Engraftment syndrome / 中国小儿急救医学

Engraftment syndrome (ES) is a clinical syndrome that occurs after hematopoietic stem cell transplantation and during the recovery process of neutrophils.The main clinical manifestations include non-infectious fever, rash, capillary leakage and non-cardiogenic pulmonary edema, which may be similar with many early complications after transplantation.Therefore, it is sometimes difficult to be diagnosed and differentiated among different kinds of complications.Typical ES is self-limiting and has good response to steroids.However, patients with ES may result in encephalopathy and multi-organ failure if it is untreated without notice.In this review, we discussed the pathophysiological mechanisms, clinical manifestations, diagnosis and differential diagnosis, risk factors, treatment and prognosis of ES, aiming to provide guidance and reference for clinicians.

Expression of programmed death-1 and programmed death-ligand 1 in post-transplant lymphoproliferative disorders after allogeneic hematopoietic stem cell transplantation in children / 中华实用儿科临床杂志

Objective:To explore the expressions of programmed death-1 (PD-1) and programmed death-ligand 1 (PD-L1) and clinicopathological characteristics in post-transplant lymphoproliferative disorder (PTLD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) in children, with the aim of clarifying whether checkpoint inhibition of PD-1/PD-L1 inhibitors may serve as a therapy option.Methods:The clinical data of 13 cases of PTLD after allo-HSCT pathologically confirmed in Shenzhen Children′s Hospital from January 1, 2012 to December 30, 2019 were retrospectively analyzed.The detection was performed by immunohistochemical staining by MaxVision? method, Epstein-Barr virus(EBV) in situ hybridization and lymphoma gene rearrangement.The relationship between the expression of PD-1 and PD-L1 and the clinicopathological characteristics of PTLD were analyzed.Results:The expression of PD-1 was not correlated with gender, age, primary diseases, histopathological types, transplantation mode and the expression of EBV in situ hybridization (all P>0.05). The expression of PD-L1 was correlated with histopathological types ( P<0.05). Furthermore, the expression rate of PD-L1 on severe β-thalassemia was significantly higher than that of severe aplastic anemia [90.0%(9/10 cases) vs. 66.7%(2/3 cases)] and monomorphic PTLD was higher than that of polymorphic PTLD [100.0%(2/2 cases) vs. 83.3%(5/6 cases)]. Moreover, the positive PTLD in EBV was higher than the negative PTLD in EBV [90.9%(10/11 cases) vs. 50.0%(1/2 cases)]. The positive rates of PD-1 and PD-L1 in 13 cases with PTLD were 46.2%(6/13 cases) and 61.5%(8/13 cases) in tumor cells, 92.3% (12/13 cases) and 76.9% (10/13 cases) in microenvironmental cells, and 84.6%(11/13 cases) in EBV, respectively. Conclusions:PD-L1 has a higher positive rate in tumor cells with monomorphic PTLD; and routine staining for PD-1 and PD-L1 can be performed in all types of PTLD when standard immunotherapy and chemotherapy are ineffective.

Clinical and molecular genetic study on 21 children with lissencephaly / 中华实用儿科临床杂志

