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2.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 28(5): 1451-1458, 2020 Oct.
Artigo em Chinês | MEDLINE | ID: mdl-33067936

RESUMO

OBJECTIVE: To investigate the proliferation inhibition and pro-apoptotic effect of Huaier aqueous extract combined with routine chemotherapeutic drugs including Vincristine (VCR), Daunorubicin (DNR), L-aspartase (L-Asp) on human acute lymphoblastic leukemia cell lines Nalm-6 and Sup-B15. METHODS: Nalm-6 and Sup-B15 cell lines were treated with different concentrations of Huaier aqueous extract and chemotherapeutics including VCR, DNR, L-Asp alone or in combination for 48 h, and the growth inhibitory effect and IC50 values (the half maximal inhibitory concentration) were detected by CCK-8. Jin's formula was used to estimated the synergistic effect of these combinations. Apoptosis rates of Nalm-6 and Sup-B15 cells and expression of apoptosis-related proteins BAX, BCL-2, cleaved Caspase-3 were determined by flow cytometry and Western blot respectivcly. RESULTS: Huaier aqueous extract, VCR, DNR and L-Asp had inhibition effect on Nalm-6 and Sup-B15 cell lines. The inhibition rate of Huaier aqueous extract combined with VCR, DNR and L-Asp were all higher than those of each dug alone (P<0.05) and the combination index (q) was between 0.85 and 1.15 or greater than 1.15. The two kinds of drugs showed had additive or synergistic effects. The results of flow cytometry showed that the cell apoptosis rates in combined treatment group were higher than those of each drug alone (P<0.05). The results of Western blot revealed that Huaier aqueous extract and VCR all decreased protein expression of BCL-2 (P<0.05) and increase protein expression of BAX (P<0.05) and cleaved Caspase-3 (P<0.05) in Nalm-6 and Sup-B15 cells. Compared with Huaier aqueous extract or VCR alone, the effect of two drug combination were more significant. DNR down-regulated protein expression of BCL-2 (P<0.05) and up-regulated cleaved Caspase-3 (P<0.05). However, it had no effect on the expression of BAX in Nalm-6 and Sup-B15 cells. When it was combined with Huaier aqueous extract, the expression of cleaved Caspase-3 and BCL-2 showed more significant changes. The expression of BAX in combined treated group did not show significant difference, compared with group treated with Huaier aqueous extract in Nalm-6 and Sup-B15 cells. L-Asp did not show significant effect on the three apoptosis-related proteins and there was no significant difference between the combination group and the Huaier aqueous extract group. CONCLUSION: the combination of Huaier aqueous extract and VCR, DNR, L-Asp shows additive or synergistic effects on human acute lymphoblastic leukemia cell lines Nalm-6 and Sup-B15.


Assuntos
Antineoplásicos , Leucemia-Linfoma Linfoblástico de Células Precursoras , Antineoplásicos/uso terapêutico , Linhagem Celular Tumoral , Misturas Complexas/uso terapêutico , Humanos , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Trametes
3.
Curr Med Sci ; 40(2): 354-362, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32337697

RESUMO

Imatinib mesylate (IM) is the first-line treatment for Philadelphia (Ph) chromosomal positive leukemia by inhibiting phosphorylation of substrates via binding to the ABL kinase domain. Because of the drug resistance, side effects and the high cost of IM, it is necessary to find anti-cancer drugs with relatively low toxicity and cost, and enhanced efficacy, such as traditional Chinese medicines (TCMs). As one of TCMs, Huai Qi Huang (HQH) was chosen to treat BV173 and K562 cells. Various concentrations of HQH were added to cells for 24-72 h. Co-treatment of HQH and trametinib, an MEK inhibitor, was used to verify the synergistic effects on cell viability and apoptosis. Knockdown and overexpression of mitogen-activated protein kinase kinase 4 (MEK4) were implemented to demonstrate the role of MEK in cell apoptosis. Cell viability and apoptosis were measured by cell counting kit-8 assay (CCK8) and flow cytometry, respectively. Western blotting and real-time quantitative PCR (RT-qPCR) were used to assess protein and mRNA expression levels, respectively. The results showed that HQH inhibited survival and promoted apoptosis of BV173 and K562 cells in a dose-dependent manner, accompanied with down-regulation of PRKCH mRNA as well as CRAF, MEK4, phospho-ERK (pERK) and BCL2 proteins, and up-regulation of cleaved caspase3 protein. Co-treatment of HQH and trametinib had a synergistic effect on inhibiting survival and promoting apoptosis. MEK4 knockdown increased apoptosis, and had a synergistic effect with HQH. In contrast, MEK4 overexpression decreased apoptosis, and had the opposite effect with HQH. Collectively, the results of this study may identify a therapeutic mechanism of HQH on promoting apoptosis, and provide a potential option for treatment of Ph+ leukemia.


