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1.
Cancer Med ; 2020 Mar 26.
Artigo em Inglês | MEDLINE | ID: mdl-32216042

RESUMO

BACKGROUND: The prognosis of children with acute monocytic leukemia (AML-M5) remains unsatisfactory and the risk profile is still controversial. We aim to investigate the prognostic value of clinical and cytogenetic features and propose a new risk stratification in AML-M5 children. METHODS: We included 132 children with AML-M5. Overall survival (OS) and progression-free survival (PFS) were documented. Cox regression was performed to evaluate the potential risk factors of prognosis. RESULTS: The 5-year-OS was 46.0% (95% confidence intervals, 41.6%-50.4%) in all patients. There was significantly lower OS in the age ≤ 3 years old (P = .009) and hyperleukocytosis (P < .001). The FMS-like tyrosine kinase 3 (FLT3)-internal tandem duplication (ITD) and MLL-rearrangement carriers were associated with fewer survivors in all patients (37.1% and 36.7%) and chemotherapy-only group (19.0% and 35.0%). Notably, the number of survivor with MLL-rearrangement did not increase in hematopoietic stem cell transplant (HSCT) group. According to the Cox regression analysis, HSCT was a significantly favorable factor (P = .001), while hyperleukocytosis, age ≤ 3 years old, and BM blast ≥ 70% adversely affected the OS in all patients (all P < .05). Additionally, FLT3-ITD was a risk factor for OS in the chemotherapy-only group (P = .023), while hyperleukocytosis and age ≤ 3 years independently contributed to poor PFS (both P < .05). In comparison to the standard-risk group, significant poorer outcome was found in the high-risk group (both P < .005). CONCLUSIONS: We propose that AML-M5 children with any of MLL-rearrangement, FLT3-ITD, hyperleukocytosis, BM blast ≥ 70%, or age ≤ 3 years old are classified into the high-risk group, and HSCT is beneficial especially in patients with FLT3-ITD mutation, hyperleukocytosis, and age ≤ 3 years old. Importantly, the choice of HSCT should be made more carefully in children with MLL-rearrangement for its suboptimal performance.

2.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 28(1): 225-229, 2020 Feb.
Artigo em Chinês | MEDLINE | ID: mdl-32027281

RESUMO

OBJECTIVE: To analyze the clinical efficacy and side effects of reduced-dose of cyclophosphamide combined cyclosporine A for severe aplastic anemia(SAA) children. METHODS: Ten pediatric patients with SAA from January 2008 to May 2012 were enrolled. All the patients were treated with reduced dose of cyclophosphamide combined cyclosporine A. The dose of cyclophosphamide was 30 mg/(kg·d)×4 d, the dose of cyclosporine A gradually increased >15 mg/L accroding to the blood concentration. RESULTS: The median follow-up time of the 10 pediatric patients was 100 months (6-126 months). Among 10 children with SAA, 4 cases achieved complete response(CR), 3 cases obtained partial response (PR) and the overall response rate was 70%, the remaining 3 cases showed no response (NR). One refractory patient treated by cyclophosphamide was progressed to paroxysmal nocturnal hemoglobinuria(PNH) at 25 months and was dead at 42 months after therapy. CONCLUSION: The results show that reduced-dose cyclophosphamide (30 mg/kg·d for 4 consecutive days) combinated with CsA (initial dose 4 mg/kg·d, and drugvallery concentration >150 ng/ml) can make 7 of 10 children with severe aplastic anemia achieve complete response or partial response, and this regimen may be the second line regimen selected for some SAA children.


Assuntos
Anemia Aplástica , Soro Antilinfocitário , Criança , Ciclofosfamida , Ciclosporina , Hemoglobinúria Paroxística , Humanos , Imunossupressores , Resultado do Tratamento
3.
Reprod Sci ; 27(1): 93-99, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32046387

RESUMO

Hepatocellular carcinoma upregulated long noncoding RNA (HULC), identified as an oncogene in cervical cancer, is involved in not only the clinical stage, lymph node metastasis, and depth of cervical invasion but also outcome. In this study, we aimed to investigate the association between 3 polymorphisms (i.e., rs1041279, rs3005167, and rs7770772) in the promoter of HULC and the risk of cervical squamous cell carcinoma (CSCC). The polymorphisms were genotyped using the multiplex ligase detection reaction assay. The promoter activity was measured using the dual-luciferase reporter assay kit. The rs1041279 GG genotype and G allele revealed a significantly higher risk of CSCC compared with the rs1041279 CC genotype and C allele (GG vs. CC, adjusted OR = 1.79, 95% CI, 1.17-2.73, P = 0.007; G vs. C, adjusted OR = 1.36, 95% CI, 1.09-1.69, P = 0.006). Haplotype analysis revealed that the rs3005167C-rs7770772G-rs1041279C or rs3005167C-rs7770772G-rs1041279G haplotype had a significantly higher risk of CSCC compared to the rs3005167G-rs7770772G-rs1041279C haplotype (CGC vs. GGC, OR = 2.38, 95% CI, 1.53-3.75, P < 0.001; CGG vs. GGC, OR = 3.76, 95% CI, 2.12-6.68, P < 0.001). Dual-luciferase reporter assay showed that the rs1041279 G promoter resulted in higher transcriptional activity compared with the rs1041279 C (P < 0.01). Additionally, the rs1041279 GG genotype carriers had an increased level of HULC expression (P = 0.03). These findings suggest that the HULC rs1041279 may be a useful marker for the etiology of CSCC.