Objective:To study the clinical features and molecular genetic mechanisms of children with lissencephaly (LIS), as well as to analyze the relationship between genotypes and phenotypes of the disease.Methods:From October 2016 to December 2017, the clinical data and follow-ups of 21 LIS children were collected in the Department of Neurology, Shenzhen Children′s Hospital.Whole genome sequencing (WGS) was performed for genetic testing.Results:Among these 21 cases, 18 cases developed epilepsy (86%), and 3 cases were seizure free (14%). The onset age of children with epilepsy was relatively young, and 16 cases occurred within 1 year old (89%). Among these cases, 16 were pachygyria (76%), 3 cases were agyria combined with pachygyria (14%) and 2 cases were agyria (10%). Epileptic syndromes included 12 cases of West syndrome (67%), 2 cases of Ohtahara syndrome (11%), 2 cases of other epileptic encephalopathy (11%), and 2 cases of focal epilepsy (11%). Brain magnetic resonance imaging(MRI) demonstrated that most cases were pachygyria, among which diffuse pachygyria was more common (56%, 9/16 cases). The results of WGS: 13 pathogenic or likely pathogenic single nucleotide variants (SNV) and copy number variants (CNV) were detected.The total detection rate was 62%, of which 2 cases were frameshift, 1 case was nonsense and 1 case was missense variants of PAFAH1B1, 6 cases were chromosome 17p13.3 deletion syndrome, thus lea-ding to the whole gene deletion of PAFAH1B1, and 1 case was missense variant of DCX, frameshift variant of KIF2A, and missense variant of PIK3R2, respectively.Totally, 48% (10/21 cases) of the cases were variants or deletions of PAFAH1B1, which resulted in lissencephaly in the parietal-occipital region of the brain.Novel variants were PAFAH1B1: c.1067G>A, PAFAH1B1: c.897delT and KIF2A: c.2225delG. Conclusions:Most cases of LIS accompanied with epilepsy, in which West syndrome was relatively more common.Brain MRI showed that most cases were diffuse pachygyria.The variants and deletions of PAFAH1B1 was the main genetic cause of LIS.The identification of the novel variants expanded the genotypical spectrum of LIS.

Examining geographical disparities in the incubation period of the COVID-19 infected cases in Shenzhen and Hefei, China

BACKGROUND@#Current studies on the COVID-19 depicted a general incubation period distribution and did not examine whether the incubation period distribution varies across patients living in different geographical locations with varying environmental attributes. Profiling the incubation distributions geographically help to determine the appropriate quarantine duration for different regions.@*METHODS@#This retrospective study mainly applied big data analytics and methodology, using the publicly accessible clinical report for patients (n = 543) confirmed as infected in Shenzhen and Hefei, China. Based on 217 patients on whom the incubation period could be identified by the epidemiological method. Statistical and econometric methods were employed to investigate how the incubation distributions varied between infected cases reported in Shenzhen and Hefei.@*RESULTS@#The median incubation period of the COVID-19 for all the 217 infected patients was 8 days (95% CI 7 to 9), while median values were 9 days in Shenzhen and 4 days in Hefei. The incubation period probably has an inverse U-shaped association with the meteorological temperature. The warmer condition in the winter of Shenzhen, average environmental temperature between 10 °C to 15 °C, may decrease viral virulence and result in more extended incubation periods.@*CONCLUSION@#Case studies of the COVID-19 outbreak in Shenzhen and Hefei indicated that the incubation period of COVID-19 had exhibited evident geographical disparities, although the pathological causality between meteorological conditions and incubation period deserves further investigation. Methodologies based on big data released by local public health authorities are applicable for identifying incubation period and relevant epidemiological research.

Effects of miR-200c-3p on proliferation and apoptosis of nephroblastoma SK-NEP-1 cells / 中华实用儿科临床杂志