Assuntos
Regulação para Baixo , Medicamentos de Ervas Chinesas/farmacologia , Leucemia Mielogênica Crônica BCR-ABL Positiva/metabolismo , Proteína Quinase C/metabolismo , Piridonas/farmacologia , Pirimidinonas/farmacologia , Linhagem Celular Tumoral , Proliferação de Células/efeitos dos fármacos , Sobrevivência Celular/efeitos dos fármacos , Relação Dose-Resposta a Droga , Sinergismo Farmacológico , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Humanos , Células K562 , Leucemia Mielogênica Crônica BCR-ABL Positiva/tratamento farmacológico , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Sistema de Sinalização das MAP Quinases/efeitos dos fármacos , Proteína Quinase C/genética
4.
World J Pediatr ; 16(3): 232-239, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32333248

RESUMO

In the early February, 2020, we called up an experts' committee with more than 30 Chinese experts from 11 national medical academic organizations to formulate the first edition of consensus statement on diagnosis, treatment and prevention of coronavirus disease 2019 (COVID-19) in children, which has been published in this journal. With accumulated experiences in the diagnosis and treatment of COVID-19 in children, we have updated the consensus statement and released the second edition recently. The current version in English is a condensed version of the second edition of consensus statement on diagnosis, treatment and prevention of COVID-19 in children. In the current version, diagnosis and treatement criteria have been optimized, and early identification of severe and critical cases is highlighted. The early warning indicators for severe pediatric cases have been summarized which is utmost important for clinical practice. This version of experts consensus will be valuable for better prevention, diagnosis and treatment of COVID-19 in children worldwide.


Assuntos
Infecções por Coronavirus , Coronavirus , Pandemias , Pneumonia Viral/epidemiologia , Betacoronavirus , COVID-19 , Criança , Consenso , Humanos , SARS-CoV-2
5.
Curr Med Sci ; 40(2): 275-280, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32207032

RESUMO

Since December 2019, COVID-19 has occurred unexpectedly and emerged as a health problem worldwide. Despite the rapidly increasing number of cases in subsequent weeks, the clinical characteristics of pediatric cases are rarely described. A cross-sectional multicenter study was carried out in 10 hospitals across Hubei province. A total of 25 confirmed pediatric cases of COVID-19 were collected. The demographic data, epidemiological history, underlying diseases, clinical manifestations, laboratory and radiological data, treatments, and outcomes were analyzed. Of 25 hospitalized patients with COVID-19, the boy to girl ratio was 1.27:1. The median age was 3 years. COVID-19 cases in children aged <3 years, 3.6 years, and ≥6-years patients were 10 (40%), 6 (24%), and 9 (36%), respectively. The most common symptoms at onset of illness were fever (13 [52%]), and dry cough (11 [44%]). Chest CT images showed essential normal in 8 cases (33.3%), unilateral involvement of lungs in 5 cases (20.8%), and bilateral involvement in 11 cases (45.8%). Clinical diagnoses included upper respiratory tract infection (n=8), mild pneumonia (n=15), and critical cases (n=2). Two critical cases (8%) were given invasive mechanical ventilation, corticosteroids, and immunoglobulin. The symptoms in 24 (96%) of 25 patients were alleviated and one patient had been discharged. It was concluded that children were susceptible to COVID-19 like adults, while the clinical presentations and outcomes were more favorable in children. However, children less than 3 years old accounted for majority cases and critical cases lied in this age group, which demanded extra attentions during home caring and hospitalization treatment.


