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OBJECTIVE To explore the pharmaceutical service model in multidisciplinary diagnosis and treatment （MDT） of rare diseases in children. METHODS Clinical pharmacists of West China Second University Hospital （hereinafter referred to as “our hospital”） participated in the process of MDT of children’s rare diseases. Clinical pharmacists took part in the entire diagnosis and treatment process of children and established the MDT pharmaceutical service model of children’s rare diseases by formulating drug treatment plans based on evidence-based practice， improving the accessibility of drugs， pharmaceutical monitoring and drug treatment management. RESULTS From January 2021 to April 2022， clinical pharmacists of our hospital had participated in a total of 39 cases of rare diseases MDT in children， including 21 hospitalized children with rare diseases and 18 outpatient com children with rare diseases， involving a total of 23 rare diseases. Clinical pharmacists completed 45 pharmaceutical zhanglingli@scu.edu.cn rounds and 26 pharmaceutical consultations for rare diseases inpatients， 25 outpatients’ MDT and 5 pharmaceutical outpatient service for outpatients with rare diseases， 38 medication educations for inpatients and outpatients with rare diseases and 25 follow-up services for out-of-hospital patients. There were 24 cases （61.54%） of off-label drug use， involving 13 rare diseases and 16 therapeutic drugs， among which off-label drug use registration of 11 drugs had been completed or was in progress. The temporary purchase evaluations of 3 drugs had been completed； 268 cases of medical insurance drug and high-value drug prescription had been reviewed. CONCLUSIONS Our hospital have primarily established a loop pharmaceutical service model of MDT for children with rare diseases， which covers inpatients and outpatients. The model improves the availability and standardization of clinical application of therapeutic drugs， and diagnosis and treatment level for children with rare diseases in our hospital.
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Objective To evaluate the clinical application value of transesophageal atrial pacing (TEAP) combined with atropine load experiment in the diagnosis of the lesions of sinoatrial node and atrioventricular node.Methods One hundred and forty-four cases selected from the outpatient and hospitalized patients in the People's Hospital of Changji Hui Autonomous Prefecture from September 2009 to December 2012,who with dizziness, syncope and other clinical symptoms and electrocardiogram showe.TEAP combined with atropine load experiment were given to these patients.Results (1) The authors detected in all patients,83 cases (57.6％) were positive, among which, 48 cases (57.8％) male, 35 cases (42.2％) female.(2) The authors detected 57 cases(39.6％) non-increased vagus nerve tension cases in 83 positive cases,among which 33 cases (57.9％) male, 24 cases (42.1％) female;Among which 29 cases (20.1％) were sinoatrial node hypofunction, and 16 cases(55.2％) male;8 cases(5.6％) were atrioventricular node hypofunction,and 4 cases(50％) male;14 cases(9.7％) were double node hypofunction, and 10 cases (71.4％) male;6 cases (4.2％) were tachycardia-bradycardia syndrome, and 3 cases (50％) male;among which, a long interval of greater than 3 seconds appeared when we stimulate one 84 years old man with S1S1 stimulate way, immediately pressed protective pacemaker until his own sinus rhythm was restored, as a safety precaution, stoped further examination and classified him as sick sinus group.Conclusion Detect the common causes of slow sinus and atrioventricular block,such as the sinoatrial node dysfunction, atrioventricular node dysfunction, double node dysfunction and increased vagus nerve tension through TEAP combined with atropine load experiment.Consider that this methods have the best diagnostic value in decreasing its rate of false positivity,and should be used as a necessary check before implantation of pacemaker in such patients, suitable used in clinical, especially in basic general hospitals.
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Objective To discuss the clinical application value of transesophageal atrial pacing ( TEAP) in basic general hospitals.Methods A total of 212 subjects were selected,with dizziness,syncope,paroxysmal palpita-tions and other clinical symptoms and electrocardiogram showed sinus slow, atrioventricular block, short PR interval period,or with the first urgent symptom of tachycardia.The author doubted these symptoms were due to decreased function of the sinoatrial and atrioventricular node or Paroxysmal Supraventricular Tachycardia (PSVT),and treated them with TEAP with xd-5a.Results 121 cases (57.1%) were positive,83 cases(68.6%) of sinoatrial node dys-function,38 cases(31.4%) of PSVT,then sinoatrial node dysfunction were given atropine load experiment and double checked TEAP.The authors detected 57 cases of non-increased vagus nerve tension in 83 positive cases,29 cases (20.1%) of sinoatrial node hypofunction,8 cases of atrioventricular node hypofunction,14 cases of double node hypofunction,6 cases of tachycardia-bradycardia syndrome;38 cases(31.4%) of PSVT,20 cases of dual atrioven-tricular nodal pathways(DAVNP),12 cases of atrioventricular bypass,4 cases of atrial tachycardia(AT),2 cases of ventricular tachycardia( VT);12 cases from 38 cases of positive results manifested as the first urgent symptom of achy-cardia,included 5 cases of DAVNP,5 cases of atrioventricular bypass,overdrive pacing terminated tachycardia suc-cessfully,2 cases of male VT,1 case terminated tachycardia through giving intravenous amiodarone successfully,1 case was taken electrical cardioversion uccessfully.Conclusion The common causes of slow sinus and atrioventricular block and the common cardiac electrophysiological types of PSVT and VT were detected through TEAP examination with the best diagnostic and differential diagnostic value.According to diagnosis guide,clinicians adopted correct and effective treatment strategies with good clinical application value.The author suggest this methods should be used in clinical,especially in basic general hospitals.