Objective:To investigate the effect of miR-200c-3p on the proliferation and apoptosis of nephroblastoma SK-NEP-1 cell and its mechanism.Methods:From January 2015 to August 2019, nephroblastoma tissue and peritumoral tissue of 30 patients in Shenzhen Children′s hospital were collected.The experimental group of mimic negative control, miR-200c-3p and miR-200c-3p inhibitor was set up.The expression of miR-200c-3p in 30 paired nephroblastoma tissues and adjacent kidney tissues were detected by real-time fluorescence quantitative PCR.The differential expression of miR-200c-3p was also detected in SK-NEP-1 and 293FT cell lines.The effects of miR-200c and miR-200c-3p inhibitor on the proliferation, cell cycle distribution and apoptosis of SK-NEP-1 cells were detected by cell counting kit-8(CCK-8)and flow cytometry assays, respectively.Xenograft tumors were generated by peri-renal adipose tissue injection to assess the effect of miR-200c-3p on tumor growth in vivo.The pathological morphology of xenograft tumors was observed by HE staining.The proliferation index of Ki-67 were detected by immunohistochemistry.Western blot method was used to detect the B-cell lymphoma 2(Bcl-2), Bcl-2 related X protein (Bax) and cleaved cysteinyl aspartate specific proteinase-3(Caspase-3)expression level on xenograft tumors. Results:The expression level of miR-200c-3p in nephroblastoma(0.420±0.587)was significantly lower than that in matched normal kidney(1.500±0.504)( t=8.613, P<0.001). The expression level of miR-200c-3p in SK-NEP-1 cells (0.363±0.006) was significantly lower than that in human embryonic kidney 293FT cells (0.807±0.186) ( t=4.136, P<0.05). The group and time interaction results of CCK-8 proved that miR-200c-3p could inhibit the proliferation of SK-NEP-1 cells( F=16.81, P<0.001). The flow cytometer test of cell cycle and apoptosis showed that miR-200c-3p could block in G0/G1 and S phase( t=-7.770, P<0.01; t=11.501, P<0.001). Moreover, it increased the early apoptosis rate and decreased the late apoptosis rate ( t=-22.270, P<0.001; t=4.612, P<0.01). A orthotopic transplantation assay was employed to evaluate the effect of miR-200c-3p and miR-200c-3p inhibitor on the proliferation of SK-NEP-1 cells.The final volume of tumor miR-200c-3p group [(0.419±0.16) cm 3]was significantly lower than those in the control group [(2.469±0.914) cm 3, t=0.507, P<0.001]. However, the miR-200c-3p inhibitor group had no significant difference [(1.627±0.189) cm 3; t=2.209, P=0.052]. miR-200c-3p overexpression upregulated expression levels of apoptotic proteins cleaved Caspase-3 and Bax ( t=-47.000, -82.730, all P<0.001), but downregulated the expression level of anti-apoptotic protein Bcl-2( t=53.740, P<0.001). Conclusions:The overexpression of miR-200c-3p can inhibit the proliferation, promote the apoptosis of the SK-NEP-1 cells and partially inhibited tumorigenicity of nude mouse acted as a tumor suppressor gene.

A resting-state functional magnetic resonance imaging study of altered brain function of benign epilepsy in children with central-temporal spikes / 中华实用儿科临床杂志

Objective:To analyze the brain functional fluctuation of benign epilepsy in children with central-temporal spikes(BECTS) by using ReHo algorithm based on the resting-state brain functional imaging, and to explore the connection of the brain function and changes of the connection pattern, so as to find the damage of the cognitive function of BECTS children in the early stage.Method:s Perspectiveness and simple random selection of 20 BECTS children and 20 healthy control children admitted to Shenzhen Children′s Hospital from January 2015 to December 2017 were conducted for basic information collection and functional magnetic resonance imaging (fMRI) testing in a resting-state.Result:s Significantly lower ReHo value appeared in the default mode network (DMN) area, and the precuneus (voxel=422, t=-5.085 6), cuneus (voxel=85, t=-4.240 3), angular gyrus (voxel=191, t=-4.681 2), cingulate cortex (voxel=313, t=-5.238 2), anterior central gyrus (voxel=12, t=-3.482 7), and supplementary motor area (voxel=1 356, t-6.596 2). The significantly increased ReHo was found in the bilateral cerebellum (voxel=71, t=5.658 2), right superior temporal gyrus (voxel=24, t= 5.184 0), operculum insulae (voxel=337, t=6.814 9), left parietal lobe (voxel=12, t=4.378 7), and inferior parietal lobule (voxel=11, t=3.433 7). Conclusions:Significant impairment of DMN, Wernicke and angular gyrus functions in BECTS children may be one of the mechanisms of cognitive dysfunction.Enhanced sensorimotor area and cortical brain functions near the operculum insulae and central sulcus lead to seizures with typical clinical symptoms.fMRI has a high specificity and sensitivity for evaluating the brain function of children with BECTS, and it can detect the impairment of cognitive function in children with this type of epilepsy at an early stage.

