1.Progress of diagnosis and treatment for aplastic anemia
International Journal of Pediatrics 2010;37(1):56-59
As the techruque of molecular biology developed, the research of aplastic anemia turns to the cytogenetical level. Scientists focused on irwestigating the relationship between the mutation of gene or chromosome and the curative effect of aplastic anemia.The results show that abnormality of particular chrromosome or gene will cause different outcome . On the other hand, the first line therapy of aplastic anemia is still IST or HSCT according to the different situation.The antibody special for T cell is still in studying, and the effect shows well .Through the typing diagnosis consummated, the treating method developed, the survival rate of the patient with apLastic anemia will be advanced.
2.Research progress in hereditary multiple exostoses
Journal of Clinical Pediatrics 2016;34(3):232-236
Hereditary multiple exostoses (HME) is an autosomal dominant genetic disease characterized by multiple benign cartilage-capped tumors primarily at the juxta-epiphyseal region of the long bone or on the lfat bones. Because the tumor can interfere with normal epiphysis, it causes bone deformities. The clinical features include short stature, the mechanical axis deviation, and function impairment. Recent studies showed that EXT gene mutation was associated with HEM. The EXT gene was involved in the biosynthesis of heparin sulfate. The gene mutations resulted in abnormal chondrocyte differentiation. This paper reviews the research progress in clinical manifestation, pathogenesis, biochemistry, the genotype-phenotype correlations, and treatment in HME.
3.Cognitive attitude of medical students towards standardized resident training in Shanghai in a university
Jingyan WANG ; Meng TANG ; Tong JIN
Chinese Journal of Medical Education Research 2012;(11):1183-1185
Objective To investigate the attitude of medical students towards standardized resident training in Shanghai and to provide references for policy adjustment.Methods Self-made questionnaire was used to investigate the attitude of 267 clinical medical students towards standardized resident training in Shanghai.Reference reviewing and expert consultation were also performed.Results Nearly eighty percent of students believed that they had basic knowledge of the training.Two thirds of students hold objective attitude towards the policy.Conclusions Income and pressure in finding jobs are the main reasons why students object the policy.Standardized resident training program need to improve in aspects of advertisement,incoming,quality of teaching staff,connection between medical education and training.
4.Survival and late effects of comprehensive treatment on children with neuroblastoma
Anan ZHANG ; Haoxian JIANG ; Jianmin XU ; Ci PAN ; Jingyan TANG
Chinese Journal of Applied Clinical Pediatrics 2017;32(15):1153-1157
Objective To investigate the organ function survival and late effects and secondary malignancy of childhood neuroblastoma (NB) with high intensity comprehensive treatment.Methods A total of 23 children with NB who received comprehensive treatment in Shanghai Children's Medical Center from December 1998 to October 2010 were enrolled.All tests were formulated according to the Children's Oncology Group (COG)'s guidelines with the approval of parents.The late side effects were graded by CTCAE v3.0,and hearing loss related to platinum was graded by Brock and Chang.Results The median follow-up was 96 (65-170) months.All the patients had at least one late side effect.The occurrence rate of 1-2 adverse effects was 100%,and 17.4% (4/23 cases) of patients had grade 3-4 adverse effects.90.5% of children (19/21 cases)developed hearing loss on both sides.Eleven children (52.4%,11/21 cases) developed dental abnormities,comprising microdontia,missing teeth,root stunting and enamel hypoplasia.9.5% of children (2/12 cases) had scoliosis.42.8% of children (9/12 cases) developed hypogonadism with the approval of parents or delayed growth.47.4% of children (9/19 cases) had abnormal respiratory function.36.8% of children (7/19 cases)had abnormal cardiac function.33.4% of children (7/21 cases)developed renal damage.23.8 % of children (5/21 cases) had abnormal liver function.66.7% of children (14/21 cases) had low level of cortisol,but adrenocorticotrophic hormone was normal.All children had normal function of thyroid.Three children developed secondary malignancy:leukemia,malignant fibroma and liver tumor.Conclusions The incidence of long-term adverse reactions of patients with neuroblastoma treated with high intensity comprehensive treatment is very high and the patients may have adverse effects,like hearing loss,dental abnormality,cardiopulmonary function abnormality,musculoskeletal problems and secondary malignancy.The upmost importance is to establish long-term follow-up program to detect the life quality of children and amend current therapeutic schemes improve life quality.
