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06 April 2016, Volume 31 Issue 4 Previous Issue    Next Issue

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Research in the diagnosis and treatment of childhood leukemia：A model in practising precision medicine WANG Tian-you，LI Zhi-gang 2016, 31(4): 241-245.  DOI: 10.7504/ek2016040601 Abstract ( )   Great progress has been made in treatment outcome of children with leukemia. Recently， clinical classification has been more accurate，resulting from discovery of many molecular genetic anomalies. High throughput sequencing  has made it possible to identify minimal residual disease at very low level. New therapy approaches，such as small molecule targeted drugs， monoclonal antibodies， and chimeric antigen receptor modification，bring hope to the treatment of refractory/relapsed leukemia. Pharmacogenetics research， represented by the polymorphisms of 6-mercaptopurine metabolism related genes， has made great contribution to the toxicity reduction. The progress deeply reflects the concepts and essence of precision medicine. Furthermore， the results lay a solid foundation for future research.

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Advances in management of childhood acute myeloid leukemia YU Jiao-le， ZHENG Hu-yong 2016, 31(4): 246-252.  DOI: 10.7504/ek2016040602 Abstract ( )   Acute myeloid leukemia（AML） accounts for about 20% of childhood leukemia with the cure rate of more than 65%. Although dramatic improvement in treatment of childhood AML has been achieved based on the optimization of risk classification，risk-based therapeutic regimen，monitoring and supportive care，currently，the prognosis of pediatric AML has reached plateau due to the heterogeneity of AML. Targeted therapy based on the precise genotyping and comprehensive treatment will improve the cure rate and quality of life of children with AML.

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Progress in diagnosis and treatment of childhood acute lymphoblastic leukemia of central nervous system ZHAO Yan-xia， SUN Li-rong 2016, 31(4): 252-256.  DOI: 10.7504/ek2016040603 Abstract ( )   Childhood acute lymphoblastic leukemia is the first disseminated cancer shown to be curable. Central nervous system leukemia （CNSL） is one of reasons that cause leukemia replase and is associated with a poor prognosis. Pediatricians should master the diagnostic techniques，modalities of prophylaxis and therapy of CNSL in patients with childhood acute lymphoblastic leukemia.

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Recent progress in the diagnosis and treatment of infant leukemia HU Qun 2016, 31(4): 257-260.  DOI: 10.7504/ek2016040604 Abstract ( )   The leukemia in infants is rare. Because of its unique biology and poor response to current therapies， it is still a major challenge for pediatric hematologists and oncologists worldwide. Pediatricians should know well about the epidemiologic， clinical and biologic features， prognosis and treatment of infant leukemia.

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Controversy and consideration on the treatment of acute lymphoblastic leukemia with Philadelphia chromosome-positive in children GUO Ye，ZHU Xiao-fan 2016, 31(4): 260-264.  DOI: 10.7504/ek2016040605 Abstract ( )   The development of tyrosine kinase inhibitors（TKIs） has revolutionized the therapy of pediatric Philadelphia chromosome-positive acute lymphoblastic leukemia（Ph+ALL）. The prospects for a favorable therapeutic outcome in patients with Ph+ALL have improved markedly， as demonstrated in a recent study that has combined intensive chemotherapy with TKIs. HSCT is no longer universally recommended for Ph+ALL. These would give rise to our thinking of the therapy.

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Progress of minimal residual disease detection in guiding the treatment of leukemia GUO Qing，JIN Run-ming 2016, 31(4): 264-268.  DOI: 10.7504/ek2016040606 Abstract ( )   Although in recent years the effect of the treatment of leukemia is increasingly improved， recurrence due to minimal residual disease（MRD） is still a big problem. Recently the study on MRD detection in acute lymphoblastic leukemia is better， but the research of MRD in other types of leukemia needs to be improved. There are two main techniques of MRD detection：molecular biology technique，such as PCR and molecular immunology technique，such as the flow cytometry （FCM）. Clinicians should be aware of the progress in the detection of MRD in leukemia.

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Study of asparaginase in the treatment of acute lymphoblastic leukemia SHI Pei-jie，FANG Jian-pei 2016, 31(4): 268-274.  DOI: 10.7504/ek2016040607 Abstract ( )   Asparaginase（Asp） is a critical agent used to treat acute lymphoblastic leukemia（ALL）. The basic information about Asp in ALL treatment such as efficacy and side effect，the administration of different kinds of Asps at home and abroad. As well as the three suggestions should be mastered by pediatricians for the improvement of the outcome of the ALL treatment.