Updates on human equilibrative nucleoside transporter 1 in neoplastic diseases / 国际儿科学杂志

The human equilibrative nucleoside transporter 1(hENT1)is a multi-integrated membrane protein that mediates the transport of nucleosides, nucleobases and nucleoside analogs.It is mainly distributed on the cell membrane in humans.hENT1 maintains the homeostasis of cells and the intracellular concentration of nucleoside drugs through facilitating diffusion, thereby affecting DNA and RNA synthesis, cell signaling, metabolic regulation and tumor chemotherapy.This article reviews the progress in the structure and function of hENT1, the distribution in tumor tissues, the nucleoside analogs response and the prognosis of neoplastic diseases.

The expression of TCRP1 in chronic myeloid leukemia cells and its effects on cell proliferation / 中国医师杂志

Objective To investigate the effects of tongue cancer resistance-associated protein 1 (TCRP1) in proliferation of chronic myeloid leukemia cells (CML),and explore the new thoughts of pathogenesis of CML.Methods The expression of TCRP1 was detected in the peripheral blood mononuclear cells (PBMC) of CML with real-time quantitative polymerase chain reaction (PCR) and Western blot.After the expression of TCRP1 was interfered in K562 cells,the proliferation of cells was detected by 3-(4,5-dimenthylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay and soft agar colony forming assay,and the expression of protein kinase B (AKT) and its phosphorylation were tested by Western blot.Results In PBMC of CML patients,the mRNA and protein levels of TCRP1 were significantly higher than those of normal controls.The results of MTS assay and soft agar colony forming assay showed that the proliferation of K562 cells was significantly decreased after the expression of TCRP1 was interfered.After knockdown of TCRP1 in K562 cells,the phosphorylation of AKT was significantly decreased while the expression of total AKT did not change.Conclusions The expression of TCRP1 was increased in CML cells.High expression of TCRP1 might contribute to proliferation of K562 cells via the phosphorylation of AKT.

Variation in genes related to lipid metabolism and the susceptibility of nonalcoholic fatty liver disease in obese children / 临床儿科杂志

Objective To explore the association between variation in genes related to lipid metabolism and the susceptibility of nonalcoholic fatty liver disease (NAFLD). Methods Obese children with fatty liver aged 6~18 years old were included. All of them got ultrasonic testing, routine examination and biochemical detection. In addition, the DNA of peripheral blood was extracted and the 36 target genes related to lipid metabolism were detected by next generation sequencing. Results In 368 obese children who met the inclusion criteria, 183 children (49.7%) were detected to have NAFL . 100 children with NAFLD and 100 children without NAFLD were randomly selected from obese children. The levels of body mass, waistline, alanine aminotransferase (ALT), triacylglycerol (TG), cholesterol, low density lipoprotein (LDL) and apolipoprotein B (ApoB) in NAFLD children were all higher than those in non-NAFLD children, and there were significant differences (P all0.05). The levels of bilirubine in the two groups were within normal range. Logistic regression analysis showed that the genes of MTTP rs2306986 (OR=2.70, 95%CI: 1.38~5.27) and MTTP rs3792683 (OR=7.34, 95%CI: 2.04~26.50) that encode microsomal triglyceride transfer protein (MTTP or MTP), and the mutation of rs738409 (OR=2.11, 95%CI:1.31~4.48) in gene PNPLA3 that encode patatin-like phospholipase domain-containing protein 3 are the independent risk factors for the occurrence of the disease. Conclusion Genovariation of MTTP rs2306986, MTTP rs3792683, and PNPLA3 rs738409 may increase susceptibility to NAFLD in children.

