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06 November 2014, Volume 29 Issue 11 Previous Issue    Next Issue

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Recognition of the diagnostic significance of erythrocyte parameters in childhood anemia. ZHU Xiao-fan， CHANG Li-xian. 2014, 29(11): 801-804.  DOI: 10.7504/ek2014110601 Abstract ( )   PDF (913KB) ( )   Abstracts： More attention should be paid to erythrocyte parameters， which provide an important basis for the diagnosis of anemia in children. Analysis of erythrocyte parameters should take full account of the influence of sample collection and detection system. Detecting the alteration of erythrocyte parameters contributes to the diagnosis and differential diagnosis of anemia in children， such as hypochromic microcytic anemia and macrocytic anemia.

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Diagnostic approach to childhood anemia. GUO Xia， GAO Ju. 2014, 29(11): 805-810.  DOI: 10.7504/ek2014110602 Abstract ( )   PDF (1068KB) ( )   Abstract：Anemia，a very common clinical symptom，adversely affects the health of anemic patients and hinders socioeconomic development. Nevertheless， anemia is not an independent disease entity， but rather a constellation of symptoms， resulting from a great variety of causes and underlying disorders. Obviously， it is very important to follow established diagnostic steps when approaching a child with anemia. This aids greatly in the documentation of underlying etiology and formulation of management of anemia. In the present paper， following a brief review of anemia definition and classification， we primarily focus on the diagnostic steps of anemia and present the diagnostic flowcharts for microcytic， normocytic and macrocytic anemias respectively.

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Principles of treatment  for congenital hemolytic anemia ZHANG Zhi-quan，JIN Run-ming. 2014, 29(11): 810-814.  DOI: 10.7504/ek2014110603 Abstract ( )   PDF (1017KB) ( )   Abstract：Congenital hemolytic anemia （CHA） is a hereditary disorder with main clinical presentation of hemolytic anemia．It is the most common disease of hereditary disorders in the world. According to the pathogenic mechanism，the disorders can be divided into three groups：abnormalities of the red cell membrane，red cell enzyme disorders and hemoglobinopathies.The most common CHA are hereditary spherocytosis，G-6-PD deficiency and the thalassemias．This article focuses on the management of these three types of diseases.

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Therapy selection of autoimmune hemolytic anemia in children. ZHAO Yan-xia，SUN Li-rong. 2014, 29(11): 814-818.  DOI: 10.7504/ek2014110604 Abstract ( )   PDF (958KB) ( )   Abstract：Autoimmune hemolytic anemia （AIHA） is an uncommon acquired immune disorder. The classification of AIHA is based on the pattern of the direct antiglobulin test and on the immunochemical properties of the autoantibody， but also on the presence or absence of an underlying condition or disease. Different type of AIHA has different treatment and outcome. Treatment for AIHA has long been empirical and first line therapy is corticosteroids， especially for warm AIHA （wAIHA）.For relapsed or refractory AIHA， second-line therapy，such as rituximab，may be a good alternative and has been more reported in childhood patients. In this article， the classification and the recent progress in therapies for AIHA are discussed to provide treatment recommendations for pediatricians.

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Treatment for beta-thalassemia with hematopoietic stem cell transplantation. XU Hong-gui，FANG Jian-pei. 2014, 29(11): 818-822.  DOI: 10.7504/ek2014110605 Abstract ( )   PDF (966KB) ( )   Abstract：Allogeneic hematopoietic stem cell transplantation （HSCT） is the only curative therapy for severe beta-thalassemia major. Patients with an available human leukocyte antigen （HLA） identical sibling donor should be offered HSCT as soon as possible before development of iron overload and transfusion associated complications， and the cure rate was up to 80%～90%. High-resolution HLA typing matched unrelated donor transplants also achieved good results. HSCT from HLA-mismatched relatives or HLA-phenotypically-identical donor is an option to be performed in expert centers. Optimization of conditioning regimen and techniques to control transplant-related complications will further improve outcomes.

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Prevention and treatment for iron overload in patients with β-thalassemia. LUO Xue-qun. 2014, 29(11): 823-826.  DOI: 10.7504/ek2014110606 Abstract ( )   PDF (920KB) ( )   Abstract：Organ failure especially heart failure due to iron overload is the main cause of death in patients with β-thalassemia. Recently the prognosis of the patients has significantly improved. This attributes to the new understanding related to the disease， including iron overloading and its diagnosis， as well as the characteristics of iron chelators and their reasonable application in treatment.