4.
J Colloid Interface Sci ; 565: 315-325, 2020 Jan 16.
Artigo em Inglês | MEDLINE | ID: mdl-31978794

RESUMO

The carbon layer with good electrical conductivity and outstanding mechanical stability are essential in designing high-performance silicon/carbon (Si/C) anodes to replace the commercial graphite in lithium-ion batteries (LIBs). In terms of solving the two inherent defects of poor conductivity and big volume change of silicon, we fabricate a spongy carbon matrix derived from ZIF-8 to anchor saclike silicon synthesized by molten salt magnesiothermic reduction method. This spongy matrix can anchor saclike silicon to provide a stable reaction interface and support fast electronic transmission. At the same time, buffer space in saclike Si nanoparticles and spongy matrix can synergistically accommodate the volume change of Si to maintain the integrity of the electrode. The resulting composite with a high Si content of 77.58% exhibits good capacities of 1448 mAh g-1 at 2 A g-1 and 848 mAh g-1 at 4 A g-1 after 500 cycles. High initial coulombic efficiency of 84% at 0.2 A g-1 is also exhibited in the first three activation cycles. Therefore, this novel multifunctional N-doped spongy matrix can supply multifaceted benefits in accommodation of volumetric variation, enhancement of conductivity, and integrity of structure during cycling.

5.
Sci Rep ; 9(1): 20174, 2019 Dec 27.
Artigo em Inglês | MEDLINE | ID: mdl-31882825

RESUMO

Under a controlled adsorption environment, L-cysteine molecules can be chemically adsorbed to the dendritic silver (Ag-D) surface by electrochemical methods with different functional groups. It is verified by surface-enhanced Raman spectroscopy that under alkaline conditions (pH = 13.50), the two functional groups of thiol and acid are simultaneously adsorbed on the surface of Ag-D, while NH2 is far from the surface; under acidic conditions (pH = 1.67), adsorption behavior suggests that both NH3+ and COO- are oriented toward the Ag-D surface, and that SH is far from the surface. The structure of L-cysteine adsorption under acidic conditions can be further verified by the addition of an L-cysteine molecule through light-induced coupling reaction to form cystine. Finally, in-situ two-dimensional Raman scattering spectroscopy confirmed the feasibility and uniformity of the coupling reaction.

6.
Zhongguo Dang Dai Er Ke Za Zhi ; 21(9): 890-893, 2019 Sep.
Artigo em Chinês | MEDLINE | ID: mdl-31506148

RESUMO

OBJECTIVE: To study the long-term clinical effect of the CCLG-ALL2008 regimen in the treatment of children newly diagnosed with acute lymphoblastic leukemia (ALL) with different molecular biological features. METHODS: A total of 940 children who were newly diagnosed with ALL were enrolled in this study. The children were treated with the CCLG-ALL2008 regimen. A retrospective analysis was performed for the long-term outcome of ALL children with different molecular biological features. RESULTS: Among the 940 children with ALL, there were 570 boys and 370 girls, with a median age of onset of 5 years (range 1-15 years) and a median follow-up time of 65 months (range 3-123 months). The complete response (CR) rate was 96.7%, the predicted 10-year overall survival (OS) rate was 76.5%±1.5%, and the event-free survival (EFS) rate was 62.6%±3.0%. After CR was achieved after treatment, the overall recurrence rate was 21.9%. The children with positive ETV6-RUNX1 had the lowest recurrence rate and were prone to late recurrence, and those with positive MLL rearrangement had the highest recurrence rate and were prone to early recurrence. The children with positive ETV6-RUNX1 had a significantly higher predicted 10-year OS rate than those with positive TCF3-PBX1, BCR-ABL, or MLL rearrangement and those without molecular biological features (P<0.05). The children with positive ETV6-RUNX1 had a significantly higher predicted 10-year EFS rate than those with positive BCR-ABL or MLL rearrangement (P<0.05). CONCLUSIONS: Molecular biological features may affect the long-term prognosis of children with ALL, and positive MLL rearrangement and BCR-ABL fusion gene are indicators of poor prognosis. Children with positive ETV6-RUNX1 fusion gene have the highest long-term survival rate.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia-Linfoma Linfoblástico de Células Precursoras , Adolescente , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Proteínas de Fusão bcr-abl , Humanos , Lactente , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Prognóstico , Estudos Retrospectivos
7.
Gynecol Endocrinol ; 35(12): 1059-1062, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31185760

RESUMO

To evaluate the feasibility and clinical value of three-dimensional ultrasound in evaluating ovarian function in perimenopausal women. In this prospective cohort study, 102 patients with clinically suspected perimenopause and 90 patients with menopause were enrolled. These patients were classified into three groups according to the level of follicle stimulating hormone (FSH) and estradiol (E2): menopause group, perimenopause group, and normal group. Perimenopause group: There were significant differences in volume, vascularization index (VI), flow index (FI), and vascularization-flow index (VFI) in the ovaries after treatment. Cycle 1 > cycle 0 (p < .05) and cycle 3 cycle 0 (p < .05), and in FSH: cycle 3 < cycle 0 (p < .05). Three-dimensional ultrasound in ovarian quantitative measurement can objectively reflect the change in the ovarian function, predicting the effect of drug treatment, and provided an objective information for early intervention to menopausal.