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Application of chimeric antigen receptor gene modified T cell therapy in the treatment of childhood leukemia CHEN Xiao-juan，ZHU Xiao-fan 2016, 31(4): 274-277.  DOI: 10.7504/ek2016040608 Abstract ( )   The development of biotechnology has spawned a new generation of cell therapy represented by chimeric antigen receptor gene modified T cells（CART）. The treatment for refractory and relapsed childhood leukemia is a formidable clinical problem. CART cell therapy has preliminary improved outcome，which is initiating a new idea for the treatment.However，the long-term efficacy and safety of CART cell therapy needs to be further observed.

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Ph-like acute lymphoblastic leukemia SHEN Shu-hong 2016, 31(4): 278-281.  DOI: 10.7504/ek2016040609 Abstract ( )   Ph-like acute lymphoblastic leukemia（ALL） is a recently defined subgroup of leukemia with gene expression profile and prognosis similar to Ph chromosome positive ALL. Genomics study has revealed Ph-like ALL is associated with mutations happening to cytokine receptor， tyrosine kinases and Ras family. Kinase inhibitors targeting the mutated molecules have already benefited several patients with Ph-like ALL.

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Advances of hematopoietic stem cell transplantation in the treatment of childhood leukemia LIU Xiao-ting，LI Chun-fu 2016, 31(4): 281-286.  DOI: 10.7504/ek2016040610 Abstract ( )   Hematopoietic stem cell transplantation（HSCT） is an important method in the treatment of childhood leukemia. The indication of HSCT is different according to the typies of leukemia and donor. In the past a matched sibling donor（MSD） was the first choice，however，the results in HSCT from unrelated donor（MUD） have already been close to those from MSD transplant now，and so，haploidentical HSCT for leukemia has been increasingly accepted. A lot of cell therapies have gradually shown the advantages. The advances of HSCT in the treatment of leukemia are important for the clinicians.

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Individualized treatment for childhood acute lymphoblastic leukemia: Gene polymorphisms of TPMT and MTHFR YE Qi-dong，GU Long-jun 2016, 31(4): 286-291.  DOI: 10.7504/ek2016040611 Abstract ( )   The development in the areas of pharmacogenetics has provided ample examples of causal relations between genotypes and drug response. The studies of gene polymorphism raise the hope of individualized treatment. So the genotyping of thiopurine S-methyltransferase and methylenetetrahydrofolate reductase might be useful in treatment for childhood acute lymphoblastic leukemia to optimize 6-mercaptopurine and methotrexate therapy， which will be possible to have effects on survival.

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Significance of EBF1 gene copy number abnormalities in pediatric B-ALL LIU Xiao-ming，ZHANG Li，ZOU Yao，CHEN Yu-mei，RUAN Min，GUO Ye，CHEN Xiao-juan，YANG Wen-yu，WANG Shu-chun，LIU Tian-feng，ZHANG Jia-yuan，LIU Fang，QI Ben-quan，ZHU Xiao-fan 2016, 31(4): 292-296.  DOI: 10.7504/ek2016040612 Abstract ( )   Objective    To identify EBF1 gene copy number abnormalities in pediatric B-ALL patients and to further investigate its value in the pathogenesis and prognosis. Methods    A total of 195 children with B-ALL whose bone marrows could be extracted enough DNA for the detection were selected retrospectively. All the patients were diagnosed and systematically treated according to CCLG-2008 in Department of Pediatrics，Institute of Hematology and Blood Diseases Hospital，CAMS and PUMC from April 2008 to April 2013. The 195 cases were divided into two groups according to the results of EBF1 gene copy number abnormalities by MLPA： the group with EBF1 deletion and the group without EBF1 deletion. Results    Fifteen（7.7%） out of 195 B-ALL patients had EBF1 deletion. There were no differences between the two groups in clinical characteristics at diagnosis（all P＞0.05）. Patients with EBF1 deletions had a lower 4-year disease-free survival（DFS） ［（59.5±14.8）% vs. （85.5±3.2）%，P＝0.020］ and event-free survival （EFS） ［（55.6±14.3）% vs. （84.2±2.9）%，P＝0.017］ compared to those without. The overall survival rate had no statistical difference between the two group ［（86.7±8.8）% vs. （91.9±2.1）%，P＞0.05］. Excluding many influencing factors，Cox analysis showed that EBF1 deletion still affected the patients’DFS and EFS（P＜0.05）. Conclusion    Some of pediatric B-cell precursor ALL can be detected EBF1 deletion；And EBF1 deletion is an independent poor prognosis factor to the patients’DFS and EFS.