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Management of anemia in prematurity. SHEN Jing，DING Guo-fang. 2014, 29(11): 826-828.  DOI: 10.7504/ek2014110607 Abstract ( )   PDF (891KB) ( )   Abstract：Anemia of prematurity is one of the main problems of premature infants.This article summarized the definition， pathogenesis， clinical and laboratory features of anemia of prematurity，and mainly introduced the treatment，including RBC transfusion，iron supplementation and usage of erythropoietin（EPO）.

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Interpreting “Recommendation for diagnosis and treatment of acquired aplastic anemia in children”. XIE Xiao-tian. 2014, 29(11): 829-833.  DOI: 10.7504/ek2014110608 Abstract ( )   PDF (931KB) ( )   Abstracts：“Recommendation for diagnosis and treatment of acquired aplastic anemia in children”recommend by The Hematology Group of Chinese Pediatrics Medical Committee and Chinese Journal of Pediatrics in 2014 is of greater reference value in academic guidance document and will be well worth learning and referring in practice. This paper appropriately summarized and discussed the contents closely related to clinical practice and experience.

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Refractory cytopenia of childhood. ZHU Xiao-fan. 2014, 29(11): 834-836.  DOI: 10.7504/ek2014110609 Abstract ( )   PDF (888KB) ( )   Abstracts： Refractory cytopenia of childhood （RCC） is proposed by WHO 2008 MDS tentative classification based on morphology.This article expounds the classification system of children's MDS and diagnostic criteria of RCC. There are 75 percent of the RCC cases characterized by low bone marrow hyperplasia， so it is necessary for differential diagnosis with children acquired aplastic anemia.

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A multicenter clinical study on the treatment for children’s acute bronchiolitis by nebulized recombinant human interferon α 1b. SHANG Yun-xiao*, HUANG Ying,LIU En-mei, CHEN Qiang, CAO Ling, LU Min, ZHAO De-yu, WANG Ying, LIU Han-min， YU Jing, LI Yu-ning, WU Cheng-qing. 2014, 29(11): 840-844.  DOI: 10.7504/ek2014110611 Abstract ( )   Abstract： Objective To evaluate effectiveness and safety of treatment for children’s acute bronchiolitis by nebulized recombinant human interferon α 1b in a multicenter clinical study，and to recommend a reasonable clinical dosage for children. Methods With the design of a randomized, controlled, multi-center study, totally 330 children who were hospitalized from December 2012 to May 2013 for acute bronchiolitis were randomly divided into three groups. The control group had routine and symptomatic treatment. Therapy groups inhaled human recombinant interferon α1b 2 μg/kg and 4 μg/kg respectively, twice a day plus routine treatment, for 5 to 7 days. We used four-level-scoring method to evaluate disease conditions and performed pathogen detections. Results （1）The total improvement rates of low and high dose group were 92.3% and 95.0%, respectively, which were significantly higher than control group （85.3%, P<0.05）. High dose group had obviously improved primary index （wheezing, wheezing rale and three depression sign） compared with low dose group. The disappearance time of symptoms was shortened significantly （P<0.05）, and the improvement of three depression signs was obvious （P<0.01）.（2）Treatments within and after 72 h were both effective, but the total improvement rate within 72 h was better.（3）The effectiveness of interferon α1b in RSV positive children was significantly better than in RSV negative children. Effectiveness of treatment group was superior to that of control group.（4）All children did not show irritation symptoms in local respiratory tracts, and no serious adverse reactions was observed. Conclusion The treatment for acute bronchiolitis in young children by nebulized human recombinant interferon α1b is effective, which shortened the duration of symptoms, with no safety problem. The effect is better when used in the early stage.

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A multiple-center clinical study on quantitative T2* magnetic resonance imaging of iron deposition of liver， pancreas and heart of beta-thalassemia major patients. LIU Si-xi*，WANG Ying，LI Chang-gang，LI Chun-fu，WU Xue-dong，FANG Jian-Pei，CHEN Guang-fu，GAO Hong-ying，HAO Wen-ge，LIU Ri-yang， CHEN Guo-hua， ZHANG Xin-hua，ZHU Zhao-ying，AU Wing-yan. 2014, 29(11): 845-848.  DOI: 10.7504/ek2014110612 Abstract ( )   Abstracts： Objective To study the T2* magnetic resonance imaging for evaluation of iron deposition of liver， pancreas and heart in β- thalassemia major （β-TM） patients and the relationship of the serum ferritin with iron overload. Methods Measurement of hepatic， pancreatic and cardiac MRI T2* was performed in 153 patients with β-TM from May 1， 2010 to December 31， 2010．The demographics and serum ferritin （SF） were collected. The rate of the iron overload in liver， pancreas and heart was analyzed. The rates of combined pancreatic and cardiac iron overload in different hepatic iron overload groups were compared. Results According to MRI T2*， there was serious hepatic iron overload in 85 cases， moderate in 39， mild in 21， no iron overload in 8. Similarly， there was pancreatic iron overload in 134， no iron overload in 19. In terms of cardiac iron overload， there was serious cardiac iron overload in 37 cases， mild in 21， no iron overload in 95. No correlation was found between hepatic， pancreatic and cardiac MRI T2* and age or SF， but hepatic MRI T2* correlated with pancreatic and cardiac MRI T2*（P = 0.000， r = 0.529， r = 0.369）， and pancreatic MRI T2* correlated with cardiac MRI T2* well （P = 0.000， r = 0.715）. As grade of hepatic iron overload increases， the patients demonstrated higher rate of combination of pancreatic and cardiac iron overload simultaneously （χ2 = 20.78，P = 0.000）.

