Department of Pediatrics of Tongji Hospital Affiliated to Tongji University, through med-ical school practice teaching demonstration ward construction project, obtained remarkable achievements in perfecting project construction plan, paying attention to the implementation of project tasks and improving the teaching quality and personnel training, and in improving the teaching quality of the sustainable devel-opment condition and so on.

Childhood myelodysplastic syndromes(MDS) is a disease with lower incidence and very difficult in clinical diagnosis and treatment.The Chinese expert consensus for the diagnosis and treatment of childhood myelodysplastic syndromes recently recommended by Subspecialty Group of Hematology,the Society of Pediatrics,Chinese Medical Association,is a high academic significance of guidance documents to improve the efficacy of diagnosis and treatment,as well as relevant research of children with MDS in our country.This paper combined with the relevant lite-rature review at home and abroad in recent years and summed up the experience of clinical diagnosis and treatment,in order to contribute the promotion of this consensus of the experts.

According to the principles on the continuing medical education by Ministry of Health and all the problems which may exist in continuing education classes currently held in our country,the author conducted in-depth thinking and meaningful reform practice in the following areas of teaching direction and content scope,teaching and class scale,teaching methods and examination system,etc.and summarized the experience and achievements in improving the quality of the continuing medical education and effective technology promotion,as well as the promotion of medical education career and subject construction development.

Based on the analysis of the recent possible problems of diagnosis and treatment in the ifeld of childhood hema-topoietic diseases, this paper provides a brief interpretation on recent recommendations or guidelines for children with non-ma-lignant hematological diseases issued by the Chinese Pediatric Hematology Group, including iron deifciency anemia, immune thrombocytopenia, hemophilia, beta thalassemia and aplastic anemia.

Aplastic anemia (AA)is a serious disease of hematological malignant disease in children.Severe AA is difficult to treat and may lead to high motality.Acquired AA accounts for 95% of all cases,so it′s significant to lucubrate the pathogenesis of acquired AA for clinic diagnosis and therapy.The documents in recent 5 years are collected,and a reviews about the progress of pathogenesis in acquired AA children is provided.

Aplastic anemia is a severe hematological disease characterized by bone marrow failure and pancyto-penia.Immunosuppressive therapy(IST)with antithymocyte globulin(ATG)and cyclosporine A(CSA)is the first -line treatment for patients who lack an HLA -matched sibling.One third of the patients has no response to IST and the high risk of ATG -related adverse effects may lead to ATG -related death.So it is necessary to explore the predictive marker of response to IST.A defined predictive marker may make the selection of treatment more reasonable and further improve the long -term efficacy.The relevant literatures were analyzed and summarized in recent years,in order to pro-vide a reference for clinical treatment and a baseline for prospective studies.

Acquired aplastic anemia is a refractory blood disease for children,and severe acquired aplastic anemia can be a great threaten for life.China is a high incidengce area,children at a period during which this disease can mostly occur.In China,except some congenital aplastic anemia,most children who have this diease is acquired aplastic anemia.In 2009,the Britain society of hematology recommended Guidelines for the diagnosis and management of aplastic anaemia,which represented the latest diagnosis and treatment principle and mainstream view of the clinical methodology about aplastic anemia.They make it clear that the main treatment for the disease are allogeneic hematopoietic stem cell transplantation(allo-HSCT) and immunosuppressive therapy(IST).This article collects the documents in recent 5 years,and provides a overview about the latest advance of the allo-HSCT and IST in the word.

Objective To improve the early diagnosis of Wiskott-Aldrich syndrome (WAS),through summing up and analyzing the clinical characteristics of children with WAS.Methods The clinical and laboratory characteristics of 18 children with WAS,including the cases from Suzhou Jiulong Hospital,Shanghai Jiaotong University School of Medicine,and Tongji Hospital Affiliated to Tongji University,and the domestic reports in recent 6 years,were induced and analyzed.Results All patients were males;the average of incidence age was about 1.9 months and the average age at diagnosis was 10.8 months.All cases had the clinical characteristics of WAS including eczema,infection tendency and thrombocytopenia with small platelet size.Moreover,10/18 cases (55.56％) WAS patients progressed to severe cases (scores reached to 4 or 5 points)at diagnosis,and with the increase of age of the patients at diagnosis,the ratio of severe cases would be increased.The conventional immune function indexes showed no specific diagnostic value,and the typical clinical features and the WAS gene mutation detection were the key diagnostic basis.Seven cases used to be misdiagnosed as idiopathic thrombocytopenia,and received corticosteroids and intravenous immunoglobulin therapy.In a total of 18 cases,4 cases received allogeneic hematopoietic stem cell transplantation,of which,β cases were cured and 1 case died of transplantation-related interstitial pneumonia,while the remaining 14 cases are unknown for their with follow-up treatment and prognosis.Conclusions The typical clinical features and the WAS gene mutation detection were the key diagnostic basis.In order to improve the rate of early diagnosis and avoid misdiagnosis,it's a great need to improve the clinical understanding of WAS.

Objective To analyze and summarize the characteristics of Dyskeratosis congenita(DC)in Chinese children,so as to provide reference for early diagnosis and reasonable prevention and treatment of DC.Methods The data collected from 43 cases with DC in the domestic literature in recent 10 years,as well as the clinical data of 2 cases with DC treated at Tongji Hospital,Medical School,Tongji University,and a total of 45 cases with DC were analyzed and compared with those reported in the literatures of foreign countries.Results Based on the detailed data of 2 typical cases at Tongji Hospital,Medical School,Tongji University,and the data of 45 cases in China were summarized,so the major differences in the main characteristics of DC between Chinese Children and the foreigners were as follows:(1)Onset were earlier(mean age 4.5 years,median age of 3 years),but the diagnosis was delayed(mean age of 17.9 years,median age of 18 years).(2)The presence of skin pigmentation,nail lesions and mucosal leukoplakia,such as the proportion of the complete DC triad was higher(42/45 cases,93.3％).(3)There was an earlier onset of hematopoietic suppression(mean age 5.6 years).(4)Telomerase related gene mutation types were relatively minor,DKC1(7 cases)and TINF2(6 cases)were reported in recent years,and no other type of mutation was found.(5)Effective therapy of hematopoietic reconstitution was administered in 2 cases after allogeneic hematopoietic stem cell transplantation(allo-HSCT).The effective rates were about 70％(7/10 cases)in treating bone marrow failure with low dose androgen and low dose glucocorticoid.Conclusions DC is very common in infants in China,the clinical manifestations of triad are more typical,but the age of diagnosis was significantly delayed.Improving the understanding of DC and combination with the detection of related gene mutation may improve the early diagnosis rate and clinical efficacy with allo-HSCT or effective drug maintenance therapy,and also provide reference for propitious familial eugenics and prenatal examination.

Objective To explore the application value of PBL combined with medical simulation training in pediatric clinical practice for long-schooling program medical students.Methods Totally 41 long-schooling program medical students in pediatric department were taken as study group while 40 medical students as control group.PBL combined with medical simulation training was used in study group while traditional method was used in control group.Teaching effect was evaluated using score analysis,PBL learning performance rating scale and student questionnaire.Results Scores of theory test and clinical skill exam after course completion in study group were significantly higher than those of control group (P ＜ 0.01).PBL learning performance rating scale and student questionnaire showed that the student's comprehensive ability has significantly improved.Conclusions Application of PBL and medical simulation training in the pediatric clinical practice for long-schooling program medical students can enhance students' problem analyzing and summarizing abilities,problem-solving ability and practical operation ability.It can effectively cultivate team collaboration and doctor-patient communication skills as well as improve the quality of teaching.