Homology of methicillin-resistant Stphylococcusaureus isolated from neo-nates,health care workers,and environment / 中国感染控制杂志

Objective To investigate the homology of methicillin-resistant Stphylococcusaureus(MRSA)from the neonatal intensive care unit(NICU)of a children's hospital,and evaluate routes and preventive strategies of MRSA healthcare-associated infection(HAI). Methods MRSA strains from neonates and environment of NICU between October and December 2014 were collected,and strains were identified by VITEK-2 microbial analysis system and cefoxitin Kirby-Bauer method,homology of MRSA was analyzed by pulsed-field gel electrophoresis (PFGE ). Results A total of 6 MRSA strains were isolated from NICU between October and December 2014,3 of which (bed-58,70,and 100)were detected MRSA from specimens,MRSA were isolated from neonatal incubator and nurse (nasal swabs and hands)who cared for neonate at bed 58. 5 of 6 MRSA strains were homology,antimicrobial susceptibility testing result showed that No. 1-5 strains were resistant to clindamycin and amoxicillin/clavulanic acid,No. 6 strain was slightly different from No. 1-5 strains,No. 6 strain was susceptible to both clindamycin and amoxicillin/clavulanic acid. PFGE results showed that No. 1-5 strains were of the same type,No. 6 strain was a different type. Conclusion The main route of this MRSA transmission is contact transmission,especially through the hands of health care workers,identification and analysis of epidemic strains by PFGE technique is an effective measures to prevent HAI outbreak and perform epidemiological study.

Clinical features and gene mutations in a patient with multiple aeyl-CoA dehydrogenase deficiency with severe fatty liver / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To analyze the clinical features and gene mutations in an adolescent patient affected with late-onset multiple aeyl-CoA dehydrogenase deficiency (MADD) with severe fatty liver.</p><p><b>METHODS</b>Potential mutations of the ETFDH gene were detected with polymerase chain reaction (PCR) and DNA sequencing.</p><p><b>RESULTS</b>The 13-year-and-10-month girl has presented with weakness without any other special manifestation. Laboratory tests demonstrated an elevation of myocardial enzyme levels, total cholesterol, lactic acid and abnormal serum free fatty acids. H magnetic resonance spectroscopy revealed severe fatty liver. An increase in multiple plasma acyl-carnitines was detected by gas chromatography/mass spectrometry and isobutyrylglycine in urine by screening with tandem mass spectrometry. Genetic analysis demonstrated 2 heterozygous missense mutations c.250G>A (p.Ala84Thr) and c.353G>T (p.Cys118Phe) in the ETFDH gene. The diagnosis of MADD was confirmed. The patient was given large dose of vitamin B2, which resulted in rapid clinical and biochemical improvement.</p><p><b>CONCLUSION</b>A common mutation c.250G>A and a novel mutation c.353G>T in the ETFDH gene were identified in the patient. The pathogenic role of c.353G>T (p.Cys118Phe) deserves further study. Early diagnosis of MADD and appropriate therapy is crucial for the prognosis.</p>

Therapy for disseminated intravascular coagulation / 中华实用儿科临床杂志

Disseminated intravascular coagulation ( DIC ) is a exceedingly complicated pathophysiological process,both pro and anti coagulation factors are activated and consumed,it is difficult to determine acute stage of the syndrome for physicians based on clinical presentations and laboratory tests,management strategy of DIC is not same due to different etiology,the dose and regimen of drug administration are uncertain. At present pediatricians usually treat children patients with DIC according to researches and guidelines for adults. The key strategies to DIC management is to treat underlying diseases aggressively so as to clear risk factors triggering extensive coagulation and resuscitate with ap-propriate blood products. No clinical trials have proved that heparin can improve clinical outcome,efficacies of activated protein C,antithrombin, tissue factor pathway inhibitor and thrombomodulin are needed to be verified by more high qua-lity clinical trials in DIC treatment.