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MiR-125b regulates pediatric classical APL cells proliferation，apoptosis and drug resistance by targeting Bak1. WANG Li-na，HUANG Li-bin，LIANG Yan-ni，ZHANG Xiao-li，KE Zhi-yong，LUO Xue-qun. 2014, 29(11): 849-854.  DOI: 10.7504/ek2014110613 Abstract ( )   Abstract：Objective To explore the role of miR-125b in pediatric classical APL， in order to seek new therapeutic strategies for drug resistant APL. Methods The target genes of miR-125b were predicted online， validated by Dual-luciferase assay and western blot assay. MiR-125b expression levels were measured in 33 matched-pair APL samples（treated in the First Affiliated Hospital of Sun Yat-sen University and other members of South China Children APL Cooperative Group from March 2007 to September 2012）at initial diagnosis and complete remission （CR） and in 5 relapsed patients by qRT-PCR. Proliferation and apoptosis were analyzed respectively using the RNA transfection， MTT assay and flow cytometry. Results The expression of miR-125b was up-regulated in pediatric APL at diagnosis and relapse bone marrow samples， but returned to normal after complete remission； miR-125b could promote leukemic cell proliferation and inhibit cell apoptosis by regulating the expression of tumor suppressor Bak1. Remarkably，it was also found to be up-regulated in leukemic drug-resistant cells（NB4-R1、NB4-R2及HL-60/DOX）， and overexpression of exogenous miR-125b could increase their resistance to therapeutic drugs. Conclusion MiR-125b can regulate pediatric classical APL cells proliferation， apoptosis and drug resistance  by repressing BAK1 protein expression.

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Prevalence and factors associated with diarrhea among infants in Shanghai. XIAO Li-ping,WANG Xin-qiong， HUANG Jun， LI Yun， WANG Xiao-jin， XU Chun-di*. 2014, 29(11): 855-859.  DOI: 10.7504/ek2014110614 Abstract ( )   Abstract：Objective To explore the prevalence of diarrhea among infants in Shanghai and to analyze the risk and protective factors of diarrhea. Methods A multicenter， population-based cohort study was carried out in 12 communities in Minhang district in Shanghai from July 2011 to April 2013. A total of 6661 infants with first visit at the community hospitals from July 2011 to April 2012 were enrolled in the study. Birth history， feeding modes， sociodemographic characteristics and diarrhea morbidity were collected every 3 months from 0 to 12 months in these infants. Results Among the 6661 infants， 1.3% had diarrhea within 0～3 months， 3.0% within 4～6 months， 3.9% within 7～9 months， and 4.4% within 10～12 months. The incidence rate among these infants with diarrhea was 0.13 episodes per person-year. Family income， feeding types， caregivers， level of maternal education and original residence area were associated with diarrhea. Conclusion The incidence rate of diarrhea among infants in Shanghai has been decreased these years. Family income， feeding types， caregivers， level of maternal education and original residence area are considered to be predictors of diarrhea.