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Cost-effectiveness analysis of partially hydrolysed formula-whey on eczema prevention in infants without allergic family history FENG Mei，XIAO Yi-zhu，LUO Xiao-yan，HU Yan 2016, 31(4): 297-300.  DOI: 10.7504/ek2016040613 Abstract ( )   Objective    To compare the incidence of eczema in infants without allergic family history and in those fed with partially hydrolysed formula-whey （pHF-W） or standard formula （SF），and to preliminarily discuss the preventive cost-effectiveness of those two formulas. Methods    Totally 50 healthy term newborns without allergic family history  born between June 2013 and April 2014 in Department of Obstetrics，the First Affiliated Hospital of Chongqing Medical University were enrolled. The incidence of infantile eczema and total cost for eczema were recorded during the 12 months of follow-up period.Cost-effectiveness of pHF-W vs. SF was compared according to the cost-effectiveness ratio. The infantile eczema was diagnosed by dermatologists according to Guidetines for Diagnosis and Treatment of Eczema in China. Results    The incidence of eczema in the infants was 54.0%（27/50） until 12 months old. There was no statistical significance of eczema incidence in the infants fed with pHF-W or SF（50.0% vs. 56.3%，P＞0.05）. Compared with SF，the good cost-effectiveness in pHF-W for eczema prevention did not exist in these infants，and the difference of cost-effectiveness ratio was ¥2003.2/person. Conclusion    There is no significant difference of eczema incidence in infants without allergic family history fed with pHF-W or SF in the first 4 months. egarding the cost-effectiveness，pHF-W is not superior to SF in eczema prevention.Hence，preventing infantile eczema by pHF-W seems not a wise choice in present medicare situation of China.

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Characteristics of body composition and the relativity between fat mass and lean mass with bone mineral density of low weight and obese children aged from 5 to 7 years old LIAO Xin，MU Ya-ping 2016, 31(4): 301-304.  DOI: 10.7504/ek2016040614 Abstract ( )   Objective    To analyze characteristics of body composition and the relativity on percentage of body fat（PBF），fat mass（FM），lean mass（LM） with the local and systemic bone mineral density（BMD） of low weight and obese children aged from 5 to 7 years old. Methods     A total of 311 children aged from 5 to 7 years old were divided into three groups according to gender and body mass index（BMI），the low weight group，the control group and the obesity group，who came to Department of Child Healthcare of Shengyang Children’s Hospital for roution physical examination from February 2010 to February 2012 were selected as the subjects of the study. The body composition were measured by dual energy X-ray absorptiometry（DEXA）. Data had been compared between groups and correlation had been analysised among PBF，FM，LM and BMD. Results    In obesity group， the LM，upper limbs，lower limbs and systemic BMD in boys were significantly higher than those in girls（P＜0.05）. In control group，girls’s PBF，FM were significantly higher than boys’. LM， upper limbs， lower limbs and systemic BMD in girls were lower than those in boys（P＜0.05）. In low weight group，when FM was controlled，there was positive correlation between LM and BMD（P＜0.01）. When LM was controlled，there was positive correlation among PBF，LM and lower limbs BMD in obesity girls（P＜0.05）. When FM was controlled，there was significantly positive correlation between LM and lower limbs BMD（P＜0.01）. When FM was controlled，there was positive correlation between LM and lower limbs BMD in obesity boys（P＜0.05），When LM was controlled，there was positive correlation among PBF，FM and truncal BMD（P＜0.05）. Conclusion    The proportion is different from controls in low weight and obese children， and the contribution to BMD is different. It should not only pay attetion to the change of body weight，but also pay attetion to the changes of PBF，FM，LM in body composition，so as to promote the healthy development of the bone.

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Combined radiation sensitive immunodeficiency disease：A clinical analysis of 3 cases HE Jian-xin, WU Run-hui, XU Xi-wei, et al 2016, 31(4): 305-307.  DOI: 10.7504/ek2016040615 Abstract ( )

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One case of 17β-hydroxysteroid dehydrogenase type 10 deficiency and the literature review DUAN Li-fen, BAO Xin-hua, ZHANG Qing-ping 2016, 31(4): 308-310.  DOI: 10.7504/ek2016040616 Abstract ( )

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Value of auxiliary examination in the diagnosis of autism spectrum disorders QU Ling-ling, CHENG Qian 2016, 31(4): 311-314.  DOI: 10.7504/ek2016040617 Abstract ( )

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Childhood anti N-methyl-D-aspartate receptor encephalitis：A report of 3 cases ZHAO Li-li, LUO Rong 2016, 31(4): 315-317.  DOI: 10.7504/ek2016040618 Abstract ( )

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Childhood central cord syndrome without fracture or dislocation：A report of 1 case LIU Lu-qiong, NA Yu-hui, MO Ya-xiong 2016, 31(4): 318-320.  DOI: 10.7504/ek2016040619 Abstract ( )