Clinical analysis of severe liver damage in children / 临床儿科杂志

ObjectiveTo explore the clinical characteristics, treatment and prognosis of severe liver damage in children.MethodsClinical data of 55 children with severe liver damage were retrospectively analyzed.Results In 55 children (31 boys and 24 girls) aged from 28 days to 12 years, forty-five children had acute liver injury mainly caused by infectious diseases (21 cases, 53.3%), blood tumor diseases (5 cases, 11.1%), hereditary metabolic diseases (4 cases, 8.9%), and unexplained diseases (10 cases, 22.2%), ten children had chronic liver injury with decompensated cir-rhosis. Most of severe liver damage in children was caused by antipyretic drugs, traditional Chinese medicine and cold medicine, including 31 cases of acute liver injury and 4 cases of chronic liver injury. In children with acute liver injury, clinical symptoms included gastrointestinal symptoms (32 cases, 71.1%), jaundice (26 cases, 57.8%), hemorrhage (9 cases, 20.0%), multiple organ dysfunction (13 cases, 28.9%) and hepatic encephalopathy (6 cases, 13.3%). In children with chronic liver damage, clinical symptoms included abdominal distension and ascites (10 cases), jaundice (9 cases), gastrointestinal bleeding (7 cases), hepatic encephalopathy (3 cases) and multiple organ dysfunction (1 case). In 55 chil-dren, 39 children were died and the total mortality was 70.91%. In 14 cases of multiple organs dysfunction syndromes, 13 cases (92.9%) were died. All three cases of hepatic encephalopathy were died.ConclusionsInfectious diseases are the leading cause of sever liver damage in children. The most common inciting factors are antipyretic drugs, traditional Chinese medicine and cold medicine. Children with severe liver damage have a high mortality. Rational use of medicine and the concept of the prevention first should been strengthened.

Liver and heart iron deposition status in patients with β thalassemia major: a multicenter study / 中华儿科杂志

<p><b>OBJECTIVE</b>To observe the status of iron deposition in patient with β thalassemia major, and to formulate appropriate treatment strategies.</p><p><b>METHOD</b>The data of status of transfusion and chelation in 135 patients aged from 6 years and 4 months to 17 years and 11 months with β thalassemia major were collected and analyzed. Serum ferritin levels were determined and cardiac and hepatic iron deposition was determined using MRI T2(*) technology.</p><p><b>RESULT</b>Of the 135 cases studied, 66 were male, and 69 were female, their average age was 12.1 years. Serum ferritin (SF) was determined for 111 cases, it varied from 1 086.8 µg/L to 15 011.5 µg/L. Among them, 16 cases had SF level <2 000 µg/L (14.5%) , in 41 cases SF were between 2 000 and 4 000 µg/L (36.0%) ;in 54 cases SF >4 000 µg/L (48.7%) . Liver MRI T2(*) results showed that in only 8 cases (5.9%) iron content in the liver was in normal range, 19 cases (14.9%) showed mild liver iron deposition;34 (25.2%) moderate and 74 (54.8%, the youngest one was only 6 years and 4 months of age) had severe iron deposition respectively. Cardiac MRI T2(*) showed that in 89 cases (65.9%) iron content in the heart was in normal range;19 cases (14.1%) had mild cardiac iron deposition and 27 (20.0%) presented severe iron deposition (the youngest one was only 9 years and 3 months of age) . SF level was obviously related to liver and cardiac iron deposition (MRI T2(*)) r and P value were -0.284, 0.003 and -0.374, 0.000 respectively. In 108 cases regular transfusion and chelation were delayed due to financial problem. The late and insufficient dosage administered and irregular chelation caused the higher SF level and the severe iron deposition.</p><p><b>CONCLUSION</b>The survival status of β thalassemia major in China is worrisome. Majority of them had not received regular transfusion and chelation. Liver and cardiac iron deposition occur early and had a high incidence.</p>

Therapeutic effects of exercise-based treatment programme on children with attention-deficit/hyperactivity disorder / 中华儿科杂志