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Value of the single luteinizing hormone determination 1 hour after GnRHa treatment in therapy monitoring of gonadotropin-dependent precocious puberty in girls. CAI Xi-ding，LI Zhen，BAI Min，ZHU Bei. 2014, 29(11): 860-864.  DOI: 10.7504/ek2014110615 Abstract ( )   Abstract： Objective To determine the value of luteinizing hormone determination 1 hour after GnRHa treatment in monitoring GnRHa therapy for girls. Methods From January 2009 to January 2014， 20 girls received GnRHa to suppress central puberty for 6～24 months in Maternal and Child Health Hospital of Zhoushan， who had under gone a GnRH stimulation test before therapy. Fourteen girls received GnRH stimulation test 3 months after initiation of therapy. All of the 20 girls were given LH determination 1 h after the third GnRHa treatment . In order to define appropriate level of LH， a clinical score for pubertal suppression were developed by using Tanner staging， skeletal maturation， and growth velocity，to analyze the best correlation between this clinical score and the value of LH. Results Adequate hypothalamic-pituitary-gonadal axis suppression during treatment was achieved in 19 of the 20 girls according to the clinical parameters and LH levels. In these 19 well-controlled girls， the LH peak after GnRH test was compared with that of a single LH measurement obtained 1 h after GnRHa administration before and during GnRHa treatment. Before therapy， the LH peak after GnRH test was （13.8±5.7） U/L （7～24.5 U/L）， and （21.7±7.3） U/L 1 h after the first GnRHa administration （10.0～35.3 U/L） . During therapy， the LH peak after GnRH test was （1.2±0.5） U/L（ 0.6～2.0 U/L）， and （1.4±0.6） U/L（0.5～2.5 U/L） 1 h after GnRHa administration. The LH peak after GnRH test and that of 1 h after GnRHa administration correlated significantly before and during treatment. One girl presented evidence of pubertal progression and the value of LH 1 h after the third GnRHa administration was 4.5 U/L . Conclusion We establish the LH cut-off values for GnRHa therapy as an LH peak below 2.0 U/L after GnRH test or below 2.5 U/L 1h after GnRHa administration. The latter measurement may replace the GnRH test as a useful and convenient tool in monitoring therapy in female gonadotropin-dependent precocious puberty.

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Comorbidity in school-aged children with high-functioning and low-functioning autism disorder. YU Ming，LIU Jing，LI Xue， JIA Mei-Xiang. 2014, 29(11): 865-870.  DOI: 10.7504/ek2014110616 Abstract ( )   Abstract： Objective To investigate the occurrence of comorbidity in school-aged children with high-functioning and low-functioning autism disorder. Methods Sixty-two outpatients in Peking University Institute of Mental Health， aged 6 to 16 years old， meeting the criteria of the Diagnostic and Statistical Manual of Mental Disorders， Fourth Edition（DSM-IV） visiting the hospital for autism were included in this cross-sectional study from September 2011 to November 2011. They were assessed with the self-made general condition questionnaire and Chinese Wechsler Intelligence Scale for Children （WISC）. Raven’s Standard Progressive Matrices（SPM），Stanford-Binet Intelligence Scale and Peabody picture vocabulary test（PPVT） were performed for patients who could not perform WISC test）. Kiddie-Sade-Present and Lifetime Version （K-SADS-PL） ， Child-Global Assessment Scale（C-GAS ）and Childhood Autism Rating Scale（CARS） were also performed. Results The lifetime prevalence and current prevalence of comorbidity in high-functioning autism（HFA） group（IQ≥70）were 100%（29/29） and 96.5%（28/29） respectively. The lifetime prevalence and current prevalence of comorbidity in low-functioning autism（LFA） group（IQ＜70）were all 100%（33/33）. The lifetime prevalence and current prevalence of mood disorders， anxiety disorders， generalized anxiety disorder， attention deficit hyperactivity disorder， oppositional defiant disorder and tic disorders in high-functioning autism group were higher those of low-functioning autism group （all P＜0.05）. High-functioning autism group had more psychiatric medication taking history and poor development history， higher current percentage of attending school，and lower CARS score and higher C-GAS score than low-functioning autism group （all P＜0.05）. Conclusion Comorbidity in school-aged children with high-functioning and low-functioning autism disorder are very common. Children with high-functioning autism disorder have more emotional and behavior disorders， milder symptoms of autism， more psychiatric medication taking history， and higher overall function than children with low-functioning autism disorder.

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Clinical study of 28 childhood myelodysplastic syndrome. LIN Ming*， XIAO Sheng-jun， JIN Run-ming. 2014, 29(11): 871-873.  DOI: 10.7504/ek2014110617 Abstract ( )   Abstract： Objective To analyze the clinical data and laboratory examination of childhood myelodysplastic syndrome（MDS）and discuss the key point of early diagnosis. Methods Twenty-eight patients from Pediatric Hematology Department of Union Hospital， Tongji Medical College， Huazhong University of Science and Technology with MDS from March 2005 to August 2009 were retrospectively analyzed. Results The diagnosis in 4 patients was RA， 3 patients was RAEB， and 1 patient was RAEB-t. Conclusion The diagnosis of MDS mainly depends on the bone marrow examination. Flowcytometric immunophenotyping and fluorescence in situ hybridization examination （FISH） can facilitate accurate diagnosis ，and they are also helpful for the early diagnosis and differential diagnosis of MDS.