DA水平,RT-PCR检测纹状体中DAT、DRD2 mRNA表达水平.结果:与空白对照组比较,模型组运动行为评分、刻板行为评分均显著升高,差异均有统计学意义(P＜0.05).与模型组比较,治疗后高剂量、低剂量升清降浊制动颗粒均能降低IDPN诱导的TS模型大鼠运动行为评分、刻板行为评分,差异均有统计学意义(P＜0.05).与空白对照组比较,模型组DA水平显著降低,差异有统计学意义(P＜0.05).与模型组比较,治疗后高剂量、低剂量升清降浊制动颗粒均能升高IDPN诱导的TS模型大鼠DA水平,差异均有统计学意义(P＜0.05).与空白对照组比较,模型组DAT mRNA表达显著降低,DRD2mRNA表达显著升高,差异均有统计学意义(P＜0.05).与模型组比较,治疗后高剂量、低剂量升清降浊制动颗粒均能升高DAT mRNA表达,降低DRD2mRNA表达,差异均有统计学意义(P＜0.05);具有升高纹状体DA含量及上调DAT mR-NA、下调DRD2 mRNA表达量的作用(P＜0.05).结论:升清降浊制动颗粒能改善IDPN诱导的TS模型大鼠行为异常,其机制可能与升高纹状体DA水平及上调DAT mRNA表达、下调DRD2 mRNA表达有关.

Objective: To analyze the regularity of professor Zheng Qizhong,s application of Fengchi Qichi Dianostic Method.Methods: 30 clinical cases of syndrome of endogenous liver wind,cough,diarrhea and anorexia were collected respectively and the data was logged in the Traditional Chinese medicine auxiliary platform,and two syndromes of each diseases were selected.Results: For Syndrome of endogenous liver wind,patients with insufficiency of the spleen and hyperactivity of liver show navy and purple Qichi,while syndrome of phlegm-fire disturbing mind shows red and purple Qichis.For cough,patients with wind-cold syndrome show navy and reddish Qichi,while phlegm-heat syndrome shows red and purple Qichis.For diarrhea,patients with spleen deficiency syndrome show yellow Qichi,while dampness-heat syndrome shows red and yellow Qichi.For anorexia,patients with dysfunction of spleen in transportation show yellow Qichi,while dampness-heat syndrome shows red and purple Qichi.The colour and lustre changes of Fengchi is in accordance with that of the Fengchi in one patient,while the changes of Qichi occurs more frequently and obviously.Conclusion: The colour and lustre changes of Fengchi Qichi in different diseases and syndromes follow some rules,which direct the syndrome differentiation in the diagnosis of many diseases.Inspection method of Fengchi Qichi is one of the characteristics,which compensates for the weakness of pediatric inspection.