中华实用儿科临床杂志最新文献

{"title":"Clinical features and survival analysis of neuroblastoma with cervical lymph node metastasis in children: su-mmary of 10 years in single center","authors":"Cheng Huang, Yan Su, Shengcai Wang, M. Jin, Xiufang Chai, Tian-xing Liu, Xiaoman Wang, Lejian He, H. Qin, Huanmin Wang, Xiao-li Ma","doi":"10.3760/CMA.J.CN101070-20190127-00063","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190127-00063","url":null,"abstract":"Objective \u0000To improve the awareness of neuroblastoma (NB) with cervical lymph node metastasis in children patients by summarizing the clinical features of such patients and analyzing their survival situation. \u0000 \u0000 \u0000Methods \u0000The research analyzed the medical records of 225 patients with cervical lymph node metastasis of NB from April 1, 2007 to May 31, 2017 in Beijing Children′s Hospital, Capital Medical University.The treatment were divided into 2 phases according to treatment time (one from April 1, 2007 to December 31, 2011 and the other from January 1, 2012 to May 31, 2017). The survival situation and treatment effect in each phase were analyzed.The following up time ended at Jan 1, 2018. \u0000 \u0000 \u0000Results \u0000(1)Clinical features: total 225 cases admitted in the research with about 37.3% of all the NB patients.One hundred girls(44.4%) and 125 boys(55.6%). The age from 2 months to 147 months, the me-dian age of patients was 37 months.The age under 18 months were 27 cases(12.1%). The majority primary site of tumor was located in retroperitoneal(174 cases, 77.3%) and mediastinum(48 cases, 21.3%). There were 33 cases which metastasic site confined to cervical lymph node(4N stage, 14.7%); 139 cases (62.1%) with bone marrow metastasis, 159 cases (71.0%) with bone metastasis.The number of patients in high-risk group was 208 cases(91.2%), while the low-risk and the medium-risk group was 17 cases(7.5%). (2)Treatment effect and survival analysis: a total of 200 cases received systemic treatment and followed-up in our hospital.The follow up time from 2 months to 123 months, the median follow up time was 23 months.The 3 year overall survival (OS) rate of all the patients was 56.4%, the 3 year event free survival (EFS) rate was 52.0%.While the 5-year OS was 46.5%, and the 5-year EFS was 39.7%.According to the first phase of treatment(April 1, 2007 to December 31, 2011), the 3-year EFS was 49.6%, and the 3-year OS was 54.4%, the 5-year EFS was 39.5%, and the 5-year OS was 44.7%.According to the second phase of treatment(January 1, 2012 to May 31, 2017), the 3-year EFS was 58.1%, and the 3-year OS was 57.3%.The 5-year EFS was 48.7%, and the 5-year OS was 46.8%.About the 4N patients, the 3-year EFS was 71.2%, and the 3-year OS was 82.2%.The 5-year EFS was 61.5%, and the 5-year OS was 76.7%. \u0000 \u0000 \u0000Conclusions \u0000Cervical lymph node is one of the most common distant metastasis of NB.More than half of the patient with bone or bone marrow metastasis.The prognosis of patients′ metastatic disease limited to cervical lymph nodes(4N stage), but still worse than the foreigner haver reported.The probable reason is the proportion of high-risk patients in the center is higher than the foreign research. \u0000 \u0000 \u0000Key words: \u0000Neuroblastoma; Cervical lymph node metastasis; Clinical features; Survival analysis","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"188-192"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"43807701","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Health-related quality of life among survivors of cancer in children","authors":"Yongjun Fang, Heng Zhang","doi":"10.3760/CMA.J.CN101070-20200227-00275","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20200227-00275","url":null,"abstract":"Over the past decades, substantial improvements in diagnostics, treatment, techniques and pharmacotherapy have resulted in a steadily increasing population of childhood cancer survivors.Health-related quality of life (HRQoL) of survivors has gradually become the focus of attention.Survivors of cancer in children will face a range of problems, such as physical, psychological and social pressure brought by the disease, growth and development disorders, gonadal dysfunction and late effects (organ dysfunction and secondary cancer). Previous studies have shown that the HRQoL of cancer survivors is significantly lower than that of the normal people, but some studies dedicated that the cancer experience may have a positive impact on the HRQoL of survivors.The quality of life questionnaires is widely used to evaluate HRQoL from the aspects of physical health, mental health and social health.Information from observational, cross-sectional, but particularly longitudinal studies, is needed to further describe HRQoL of cancer survivors in children, as well as provide a foundation for the development of intervention studies.More relevant studies are expected to provide evidence-based basis in making clinical decisions. \u0000 \u0000Key words: \u0000Cancer; Survivors; Health-related quality of life; Child","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"184-187"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41375148","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Application of computer aided design 3D printed navigation template in Salter pelvic osteotomy for dislocation of hip joint in children","authors":"Ming Yong, Y. Lou, K. Tang, Peng Xu, Pengfei Zheng, Fei Liu","doi":"10.3760/CMA.J.CN101070-20190327-00253","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190327-00253","url":null,"abstract":"Objective \u0000To explore the feasibility of accurate localization for the acetabular direction in Salter pelvic osteotomy for the developmental dislocation of the hip joint in children by using computer aided design and 3D printing technique. \u0000 \u0000 \u0000Methods \u0000The clinical data of 12 patients of unilateral hip dislocation treated with Salter pelvic osteotomy by using 3D printed navigation template in Department of Orthopedics, Children′s Hospital of Nanjing Medical University From October 2016 to April 2017 were retrospectively analyzed.Among the 12 cases, there were 4 males and 8 females, 5 hips on the left and 7 hips on the right, aged 1.5 to 5.0 years old (mean 2.3 years old). According to the CT data, the models of the healthy hip joint were mirrored to the contralateral side by Mimics software.Computer-aided simulations of Salter pelvic osteotomy on models of the affected hip joints were performed.Then, the models of the affec-ted hip joint were rotated to the mirror models of the contralateral hip joint.The navigation templates were designed according to the exposed pelvic surface morphology during the operation.The navigation templates were printed by rapid prototyping technology to guide the operation.Preoperative and postoperative acetabular index (AI) and center edge angle (CEA) of affected side were compared to postoperative AI and CEA of contralateral side respectively. \u0000 \u0000 \u0000Results \u0000A method of making personalized navigation templates for Salter pelvic osteotomy was established in 12 children with developmental dislocation of the hip joint.The operation time ranged from 40.2 to 64.5 min, averaging (50.6±8.5) min.The intraoperative bleeding volume ranged from 35 to 60 mL, averaging 52 mL.No vascular and nerve injury was found in the postoperative examination, and no child had complications such as infection, residual foreign body of the guide plate and so on.There was significant difference in preoperative measurements regarding AI between the affected side [(38.4±2.8)°] and the contralateral side [(21.6±0.8)°](t=-18.77, P 0.05), there was no significant difference in postoperative measurements regarding CEA between the affected side[(21.2±2.6)°] and the contralateral side(t=-0.435, P>0.05). \u0000 \u0000 \u0000Conclusions \u0000The navigation template prepared by computer aided design and 3D printing technology has good accuracy, and is a new approach to accurate acetabular rotation in children with the developmental dislocation of the hip joint for Salter pelvic osteotomy. \u0000 \u0000 \u0000Key words: \u0000Dislocation of hip joint; Osteotomy; 3D printing","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"210-214"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"44154463","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Study on the clinical characteristics and prognosis for children high-risk group of head and