5.Clinical analysis of 14 cases of rare non-Hodgkin lymphoma in children
Mengxin CAI ; Ci PAN ; Min ZHOU ; Qidong YE ; Jingyan TANG
Chinese Journal of Applied Clinical Pediatrics 2016;(3):176-180
Objective To analyze the clinical characteristics and prognosis of 4 rare types of non -Hodgkin lymphoma(NHL)in children,and to discuss the progress in treatment.Methods Clinical data of 1 4 patients with rare types of NHL at Shanghai Children′s Medical Center,Shanghai Jiaotong University School of Medicine between January 2004 and December 201 4 were retrospectively analyzed,and their clinical features,treatment and prognosis were dis-cussed.Results Fourteen cases were reported including 6 subcutaneous panniculitis -like T -cell lymphoma (SPTCL),3 hydroa vacciniforme -like cutaneous lymphoma(HVLL),2 pediatric follicular lymphoma(PFL)and 3 ex-tranodal NK/T -cell lymphoma,and nasal type(ENKTL).Ten patients (71 .4%)primarily presented with skin lesions and underwent a long course of illness before they were finally diagnosed (the median was 1 0 months),71 .4%(1 0 /1 4 cases)of them associated with fever and 50.0%(7 /1 4 cases)with liver and spleen enlargement,and no evidence of central nervous system (CNS)and bone marrow (BM)involvement was observed,while 28.6% patients (4 /1 4 cases) had more than two lines′abnormalities in peripheral blood examination.Since there were no standard treatment guide-lines,most patients received CHOP (Cyclophosphamide +Adriamycin +Vincristine +Prednisone)and /or mature B -cell NHL -like therapy,and 50.0%(7 /1 4 cases)of them received interferon therapy in addition,while 1 patient re-ceived allogeneic hematopoietic stem cell transplantation after recurrence.The complete remission was achieved in 71 .4%(10 /14 cases)of all the patients.Two of them died,3 lost follow -up,and 1 relapsed.The 3 -year overall survi-val and event free survival rates were 0.84 and 0.57,respectively,after a median follow -up of 26 months (range 12 -64 months).Conclusions Pediatric rare types of NHL show atypical clinical manifestation,low incidence of CNS /BMinfil-tration and long course.It is hard to make pathological diagnosis and differentiation.It is also inappropriate to apply the commonly used staging system to these rare types of NHL.No standard treatment has been found by now.SPTCL,HVLL and PFL have relatively good outcomes when treated with mature B -cell NHL -type therapy plus interferon therapy.
6.Study on clinical prognosis among ETV6/RUNX1 positive childhood B-precursor acute lymphocyte leukemia
Xingwei WANG ; Benshang LI ; Shuhong SHEN ; Jing CHEN ; Jingyan TANG
Journal of Clinical Pediatrics 2016;34(5):321-325
Objective To investigate the incidence of the ETV6/RUNX1 fusion gene among Chinese pediatric patients with B-ALL and its effect on the prognosis. Methods A total of 723 patients with B-ALL from January 1, 2007 to December 31, 2014 were enrolled in this study. All patients were detected ETV6/RUNX1 fusion gene by FISH. Clinical data and ETV6/RUNX1 were combined to analyze the clinical prognosis. Results Among the 723 patients, 151 were with ETV6/RUNX1 positive B-ALL, accounting for approximately 20.89%(151/723) of B-precursor cases;91 patients were with recurrence, including 10 patients with ETV6/RUNX1 positive B-ALL, and the recurrence rate of ETV6/RUNX1 positive B-ALL was 10.99%(10/91). Among 10 recurrent patients with ETV6/RUNX1 positive B-ALL, 9 patients relapsed more than 300 days later after diagnosis, while the recurrence times among the patients with ETV6/RUNX1 negative was very different. Although the recurrence times between the two groups showed no signiifcant difference (P?=?0.09), the recurrence times of ETV6/RUNX1 positive patients were mainly found at the end of clinical chemotherapy, while the recurrence time of ETV6/RUNX1 negative patients were mainly at maintaining chemotherapy period, there was a signiifcant difference between the distribution of recurrence time (P?0.0001). Conclusions ETV6/RUNX1 fusion gene is a favorable predictor of outcome in Chinese pediatric B-ALL as well.
7.Early identification of sepsis in cancer children with neutropenia after chemotherapy
Yanwen YANG ; Ying WANG ; Jingyan TANG ; Biru LI
Chinese Pediatric Emergency Medicine 2012;19(4):383-386
Objective To investigate the clinical manifestations of the febrile neutropenia cancer children,explore the relationship between the clinical data and sepsis.Methods A prospective observation study was employed,and 66 cancer children complicated with febrile and neutropenia after chemotherapy were enrolled.Sixty-six cases were divided into two groups:septic group ( n =26 ) and non-septic group ( n =40).Clinical and laboratory data were collected and compared.Results Body temperature,neutropenia duration,absolute neutrophic count ( ANC ),C-reactive protein ( CRP ),procalcitonin (PCT) and culture positive rate showed statistically differences between the septic and non-septic groups ( P < 0.05 ).Body temperature >40 ℃,ANC < 0.1 × 109/L,increases of serum CRP and PCT levels and positive culture were correlated with sepsis.Body temperature < 39 ℃,neutropenia duration < 5 ds,ANC > 0.5 × 109/L were less correlated with sepsis.Conclusion High initial temperature,long duration of neutropenia,severely reduced ANC,increases of CRP and PCT,and culture-positive are correlated with sepsis in cancer children.