Assuntos
Betacoronavirus , Infecções por Coronavirus , Pandemias , Pneumonia Viral , Adolescente , COVID-19 , Criança , Pré-Escolar , China , Infecções por Coronavirus/diagnóstico por imagem , Feminino , Humanos , Lactente , Masculino , Pneumonia Viral/diagnóstico por imagem , SARS-CoV-2 , Tomografia Computadorizada por Raios X
6.
Zhongguo Dang Dai Er Ke Za Zhi ; 22(2): 171-176, 2020 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-32051086

RESUMO

OBJECTIVE: To study the role and mechanism of action of Huai Qi Huang (HQH) in the rat model of asthma. METHODS: Forty Sprague-Dawley rats were randomly divided into a control group, an asthma model group, a budesonide group, and an HQH group, with 10 rats in each group. A rat model of asthma was established by ovalbumin sensitization and challenge. The budesonide group was given budesonide aerosol 2 mg before each challenge. The HQH group was given HQH 4 g/kg dissolved in water by gavage before each challenge. Hematoxylin and eosin staining was used to observe the pathological changes of lung tissues. The percentage of eosinophils in bronchoalveolar lavage fluid (BALF) was measured. Enzyme-linked immunosorbent assay was used to determine the levels of interleukin-3 (IL-3), interleukin-4 (IL-4), interleukin-5 (IL-5), interleukin-10 (IL-10), interferon gamma (INF-γ), and immunoglobulin E (IgE) in BALF. Flow cytometry was used to determine T-helper type 1 (Th1)/T-helper type 2 (Th2) ratio in peripheral blood and the spleen. RT-PCR and Western blot were used to measure the mRNA and protein expression of T-bet and GATA-3 in lung tissue. RESULTS: Compared with the control group, the asthma model group showed significant increases in the degree of airway inflammation, the percentage of eosinophils in BALF, and the levels of IL-3, IL-4, IL-5 and IgE in BALF (P<0.05), however, the asthma model group showed significant reductions in the levels of IL-10 and INF-γ in BALF (P<0.05). The asthma model group had significantly lower percentage of Th1 cells but significantly higher percentage of Th2 cells in peripheral blood and the spleen compared with the control group (P<0.05). The mRNA and protein expression of T-bet in lung tissue was significantly lower, but the mRNA and protein expression of GATA-3 in lung tissue was significantly higher in the asthma group than those in the control group (P<0.05). Both HQH and budesonide significantly improved airway inflammation and the above markers in asthmatic rats (P<0.05), with comparable effects between them. However, there were still significant differences in these indices between the control group and the HQH or budesonide group (P<0.05). CONCLUSIONS: HQH can reduce the airway inflammation of asthmatic rats and alleviate the symptoms of asthma, possibly by regulating the levels of related cytokines and Th1/Th2 ratio through the T-bet/GATA-3 pathway.


Assuntos
Asma , Animais , Líquido da Lavagem Broncoalveolar , Medicamentos de Ervas Chinesas , Pulmão , Camundongos Endogâmicos BALB C , Ratos , Ratos Sprague-Dawley , Células Th2
7.
Zhongguo Dang Dai Er Ke Za Zhi ; 21(8): 772-776, 2019 Aug.
Artigo em Chinês | MEDLINE | ID: mdl-31416501

RESUMO

OBJECTIVE: To study the clinical features, treatment, and prognosis of pure red cell aplasia (PRCA) in children. METHODS: A retrospective analysis was performed for the clinical data of 16 children with PRCA. The outcome and prognosis of patients treated with prednisone combined with Huaiqihuang granules versus prednisone alone were evaluated. RESULTS: All the 16 children complained of symptoms of anemia including pale or sallow complexion. Of 12 children undergoing pathogen test, 7 (58%) were found to have pathogen infection, among which human cytomegalovirus was the most common. Lymphocyte subsets were measured for 7 children, among whom 5 (71%) had lymphocyte immune disorder. Six children were found to have abnormalities in immunoglobulin and complement. The 8 children treated with prednisone combined with Huaiqihuang granules had a median follow-up time of 21.5 months, among whom 1 was almost cured, 1 was relieved, and 6 were obviously improved; the median onset time of treatment was 1 month, and 2 children had disease recurrence in the course of drug reduction or withdrawal. The 8 children in the prednisone alone treatment group had a median follow-up time of 34 months, among whom 4 were almost cured, and 4 were obviously improved; the median onset time of treatment was 2.5 months, and 4 children had recurrence during drug reduction or withdrawal. CONCLUSIONS: Children with PRCA usually complain of anemia-related symptoms. Laboratory tests show pathogen infection in some children with PRCA, and most of children have immune disorders. Glucocorticoids have a good therapeutic effect, but some children relapse in the course of drug reduction or withdrawal. Combined treatment with prednisone and Huaiqihuang granules may have a faster onset of action and less possibility of recurrence.