neck rhabdomyosarcoma","authors":"Yi Zhang, Weiling Zhang, D. Huang, Yizhuo Wang, Huimin Hu, Song Li, Xue Meng","doi":"10.3760/CMA.J.CN101070-20190201-00081","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190201-00081","url":null,"abstract":"Objective \u0000To study the relationship between high-risk factors with the diagnosis, treatment and prognosis of children with high-risk head and neck rhabdomyosarcoma (RMS). \u0000 \u0000 \u0000Methods \u0000Children with high-risk RMS primarily found in head and neck were selected as research objects according to the criteria of RMS risk degree formulated by Children Oncology Group (COG) and Children′s Rhabdomyosarcoma Cooperative Group, Children′s Hematological Oncology Group, China Anti-Cancer Association (CCCG-RMS), and combined treatment including puncture pathology/surgery, chemotherapy and radiotherapy were performed.The relationship with primary site, age, pathology type and metastasis and prognosis were analyzed. \u0000 \u0000 \u0000Results \u0000Twenty-nine patients were collected as research object, 17 cases (58.6%) of male, and 12 cases (41.4%) of female.The primary site: orbit was 7 patients (24.1%), and para-meningeal was 22 patients (75.9%). Clinical stage: 2 cases were stage Ⅲ (6.9%), and 27 cases were stage Ⅳ (93.1%). Followed up to December 31, 2018, 14 patients were dead, 15 patients were survival, and 10 patients were event free survival.The overall survival rate was 48.3%(15/29 cases), and the event free survival rate was 34.5%(10/29 cases). According to Kaplan-Merier survival curve analysis, the average overall survival time was (76.0±12.0) months, 95% confidence interval (95% CI): (53.5-93.2)months.The event free survival time was (62.5±10.6) months, 95% CI: (47.0-83.9)months. \u0000 \u0000 \u0000Conclusions \u0000The prognosis of high-risk head and neck RMS is poor.The center system metastasis is cause of death.Thus, the main task is how to close follow-up and prevention of central nervous system invasion for improving prognosis. \u0000 \u0000 \u0000Key words: \u0000Rhabdomyosarcoma; Head and neck; High-risk group; Prognosis; Child","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"193-196"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48192784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Effects of probiotics on apoptosis and proliferation of intestinal epithelial cells in rats with necrotizing enterocolitis","authors":"Mengmeng Yuan, Xiao-yun Zhang, Yaping Song, Mingchao Li","doi":"10.3760/CMA.J.CN101070-20190125-00059","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190125-00059","url":null,"abstract":"Objective \u0000To study the effect of compound Lactobacillus acidophilus on the apoptosis of intestinal epithelial cells and expression of proliferating cell nuclear antigen (PCNA) in rats with necrotizing enterocolitis, so as to provide some theoretical reference for the application of compound Lactobacillus acidophilus in the prevention and treatment of necrotizing enterocolitis(NEC) for newborn infants. \u0000 \u0000 \u0000Methods \u0000Thirty-six 48-hour old newborn SD rats were randomly divided into normal group, model group and intervention group, with 12 rats in each group.The NEC model was established by hypoxia and cold stimulation for 3 days, and the intervention group was given the compound Lactobacillus acidophilus at the same time; after the model was established, all the rats were sacrificed after 12 hours of fasting and their intestinal tissues were excised by terminal-deoxynucleoitidyl transferase mediated nick end labeling (TUNEL) method to determine the condition of apoptosis and to detect expression of the PCNA gene in the intestinal tissues by fluorescent quantitative PCR. \u0000 \u0000 \u0000Results \u0000(1) The apoptosis rates of the intestinal tissues: in the model group, the normal group and the intervention group were (88.33±2.77)%, (4.75±0.75)% and (44.41±4.81)%, respectively, and the differences had statistical significance(all P<0.05). (2) The PCNA expression in the intestinal tissue by real-time PCR in the model group, normal group and intervention group were (9.28±3.26)×10-4, (15.35±1.91)×10-4 and (12.09±3.06)×10-4, respectively, and the differences had statistical