8.Asparagine synthetase is partially localized to the plasma membrane and upregulated by L-asparaginase in U937 cells.
Yingyi, HE ; Benshang, LI ; Changying, LUO ; Shuhong, SHEN ; Jing, CHEN ; Huiliang, XUE ; Jingyan, TANG ; Longjun, GU
Journal of Huazhong University of Science and Technology (Medical Sciences) 2011;31(2):159-63
This study investigated the intracellular localization of asparagine synthetase (ASNS) in the relation with chemoresistance in leukemia. pIRES-GFP-ASNS-Flag/Neo expression vector was transiently tansfected into SK-N-MC cells and 297T cells respectively. Immunofluorescence and Western blot analysis were performed for cellular localization of ASNS respectively. U937 cells were treated with L-asparaginase for 48 h and examined for endogenous ASNS expression on plasma membrane by immunofluorescence staining. Immunofluorescence staining showed that the transiently expressed ASNS was partly localized on transfected-SK-N-MC cell surface. Moreover, Western blotting exhibited that ASNS expressed both in cytosol and on plasma membrane of transfected-293T cells. Immunofluorescence staining with anti-ASNS-specific monoclonal antibody revealed that endogenous ASNS was localized on the plasma membrane of U937 cells, except for its distribution in the cytosol. In addition, ASNS exhibited a higher expression on plasma membrane after treatment with L-asparaginase as compared with the untreated cells. It was concluded that the subcellular translocation of ASNS may play an important role in L-asparaginase resistance in leukemia cells.
9.Long term follow-up of children with neuroblastoma without chemotherapy and radiotherapy
Jie ZHAO ; Ci PAN ; Min XU ; Min ZHOU ; Yijing GAO ; Wenting HU ; Jingyan TANG
Journal of Clinical Pediatrics 2017;35(9):678-681
Objectives To analyze the clinical features and long-term follow-up results of neuroblastoma (NB) without chemotherapy and radiotherapy, and to provide evidence for further improvement of treatment. Methods The clinical data of children diagnosed with NB who received operation during January 2005 to December 2015 was retrospectively analyzed, and the long-term follow-up results were evaluated. Results In 57 cases of NB, 43 cases (81.1%) were in stage 1, 8 cases were in stage 2 and 2 cases were in stage 4S. The median age at diagnose was 7 months (11 days - 10 years and 11 months). There were 47/51 cases had the pathological type with a good prognosis (accounting for 92.2%). FISH was detected in 1/49 case which had the amplification at greater than 10 copies. 56/57 cases underwent surgical resection of the primary tumor, 50 cases of which were completely resected, and 3 cases had very good partial remission after tumor resection. The abdominal mass was found in the uterus in 1 case, and surgical operation was not performed and the imaging was regularly checked for follow-up , and the mass subsided completely at 7-month-old. The median follow-up time was 36 months (4 - 99 months). Five children were lost to follow-up and the median time of follow-up was 19 months (4 - 45 months). One child in stage 4S relapsed at 1 year of follow-up, 2 cases in stage 1 relapsed at 6 months of follow-up. Five years event free survival rates (EFS) in all patients were 94.6%, and overall survival rate (OS) of the 5 years was 100%. Conclusions Children younger than 18 months without MYCN amplification in the stages 1 and 2 are safe by surgical treatment alone with good prognosis. Simple surgical treatment can also be extended to all age groups of NB without MYCN amplification in the stages 1 or 2.
10.Study on the clinical outcomes of children with stage Ⅳ malignant extracranial germ cell tumors
Qianghua YAN ; Jingyan TANG ; Ci PAN ; Qidong YE ; Min ZHOU ; Yijin GAO ; Wenting HU
Journal of Clinical Pediatrics 2017;35(5):321-324
Objective To evaluate the outcomes of children with stage Ⅳ malignant extracranial germ cell tumors. Methods Twenty-five patients were enrolled in the retrospective analysis. Event-free survival (EFS) and overall survival (OS) rates were estimated by Kaplan-Meier method with SPSS 13.0. Results Of the 25 children, there were 13 males and 12 females. The mean age at diagnosis was 2 years old (ranged 1 to 11). Five patients receiving chemotherapy in another hospital before (n=1), or giving up treatment after confirmed diagnosis (n=1), or giving up effective treatment after received less than 2 cycles (n=3) were excluded from this analysis. Of the 20 patients, 90.0% (18/20) achieved complete remission and 5.0% (1/20) achieved partial remission after treatment. The 5-year EFS rate and 5-year OS rate were 70.0%±10.2% and 82.4%±9.2% respectively. There was no death occurred due to complications. Conclusions The effect of this treatment program is positive. The cumulative dose of the drugs is not high, compared with other schemes such as PEB, but there are more drugs involved. Whether these drugs may cause long-term adverse reactions needs further research.