Assuntos
Aplasia Pura de Série Vermelha , Criança , Glucocorticoides , Humanos , Prednisona , Recidiva , Estudos Retrospectivos
8.
Am J Hematol ; 93(7): 913-920, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29675840

RESUMO

Acute lymphoblastic leukemia (ALL) is the most common malignancy among children. The trial Chinese Children Leukemia Group (CCLG)-ALL 2008 was a prospective clinical trial designed to improve treatment outcome of childhood ALL through the first nation-wide collaborative study in China. Totally 2231 patients were recruited from ten tertiary hospitals in eight cities. The patients were stratified according to clinical-biological characteristics and early treatment response. Standard risk (SR) and intermediate risk (IR) groups were treated with a modified BFM based protocol, and there was 25%-50% dose reduction during intensification phases in the SR group. Patients in high risk (HR) group received a more intensive maintenance treatment. Minimal residual disease (MRD) monitoring with treatment adjustment was performed in two hospitals (the MRD group). Complete remission (CR) was achieved in 2100 patients (94.1%). At five years, the estimate for overall survival (OS) and event-free survival (EFS) of the whole group was 85.3% and 79.9%, respectively. The cumulative incidence of relapse (CIR) was 15.3% at five years. The OS, EFS and CIR for the SR group were 91.5%, 87.9%, and 9.7%, respectively. The outcome of the MRD group is better than the non-MRD group (5y-EFS: 82.4% vs 78.3%, P = .038; 5y-CIR: 10.7% vs 18.0%, P < .001). Our results demonstrated that the large-scale multicenter trial for pediatric ALL was feasible in China. Dose reduction in the SR group could achieve high EFS. MRD-based risk stratification might improve the treatment outcome for childhood ALL.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Adolescente , Criança , Pré-Escolar , China , Feminino , Humanos , Masculino , Neoplasia Residual , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Estudos Prospectivos , Recidiva , Indução de Remissão , Medição de Risco , Análise de Sobrevida , Centros de Atenção Terciária , Resultado do Tratamento
9.
J Huazhong Univ Sci Technolog Med Sci ; 37(5): 807-810, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29058300

RESUMO

We report one case of pediatric acute myeloid leukemia type 2 (AML-M2) who presented with karyotypic aberration of trisomy 21 with the t(5;11) chromosomal translocation. The patient achieved complete remission after two cycles of chemotherapy of daunorubicin, cytarabine and etoposide. Then, follow-up cytogenetic analysis from bone marrow cell cultures demonstrated a normal karyotype of 46, XY. After 9 years, the patient relapsed and the karyotypic abnormalities of trisomy 21 with t(5;11) reappeared. It was concluded that trisomy 21 with t(5; 11) is a new unfavorable cytogenetic aberration in AML-M2.


Assuntos
Cromossomos Humanos Par 11/genética , Cromossomos Humanos Par 5/genética , Síndrome de Down/genética , Leucemia Mieloide Aguda/tratamento farmacológico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Aberrações Cromossômicas , Seguimentos , Humanos , Leucemia Mieloide Aguda/genética , Masculino , Indução de Remissão , Translocação Genética
10.
J Huazhong Univ Sci Technolog Med Sci ; 36(6): 895-903, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27924501

RESUMO

Many eating behaviors form in childhood, and some unhealthy behaviors may persist into adulthood and have potential impacts on people's health. This study evaluated the effectiveness of behavioral intervention in reducing consumption of Western fast food, sweetened beverages, fried food in preschool children, and changing parents' rewarding behaviors that encourage the consumption of the unhealthy foods. The research was a cluster randomized trial of seven kindergartens, involving 1138 children aged 3-6 years and their parents in Beijing, China. Parents and children allocated to the intervention group received two lectures and printed resources, including behavior cards, educational sheets. Children's behavior cards, applied with behavior-changing techniques, were used to intervene, and monitor behavior changes over time. Children in the control group just followed their usual health education curriculum in kindergartens. Intervention effects on food consumption behaviors were assessed by examining pre- and post-questionnaires. Of the 1138 children screened at baseline, 880 (77.3%) were measured at the end of the intervention period. The intervention lasted from March to June in 2010. The results showed that consumption of Western fast food, sweetened beverages, and fried food was decreased among the intervention group (P<0.001). Proportions of parents using Western fast food as rewards for their children were decreased (P=0.002). From March to June 2010, the frequency of each target behavior in children tended to decrease over the intervention period (P<0.001). Most parents favored regularly-delivered behavior cards or materials for behavioral intervention. In conclusion, the behavioral intervention encourages the healthier eating behaviors of children and reduces the parents' practice of using unhealthy foods as reward.