significance(all P<0.05). \u0000 \u0000 \u0000Conclusions \u0000The apoptosis rate of intestinal epithelial cells increases and PCNA expression decreases when NEC occurs.Compound Lactobacillus acidophilus can reduce apoptosis, promote intestinal epithelial cell proliferation, and thus help to reduce the risk and severity of NEC in neonatal rats. \u0000 \u0000 \u0000Key words: \u0000Neonatal necrotizing enterocolitis; Apoptosis; Proliferating cell nuclear antigen; Compound Lactobacillus acidophilus","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"227-230"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47382247","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Arsenic trioxide upregulates UL16 binding protein 1 expression in KG1a cells through ataxia telangiectasia mutated and RAD3-related kinase pathway","authors":"M. Ji, Jiaxing Dong, S. Cui, Xiaohui Si, Yahui Li, Xinqing Niu","doi":"10.3760/CMA.J.CN101070-20190301-00146","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190301-00146","url":null,"abstract":"Objective \u0000To observe the effect of arsenic trioxide (ATO) on the expression of NKG2D ligand UL16 binding protein 1(ULBP1) in acute myeloid leukemia KG1a cells, and explore the molecular mechanism for its regulation of ULBP1 expression. \u0000 \u0000 \u0000Methods \u0000KG1a cells were cultured in vitro.Then, the inhibition of KG1a cell proli-feration by different concentrations of ATO was detected by cell counting kit-8(CCK8) assay, and the expression of ULBP1 mRNA and surface protein in KG1a cells were examined by real-time RT-PCR and flow cytometry, respectively.After that, the blocking effects of ataxia telangiectasia mutated and RAD3-related kinase (ATM/ATR) inhibitor caffeine on ATO-upregulated expression of ULBP1 mRNA and surface protein expressions were investigated, and the effects of ATO on the expression of CHK1 and CHK2 proteins and their phosphorylation in KG1a cells were observed by Western blot method. \u0000 \u0000 \u0000Results \u0000Different concentrations (1, 2, 3, 4, 5 μmol/L) of ATO could inhibit the proliferation of KG1a cells, which was concentration dependent, and the half inhibitory (IC50) concentration to KG1a cells was 2.7 μmol/L.The expression of ULBP1 mRNA on KG1a cells were increased when incubated with ATO at concentration 1, 2, 3, 4, 5 μmol/L, compared without ATO group, ULBP1 mRNA expression level relatively increased respectively to (1.86±0.30) times, (3.02±0.71) times, (3.16±0.75) times, (4.80±0.70) times and (3.70±0.89) times, and the differences were statistically significant (all P<0.05). Furthermore, ATO (1, 2, 3, 4 and 5 μmol/L) upregulated ULBP1 protein expression on KG1a cells compared with that in the group without caffeine, and the differences were statistically significant (all P<0.05). After caffeine pretreat KG1a cell 2 h and ATO incubate KG1a cell 24 h, ULBP1 mRNA and protein expression levels were significantly reduced.When caffeine concentration was 8 mmol/L, ULBP1 mRNA expression level relatively reduces from (9.55±0.38) times to (6.36±0.93) times compared with that in the group without caffeine, and the difference was statistically significant (P<0.05). When caffeine concentration was 2, 4 and 8 mmol/L respectively, the expression of ULBP1 protein was reduced from that in the group without caffein treatment (3.50±0.08) times to (2.17±0.07) times, (2.02±0.06) times and (1.75±0.06) times, respectively, and the differences were statistically significant (all P<0.05). The expression of CHK1 and CHK2 proteins decreased with the increase of ATO concentration, while p-CHK1 and p-CHK2 are increased as ATO. \u0000 \u0000 \u0000Conclusions \u0000ATO upregulate the expression of ULBP1 mRNA and protein in KG1a cells, and the ATM/ATR-CHK1/CHK2 pathway may be involved in it. \u0000 \u0000 \u0000Key words: \u0000Arsenic trioxide; KG1a cells; Natural killer cell; UL16 binding protein 1; Ataxia telangiectasia mutated and RAD3-related kinase pathway","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"231-235"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47564808","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epidemiology and prognostic factors of infantile malignant solid