<p><b>OBJECTIVE</b>To evaluate the effects of an exercise-based treatment programme (dyslexia, dyspraxia and attention-deficit treatment, DDAT) on various subtypes of attention-deficit/hyperactivity disorder (ADHD).</p><p><b>METHOD</b>Ninety-one ADHD children with standing balance dysfunction (ADHD-I 43, ADHD-HI 15 and ADHD-C 33) were given DDAT for 6 months, the efficacy of DDAT was evaluated before DDAT, three, six months after the treatment and three month after end of the treatment according to SNAP-IV, before and after the treatment by balancing function test and Conners Parents Rating Scale.</p><p><b>RESULT</b>Inattention subscale scores of ADHD-I, ADHD-HI and ADHD-C before and after the interventions were 1.99 ± 0.34, 0.96 ± 0.31, 2.17 ± 0.31and 1.19 ± 0.45, 0.81 ± 0.28, 1.32 ± 0.37, differences of ADHD-I and ADHD-C were significant (P < 0.05), hyperactivity subscale scores of three subtypes of ADHD were 0.81 ± 0.35, 2.01 ± 0.35, 1.96 ± 0.33 vs.0.45 ± 0.33, 0.79 ± 0.41, 1.10 ± 0.35, there were significant differences as well (P < 0.05). The score of hyperactivity symptom was reduced more compared to that of inattention symptom by the SNAP-IV scale parent forms. There were significant difference before and after the treatment based on Conners parent scale for conduct problem (1.11 ± 0.48 vs. 0.76 ± 0.44) , learning problem (1.97 ± 0.58 vs.1.60 ± 0.67), psychosomatic problems (0.61 ± 0.49 vs. 0.29 ± 0.35) , activity/ hyperactivity (1.46 ± 0.69 vs.1.09 ± 0.55) and anxiousness (1.05 ± 0.63 vs.0.62 ± 0.47) as well (P < 0.05); the standing balance dysfunction improved for most of the children, total effective rate was 87.9%, no significant difference was found among the three subtypes (P > 0.05).</p><p><b>CONCLUSION</b>DDAT is a safe and efficient intervention for the ADHD children with standing balance dysfunction, the improvement on hyperactivity symptom was better than that on inattention symptom. This study shows that an exercise-based treatment programme for cerebellum function improves symptoms of ADHD and balance function.</p>

Clinical analysis of children acute lymphoblastic leukemia / 国际儿科学杂志

Objective To analysis the clinical characteristics and the long-term effect of children with acute lymphoblastic leukemia (ALL).Methods From 2005 to 2010,80 newly diagnosed ALL children were enrolled and treated with protocol based on ALL-BFM2002.The five-years overall survival (OS)and event-free survival(EFS) were analyzed by the method of Kaplan-Meier.Results For the 80 patients,male to female ratio is 1.22∶1.The median age was 4.3 years.33 were in standard risk(41.2％),37 were in medium risk(46.3％),and 10 were in high risk(12.5％).22 had white blood cell count ≥20 x 109/L(27.5％).three patients with BCR-ABL translocation(3.8％),one patient with MLL gene rearrangement(1.3％),17 patients with TEL-AML translocation (21.3％).During induction therapy,79 patients (98.8 ％) achieved complete remission(CR).The five-years OS and EFS were (85.9 ± 4.0) ％ and (79.2 ± 4.7) ％ respectively.The five-years EFS:SR group (86.6 ± 6.4) ％,IR group (81.1 ± 6.4) ％,HR group (48.0 ± 16.4) ％.The difference among risk groups was statistically significant(x2 =7.03,P ＜0.05).12 patients relapsed(15.0％),the median time from diagnosis to relapse was 23.5 months.11 patients died (13.8 ％).Conclusion According to stratification by risk factors and risk-adapted therapy,the quality of ALL children's life had improved.

Changes of CD4 +CD25highFoxp3 +regulatory T cells and their significance in childhood B-cell acute lymphocytic leukemia / 中华微生物学和免疫学杂志