Assuntos
Controle Comportamental/métodos , Terapia Comportamental/métodos , Fast Foods/efeitos adversos , Comportamento Alimentar/psicologia , Adulto , Criança , Pré-Escolar , Dieta Saudável , Dieta Ocidental/efeitos adversos , Feminino , Humanos , Masculino , Pais/psicologia , Recompensa
11.
J Huazhong Univ Sci Technolog Med Sci ; 35(5): 689-693, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26489623

RESUMO

The efficiency of dendritic cell-activated and cytokine-induced killer cell (DC-CIK) therapy on children with acute myeloid leukemia (AML) after chemotherapy was investigated. Mononuclear cells were collected from children achieving complete remission after chemotherapy, cultured in vitro and transfused back into the same patient. Interleukin-2 (IL-2) was injected subcutaneously every other day 10 times at the dose of 1 × 10(6) units. Peripheral blood lymphocyte subsets and minimal residual disease (MRD) were detected by flow cytometry. Function of bone marrow was monitored by methods of morphology, immunology, cytogenetics and molecular biology. The side effects were also observed during the treatment. The average follow-up period for all the 22 patients was 71 months and relapse occurred in two AML patients (9.1%). The percentage of CD3(+)/CD8(+) cells in peripheral blood of 15 patients at the 3rd month after DC-CIK treatment (36.73% ± 12.51%) was dramatically higher than that before treatment (29.20% ± 8.34%, P < 0.05). The MRD rate was >0.1% in 5 patients before the treatment, and became lower than 0.1% 3 months after the treatment. During the transfusion of DC-CIK, side effects including fever, chills and hives appeared in 7 out of 22 (31.82%) cases but disappeared quickly after symptomatic treatments. There were no changes in electrocardiography and liver-renal functions after the treatment. MRD in children with AML can be eliminated by DC-CIK therapy which is safe and has fewer side effects.


Assuntos
Células Matadoras Induzidas por Citocinas/transplante , Células Dendríticas/transplante , Imunoterapia Adotiva/métodos , Leucemia Mieloide Aguda/terapia , Adolescente , Antineoplásicos/uso terapêutico , Medula Óssea/efeitos dos fármacos , Medula Óssea/imunologia , Medula Óssea/patologia , Criança , Pré-Escolar , Células Matadoras Induzidas por Citocinas/citologia , Células Matadoras Induzidas por Citocinas/imunologia , Células Dendríticas/citologia , Células Dendríticas/imunologia , Feminino , Humanos , Injeções Subcutâneas , Interleucina-2/uso terapêutico , Leucemia Mieloide Aguda/imunologia , Leucemia Mieloide Aguda/patologia , Masculino , Neoplasia Residual , Cultura Primária de Células , Recidiva , Indução de Remissão , Resultado do Tratamento
12.
J Huazhong Univ Sci Technolog Med Sci ; 35(4): 490-494, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26223915

RESUMO

Abnormal cholesterol metabolism is associated with an elevated risk of developing atherosclerosis, hypertension, and diabetes etc. Na(+)/K(+)-ATPase was found to regulate cholesterol synthesis, distribution and trafficking. This study aimed to examine the effect of high-fat diet on cholesterol metabolism in rats and the role of Na(+)/K(+)-ATPase/Src/ERK signaling pathway in the process. Forty male SD rats were evenly divided into high-fat diet group and control group at random. Animals in the former group were fed on high-fat diet for 12 weeks, and those fed on basic diet served as control. Blood lipids, including total cholesterol (TC), triglyceride (TG), high density lipoprotein-cholesterol (HDL-C), and low density lipoprotein-cholesteral (LDL-C) levels, were detected at 3, 6 and 12 weeks. The ratio of cholesterol content in cytoplasm to that in cell membrane was detected in liver tissues. RT-PCR and Western blotting were used to measure the expression of lipid metabolism-associated genes (HMG-CoA reductase and SREBP-2) after 12-week high-fat diet. Na(+)/K(+)-ATPase/Src/ERK signaling pathway-related components (Na(+)/K(+)-ATPase α1, Src-PY418 and pERK1/2) were also measured by Western blotting. The results showed that the serum TC, TG, and LDL-C levels were significantly higher in high-fat diet group than those in control group, while the HDL-C level was significantly lower in high-fat diet group at 6 weeks (P<0.01). High-fat diet led to an increase in the cholesterol content in the cytoplasm and cell membrane. The ratio of cholesterol content in cytoplasm to that in cell membrane was elevated over time. The expression of HMG-CoA reductase and SREBP-2 was significantly suppressed at mRNA and protein levels after 12-week high-fat diet (P<0.05). Moreover, high-fat diet promoted the expression of Na(+)/K(+)-ATPase α1 but suppressed the phosphorylation of Src-PY418 and ERK1/2 at 12 weeks (P<0.05). It was concluded that high-fat diet regulates cholesterol metabolism, and Na(+)/K(+)-ATPase signaling pathway is involved in the process possibly by regulating the expression of lipid metabolism-associated proteins HMG-CoA reductase and SREBP-2.