tumor","authors":"X. Yuan","doi":"10.3760/CMA.J.CN101070-20200301-00298","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20200301-00298","url":null,"abstract":"As an important part of childhood tumors, infantile malignant solid tumor is relatively rare, which has some common characteristics of childhood cancer as well as its own particularity.Most infantile tumors are embryo-derived tumors, whose pathogenesis is related to developmental biology, genetics and environmental factors.The distribution of constituent ratio of infantile malignant tumors is different from that of older children, and the epidemiological data statistics can be influenced by the imperfect children tumor registration system, population migration, birth defects and perinatal factors, environmental exposure, gender, race and other factors.Heterogeneity is one of the prominent features of malignant solid tumor in infants. The experience gained from treatment of older children cannot be directly extrapolated to infants.The principle of treating infantile malignant solid tumors should take into account both the effectiveness and the long-term impact on infant growth.Early diagnosis and appropriate treatment mean that the prognosis of most infants with malignant solid tumor to be better than that of older children.Therefore, it is necessary to manage these tumors as a separate entity.The advance of epidemiology and prognostic factors of infantile malignant solid tumors at home and abroad was reviewed in this paper. \u0000 \u0000Key words: \u0000Infant; Malignant; Solid tumor; Incidence rate; Constituent ratio; Prognosis","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"178-183"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45700277","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Case report of successful treatment of severe aluminum phosphide poisoning through veno-arterial extracorporeal membrane oxygenation","authors":"Boliang Fang, Zheng Li, S. Qian, Heng-miao Gao, J. Zeng","doi":"10.3760/CMA.J.CN101070-20190108-00006","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190108-00006","url":null,"abstract":"首都医科大学附属北京儿童医院重症医学科收治磷化铝中毒并急性心力衰竭患儿1例，女，12岁，吸入磷化铝48 h出现进行性加重的心力衰竭及心源性休克，经静－动脉体外膜肺氧合支持治疗后痊愈，且未遗留后遗症。重症磷化铝中毒者常规治疗下病死率高，静－动脉体外膜肺氧合成功用于治疗此类患者在国内尚属首次报道。","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"239-240"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"42014128","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Relationship between joints bleeding and joint assessment in children with severe haemophilia A","authors":"W. Yao, Qing Zhang, Min Zhou, L. Tang, Xiaobo Luo, Ningning Zhang, Sheng Yang, Yan Wang, P. Ding, S. Cai, Zhenping Chen, Xiaojing Li, R. Wu","doi":"10.3760/CMA.J.CN101070-20190213-00097","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190213-00097","url":null,"abstract":"Objective \u0000To understand the relationship between joint bleeding and joint disease in hemophilia children, and to provide a theoretical basis for clinical treatment and prognosis. \u0000 \u0000 \u0000Methods \u0000The patients with severe hemophilia A between 1 and 7 years old and with relevant nodal bleeding records were selected.All the patients admitted in Beijing Children′s Hospital, Capital Medical University, and Chengdu New Century Women′s and Children′s Hospital since June 2016 to January 2017.All the joint bleeding of each child was taken as the study joint, and the joint bleeding was collected during the last 3 months.The joints were assessed by using ultrasound, X-ray, magnetic resonance imaging(MRI) and Hemophilia Joint Health Score (HJHS) scoring systems.The correlation analysis was conducted between the joint bleeding, ultrasound, X-ray, MRI and HJHS scores.The correlation analysis was conducted for baseline ultrasound, X-ray, MRI and HJHS scores. \u0000 \u0000 \u0000Results \u0000(1) There were 18 patients enrolled.The mean age was (5.6±1.8) years old.There were 30 joints bleeding in the observation period in total, with the annul median joint bleeding times of 4 (4-16 times), and the annul median target joint bleeding times of 8 (4-16 times). (2) Joint bleeding times of was correlated with ultrasound and X-ray evaluation (r=0.390, P=0.033; r=0.517, P=0.008), and not correlated with HJHS or MRI(all P>0.05). (3) There was