Objective To investigate the changes of CD4 +CD25highFoxp3 +regulatory T (Treg) cells and their significance in immune escape of childhood B-cell acute lymphocytic leukemia ( B-ALL ) . Methods Forty-two children with B-ALL and twenty-eight age-matched healthy children were enrolled in this study.Flow cytometry analysis was performed to evaluate the proportion of CD 4 +CD25high Foxp3 +Treg cells as well as CD4 +CD25high ICOS+Foxp3 +and CD4 +CD25high ICOS-Foxp3 +subsets in peripheral blood samples.The expression of associated molecules including IL-10, TGF-β, IL-35, TGF-βRII, ICOS and CD28 at protein level were also measured by flow cytometry analysis .The transcription level of Smad3/4, TIEG1 and Itch by CD4 +T cells were determined by quantitative real-time PCR.The concentration of TGF-βin plasma was detected by enzyme-linked immunosorbent assay.Results (1)The proportion of CD4 +CD25highFoxp3 +Treg cells in children with B-ALL were significantly higher than those of health subjects (P0.05).(3)The concentra-tion of TGF-βin plasma from children with B-ALL were higher than those from control group [ ( 25 .83 ± 12.65) ng/ml vs (8.59 ±5.73) ng/ml, P<0.05].The expression of TGF-βRII and its associated mole-cules (Smad3/4, TIEG1 and Itch) by CD4 +T cells were significantly up-regulated.Moreover, an increased expression of ICOS and CD28 by CD4 +CD25highFoxp3 +Treg cells were also observed in children with B-ALL (P<0.05).Conclusion The hyper-activity of TGF-β, ICOS and CD28 signaling might be closely associ-ated with the increased proportion of CD4 +CD25high Foxp3 +Treg cells and the imbalance of its subsets in children with B-ALL.

Clinical significance of 99mTc thyroid scintigraphy in children with congenital hypothyroidism / 中华内分泌代谢杂志

Forty-three children with congenital hypothyroidism(CH)underwent 99mTc thyroid scintigraphy, after being followed up by receiving levothyroxine till 2 to 3 years of age. The results showed that thyroid agenesia happened in 37 cases( 86.05% ) while entopic gland in 6 cases (13.95% ). Thyroid scintigraphy with 99mTc is an informative procedure in determining etiology and treatment schedules for children with CH.

Diagnosis and treatment progress of children's acute myeloid leukemia / 国际儿科学杂志

Acute mveloid leukemia(AML) is a heterogeneous group of leukemias that arise from clonal transformation of hematopoietic precursors.This review summarizes classification of pediatric AML,more precise risk-group stratification,ongoing phase Ⅲ studies and minimal residual diseasse monitoring.In addition,we discuss the opportunities for innovative chemotherapy drugs in refractory AML and relapsed AML,such as gemtuzumab ozogamicin,liposomal daunorubicin,clofarabine,cladribine and lmatinib.Finally,the roles of allogeneic hematopoietic stem cell transplantation in pediatric AML are also discussed.

Predictive effect of combined evaluation of XRCC1, XPD and GSTP1 single nucleotide polymorphisms in platinum based chemotherapy / 中国医师杂志

Objective To investigate the predictive value of combined analysis on single nucleotide polymorphisms (SNPs) of X-ray cross-complementing1 ( XRCC1 ) gene 194 and 399 codon,xeroderma pigmentosum group D (XPD) gene 312 codon and glutathione S-transferase P1 (GSTP1) gene 105 codon in platinum based chemotherapy.Methods Direct sequencing was performed to detect XRCC1,XPD and GSTP1 genotypes in peripheral blood from 50 cancer patients receiving platinum-based chemotherapy.Genetic polymorphisms of these genes related to sensitivity of platinum were reviewed.Results Favorable genotypes were Arg/Trp and Trp/Trp in XRCC1 194 codon,Arg/Arg in XRCC1 399 codon,Asn/Asn in XPD 312 codon and Val/Val in GSTP1 105 codon.The response rate to chemotherapy was 57.1％,75.0％,60.9％,85.7％ and 87.5％,respectively.The response rate for patients possessing ≥2 favorable genotypes and those possessing 1 or 0 favorable genotype was 78.9％,36.4％ and 0,respectively.Patients possessing ≥2 favorable genotypes demonstrated higher sensitivity to platinum based chemotherapy,compared with those possessing 1 or 0 favorable genotype ( x2 =25.79,P ＜ 0.01 ).Conclusions Combination analysis of genomic polymorphisms of XRCC1,XPD and GSTP1 may be useful in predicting sensitivity of platinum based chemotherapy.