Assuntos
Colesterol/sangue , Dieta Hiperlipídica/efeitos adversos , Metabolismo dos Lipídeos/efeitos dos fármacos , Sistema de Sinalização das MAP Quinases/efeitos dos fármacos , ATPase Trocadora de Sódio-Potássio/metabolismo , Acil Coenzima A/genética , Acil Coenzima A/metabolismo , Animais , Membrana Celular/metabolismo , Citoplasma/metabolismo , Regulação da Expressão Gênica/efeitos dos fármacos , Fígado/metabolismo , Masculino , Ratos , Ratos Sprague-Dawley , ATPase Trocadora de Sódio-Potássio/genética , Proteína de Ligação a Elemento Regulador de Esterol 2/genética , Proteína de Ligação a Elemento Regulador de Esterol 2/metabolismo
13.
Zhongguo Dang Dai Er Ke Za Zhi ; 16(5): 489-93, 2014 May.
Artigo em Chinês | MEDLINE | ID: mdl-24856998

RESUMO

OBJECTIVE: To study the clinical characteristics of ecotopic viral integration site-1 (EVI1) and BCR/ABL positive childhood leukemia. METHODS: Clinical data of four children with EVI1 and BCR/ABL positive leukemia and eight children with BCR/ABL positive but EVI1 negative chronic myeloid leukemia (CML) were retrospectively analyzed. RESULTS: In the four children with EVI1 and BCR/ABL positive leukemia, two were initially diagnosed with chronic phase of CML, one with accelerated phase of CML and one with high-risk acute lymphoblastic leukemia (ALL). There were no significant differences in clinical characteristics at diagnosis between the patients with EVI1 and BCR/ABL positive leukemia and BCR/ABL positive but EVI1 negative leukemia. CD33 and CD38 were highly expressed and t(9;22) abnormality was present in all patients with EVI1 and BCR/ABL positive leukemia. Two of the 3 children with EVI1 and BCR/ABL positive CML achieved complete remission one or three months after treatment. Acquired negative status conversion occurred for EVI1 but not BCR/ABL in one CML case. The 3 children with EVI1 and BCR/ABL positive CML survived 20, 13 and 14 months, respectively, without recurrence. The child with EVI1 and BCR/ABL positive ALL failed to achieve complete remission after the first course of treatment and discontinued further treatment. CONCLUSIONS: Co-expression of EVI1 and BCR/ABL fusion gene can be found in childhood CML and ALL. The relatively rare leukemia has not significant difference respect to clinical characteristics. Prognosis of the disease needs to be determined by clinical studies with a larger sample size.


Assuntos
Proteínas de Ligação a DNA/genética , Genes abl , Leucemia Mielogênica Crônica BCR-ABL Positiva/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Proto-Oncogenes/genética , Fatores de Transcrição/genética , Criança , Feminino , Humanos , Proteína do Locus do Complexo MDS1 e EVI1 , Masculino , Prognóstico , Estudos Retrospectivos
14.
Zhongguo Dang Dai Er Ke Za Zhi ; 16(2): 129-34, 2014 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-24568904