significantly positive correlation among ultrasound, X-ray, HJHS and MRI [r=0.815(ultrasound vs.X-ray), r=0.510(ultrasound vs.HJHS), r=0.812(ultrasound vs.MRI), r=0.666(X-ray vs.HJHS), r=0.911(X-ray vs.MRI), r=0.781(HJHS vs.MRI), all P 0.05). \u0000 \u0000 \u0000Conclusions \u0000The results of joint bleeding and joint evaluation are inconsistent.Joint bleeding can not truly reflect the situation of joint diseases.The assessment of hemophilia should include comprehensive evaluation of joint structure, function, activity ability and other aspects to guide the treatment of haemophi-lia children. \u0000 \u0000 \u0000Key words: \u0000Haemophilia A; Child; Bleeding joints; Joint assessment","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"197-200"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46063443","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Molecular response and prognosis of pediatric patients with Ph-positive acute lymphoblastic leukemia treated by tyrosine kinase inhibitors with chemotherapy","authors":"Yu-Juan Xue, A. Lu, Jun Wu, Y. Zuo, Y. Jia, Le-ping Zhang","doi":"10.3760/CMA.J.CN101070-20190222-00125","DOIUrl":"https://doi.org/10.3760/CMA.J.CN101070-20190222-00125","url":null,"abstract":"Objective \u0000To explore the molecular response and prognostic factors of pediatric patients with Ph-positive acute lymphoblastic leukemia (Ph+ ALL) treated by tyrosine kinase inhibitors (TKI) with chemotherapy in TKI era. \u0000 \u0000 \u0000Methods \u0000The clinical data of children newly diagnosed with Ph+ ALL admitted at Department of Pediatrics, Peking University People′s Hospital from August 2006 to February 2017 were retrospectively reviewed.The molecular biological characteristics and survival prognosis of the 30 patients who received continuous TKI with chemotherapy from early induction combined and no subsequent transplantation were analyzed. \u0000 \u0000 \u0000Results \u0000The 30 patients with Ph+ ALL had 19 males and 11 females with a median age of 8-year-old (ranging from 2 to 16 years). The complete remission (CR) rate after the first cycle of induction chemotherapy was 96.7% (29/30 cases), with overall CR rate of 100.0%; Before treatment, the mean level of BCR/ABL mRNA in the 30 patients was 73.2% (0.12%-160.60%) and the level declined significantly with the progression of chemotherapy courses, reaching the plateau stage at the 6th month of chemotherapy (Z=-1.922, P>0.05); nine patients had recurrence, with a median recurrence time of 7 months (3.7-58.8 months). Univariate analysis showed that age (P<0.05), the lever of minimal residual disease (MRD) after induction chemotherapy (P<0.01) and the MRD level at the 3th month of induction chemotherapy (P<0.01) affected the recurrence rate.The median follow-up time of 30 patients was 42.6 months (6.4-96.5 months), and the 3-year overall survival (OS) rate and event-free survival (EFS) rate were (78.6±7.8)% and (72.4±8.4)%, respectively; Cox multivariate analysis showed that the initial white blood cell count ≥34.0×109/L (OR=11.955, 95% CI: 1.075-132.899, P<0.05) and BCR/ABL mRNA reduction less than 3 log from baseline [major molecular response (MMR)] at the 3th month of induction chemotherapy (OR=8.563, 95% CI: 1.254-58.478, P<0.05) were independent risk factors affecting the 3-year EFS rate.In addition, the initial white blood cell count ≥34.0×109/L (OR=14.327, 95% CI: 1.843-243.592, P<0.05) was also an independent risk factor affecting the 3-year OS rate. \u0000 \u0000 \u0000Conclusions \u0000The application of TKI can significantly deepen the molecular response of Ph+ ALL in children.In the TKI era, the initial white blood cell count ≥ 34.0×109/L and BCR/ABL mRNA reduction less than 3 log from baseline (MMR) at the 3th month of induction chemotherapy are independent risk factors for the long-term survival of pediatric Ph+ ALL. \u0000 \u0000 \u0000Key words: \u0000Philadelphia chromosome; Acute lymphoblastic leukemia; Child; Tyrosine kinase inhibitor; Mole-cular response","PeriodicalId":9843,"journal":{"name":"中华实用儿科临床杂志","volume":"35 1","pages":"201-205"},"PeriodicalIF":0.0,"publicationDate":"2020-03-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"45659970","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}