RESUMO

OBJECTIVE: To study the expression of ecotropic viral integration site (EVI1) gene in childhood acute myeloid leukemia (AML) and the clinical features of EVI1-positive children with AML. METHODS: The clinical data of EVI1-positive children with AML were collected and analyzed. RT-PCR and real-time quantitative PCR were used for qualitative and quantitative analysis of expression of EVI1. Flow cytometry (FCM) was used for determining the immunophenotypes of bone marrow cells. Multiparameter FCM was used for monitoring minimal residual disease. The karyotypes were determined. RESULTS: Of 241 children with AML, 33 (13.7%) were positive for EVI1 expression. There were no significant differences in age at first visit as well as the white blood cell count, hemoglobin level, and platelet count in peripheral blood between EVI1-positive and EVI1-negative children with AML (P>0.05), but EVI1-positive children had a significantly increased proportion of females compared with EVI1-negative children (P<0.05). The change in EVI1 expression was not synchronous with clinical remission and the change of MRD: some children had clinical remission or negative conversion of MRD before negative conversion of EVI1, while some had negative conversion of EVI1 before clinical remission or while MRD showed positive. EVI1 gene was usually co-expressed with other fusion genes. CD33 (100%), CD38 (88%), and HLADR (76%) were highly expressed in EVI1-positive children with AML. Abnormal chromosome structure or number was found in 15 patients. Compared with EVI1-negative children, EVI1-positive children had significantly lower complete remission rates after the first course of treatment (P<0.05). CONCLUSIONS: EVI1-positive children with AML have a poor short-term prognosis. In the development of AML, the activation of EVI1 gene is not isolated, but the result of interactions with other genes or chromosome abnormalities, and the mechanism of activation and its function need further study.


Assuntos
Proteínas de Ligação a DNA/genética , Leucemia Mieloide Aguda/genética , Proto-Oncogenes/genética , Fatores de Transcrição/genética , Adolescente , Criança , Pré-Escolar , Aberrações Cromossômicas , Feminino , Citometria de Fluxo , Regulação Neoplásica da Expressão Gênica , Humanos , Imunofenotipagem , Lactente , Leucemia Mieloide Aguda/imunologia , Proteína do Locus do Complexo MDS1 e EVI1 , Masculino , Neoplasia Residual , Prognóstico
15.
Chin Med J (Engl) ; 126(18): 3446-50, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-24034087

RESUMO

BACKGROUND: Stroke occurs upon obstruction of cerebral blood circulation and is clinically characterized by sudden onset symptoms. Advanced age is the main risk factor of stroke, but cases of pediatric stroke have been rarely reported. This study aimed to determine the etiology, clinical presentation, and radiologic features of neurological deficit for pediatric arterial ischemic stroke (PAIS). METHODS: The medical records of 42 PAIS patients (age range: 9 months to 13 years) treated at Wuhan Children's Hospital between July 2007 and January 2011 were retrospectively reviewed. Infarction location was first determined by craniocerebral computed tomography and magnetic resonance (MR) imaging. The stenotic or occluded main cerebral arteries and/or branches were determined by MR angiography and digital subtraction angiography. RESULTS: The majority of the 42 PAIS cases (66.7%, n = 28) were ≤ 3 years old (vs. >3 years old: 33.3%, n = 14; P<0.05), but the male: female ratio was similar in both groups (P > 0.05). The most frequently reported signs and symptoms for both age groups were limited physical activity followed by convulsions and delirium, but convulsions were more prevalent in children ≤ 3 years-old. Children > 3 years-old mainly experienced the limited physical activity symptoms, including hemiparalysis, aphasia, and ataxia. For all 42 cases, the most frequent etiologies were infections (38.1%, n = 16), iron deficiency anemia (16.7%, n = 7), and moyamoya syndrome (11.9%, n = 5). The predominant infarcts among all cases were middle cerebral artery (63.6%, n = 21) and basal ganglia (64.3%, n = 27). CONCLUSIONS: PAIS occurs more frequently in younger children and this group most frequently presents with convulsion as the initial symptom. The overall etiologies of PAIS may be different from those of adult stroke and the involved regions may be distinguishing features of PAIS or its different forms, but more research is required.


Assuntos
Acidente Vascular Cerebral/diagnóstico por imagem , Acidente Vascular Cerebral/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Radiografia , Estudos Retrospectivos , Acidente Vascular Cerebral/diagnóstico
16.
Exp Ther Med ; 4(1): 93-98, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23060929

RESUMO

Clinical trials have demonstrated that pediatric acute promyelocytic leukemia (APL) is highly curable. Small-scale studies have reported on the treatment of APL using one or two treatment regimes. Here, we report a multiple center-based study of 119 cases of pediatric APL treated with four regimes based on all-trans-retinoic acid (ATRA). We retrospectively analyzed the clinical characteristics, laboratorial test results and treatment outcome of the pediatric APL patients. Regime 1 used an in-house developed protocol, regime 2 was modified from the PETHEMA LPA99 protocol, regime 3 was modified from the European-APL93 protocol, and regime 4 used a protocol suggested by the British Committee for Standards in Haematology. The overall complete remission rates for the four regimes were 88.9, 87.5, 97.1 and 87.5%, respectively, which exhibited no statistical difference. However, more favorable results were observed for regimes 2 and 3 than regimes 1 and 4, in terms of the estimated 3.5-year disease-free survivals, relapse rates, drug toxicity (including hepatotoxicity, cardiac arrhythmia, and differentiation syndrome) and sepsis. In conclusion, the overall outcomes were more favorable after treatment with regimes 2 and 3 than with regimes 1 and 4, and this may have been due to the specific compositions of regimes 2 and 3.

18.
Zhongguo Dang Dai Er Ke Za Zhi ; 13(11): 867-9, 2011 Nov.
Artigo em Chinês | MEDLINE | ID: mdl-22099192

RESUMO

OBJECTIVE: This study compared the differences in clinical features between chronic aplastic anemia (CAA) and myelodysplastic syndrome (MDS) in children in order to provide a basis for the differential diagnosis of the two diseases. METHODS: A retrospective study of 23 cases of CAA and 9 cases of MDS from September 2007 to September 2010 was performed. The clinical data including routine blood test results, reticulocyte counts, serum lactate dehydrogenase level, serum ferritin level, cytological examination of bone marrow, bone marrow CD34+ cell counts, bone marrow chromosome and FISH test results were compared between the CAA and MDS groups. RESULTS: Neutrophils, reticulocytes, and serum ferritin and lactate dehydrogenase levels increased in the MDS group compared with those in the CAA group. There were significant differences in bone marrow blast cell counts and dyshematopoiesis phenomena of three lines blood cells between the CAA and MDS groups. The bone marrow CD34+ cell counts and the rate of chromosomal abnormalities detected in bone marrow cytogenetic analysis in the MDS group were significantly higher than those in the CAA group. CONCLUSIONS: There are differences in the results of laboratory examinations and morphological and cytogenetic examinations of bone marrow between the children with CAA and MDS. The differences are useful to the differential diagnosis of the two diseases.


Assuntos
Anemia Aplástica/patologia , Síndromes Mielodisplásicas/patologia , Anemia Aplástica/genética , Exame de Medula Óssea , Criança , Pré-Escolar , Aberrações Cromossômicas , Doença Crônica , Feminino , Humanos , Masculino , Síndromes Mielodisplásicas/genética
19.
Leuk Res ; 35(12): 1628-31, 2011 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-21802727

RESUMO

BACKGROUND: To analyze the causes and consequence of treatment refusal and abandonment in childhood acute lymphoblastic leukemia (ALL) treated in Wuhan Union Hospital of China. METHODS: We collected recorded data and interviewed families of the children with ALL diagnosed between January 1997 and August 2007, who refused or abandoned treatment. RESULTS: 323 patients were diagnosed with ALL. 173 patients (173/323, 53.6%) refused therapy and 35 (35/323, 10.8%) cases abandoned treatment. 191 (191/208, 91.8%) of these children were telephone/mail-visited. Different people had different reasons for refusal or abandonment. Financial difficulty and belief of ALL incurability were the main reasons for abandonment. Transportation difficulties and fear of severe side effects were also important reasons. Of the 173 patients who refused treatment, 13 patients lost follow-up. 160 parents were interviewed and 1 (6.3%) child was still alive at the date of last follow-up. Of the 35 patients who abandoned treatment, 4 patients lost follow-up. 31 parents were interviewed and 2 (6.5%) children were still alive at the date of last follow-up. CONCLUSION: Medical insurance and a systemic health education are extremely required for childhood ALL in low middle income countries.


Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Recusa do Paciente ao Tratamento/estatística & dados numéricos , Idade de Início , Criança , Maus-Tratos Infantis/estatística & dados numéricos , Maus-Tratos Infantis/tendências , Criança Abandonada/estatística & dados numéricos , Pré-Escolar , China/epidemiologia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Entrevistas como Assunto , Masculino , Relações Pais-Filho , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Sistema de Registros/estatística & dados numéricos , Análise de Sobrevida
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