8.
Pediatr Res ; 86(3): 360-364, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31112993

RESUMO

BACKGROUND: In severe aplastic anemia (SAA), predictive markers of response to immunosuppressive therapy (IST) of porcine antilymphocyte globulin (pALG) have not been well defined. We investigated whether clinical and laboratory findings before treatment could predict response in a pediatric cohort. METHODS: In this study, we included 70 newly diagnosed SAA children and treated them with pALG. The response rate was documented during follow-up. The log-rank test compared response rates between the potential predictive factors. RESULTS: The response rate was 57.1% at 24 months follow-up. In log-rank test, mild disease severity was the most significant predictive marker of better response (P < 0.001); SAA patients with higher absolute reticulocyte count (ARC) and platelet level showed a higher response rate (both P < 0.001). Although insignificantly, elderly children and male sex show better response rate after treatment. The response rate worsened when the time interval before IST was more than 60 days. CONCLUSION: Modified IST with pALG was suitable for SAA children, and favorable response correlates with mild disease severity was identified. ARC and platelet status also appeared to be a reproducible prognostic model for response rate. IST should be started as soon as possible, given that the response rate worsens as the interval between diagnosis and treatment increases.

9.
Zhongguo Dang Dai Er Ke Za Zhi ; 21(1): 24-28, 2019 Jan.
Artigo em Chinês | MEDLINE | ID: mdl-30675859

RESUMO

OBJECTIVE: To investigate the complications and clinical outcome of children with acute myeloid leukemia (AML) undergoing mitoxantrone-cytarabine-etoposide (MAE) induction therapy. METHODS: A total of 170 children with AML were given MAE induction therapy, and the complications and remission rate were analyzed after treatment. RESULTS: The male/female ratio was 1.33:1 and the mean age was 7.4 years (range 1-15 years). Leukocyte count at diagnosis was 29.52×109/L [range (0.77-351)×109/L]. Of all children, 2 had M0-AML, 24 had M2-AML, 2 had M4-AML, 48 had M5-AML, 3 had M6-AML, 7 had M7-AML, 69 had AML with t(8;21)(q22;q22), and 15 had AML with inv(16)(p13.1q22) or t(16;16)(p13.1;q22). The most common complication was infection (158/170, 92.9%). Among these 158 patients, 22 (13.9%) had agranulocytosis with pyrexia (with no definite focus of infection), and 136 (86.1%) had definite focus of infection (including bloodstream infection). Other complications included non-infectious diarrhea, bleeding, and drug-induced hepatitis. Treatment-related mortality was observed in 10 children, among whom 8 had severe infection, 1 had multiple organ failure, and 1 had respiratory failure. Remission rate was evaluated for 156 children and the results showed a complete remission rate of 85.3%, a partial remission rate of 4.5%, and a non-remission rate of 10.3%. CONCLUSIONS: Induction therapy with the MAE regimen helps to achieve a good remission rate in children with AML after one course of treatment. Infection is the main complication and a major cause of treatment-related mortality.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Leucemia Mieloide Aguda , Adolescente , Criança , Pré-Escolar , Citarabina , Esquema de Medicação , Etoposídeo , Feminino , Humanos , Lactente , Leucemia Mieloide Aguda/tratamento farmacológico , Masculino , Mitoxantrona , Indução de Remissão
10.
3 Biotech ; 8(9): 380, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30148030

RESUMO

To develop a new strategy that controls vascular pathogen infections in economic crops, we examined a possible enhancer of the vascular activity of XYLOGEN PROTEIN 1 promoter (Px). This protein is specifically expressed in the vascular tissues of Arabidopsis thaliana and plays an important role in xylem development. Although Px is predicted as vascular-specific, its activity is hard to detect and highly susceptible to plant and environmental conditions. The cauliflower mosaic virus 35S promoter (35S) is highly active in directing transgene expression. To test if 35S could enhance Px activity, while vascular specificity of the promoter is retained, we examined the expression of the uidA reporter gene, which encodes ß-glucuronidase (GUS), under the control of a chimeric promoter (35S-Px) or Px by generating 35S-Px-GUS and Px-GUS constructs, which were transformed into tobacco seedlings. Both 35S-Px and Px regulated gene expression in vascular tissues. However, GUS expression driven by 35S-Px was not detected in 30- and 60-day-old plants. Quantitative real-time PCR analysis showed that GUS gene expression regulated by 35S-Px was 6.2-14.9-fold higher in vascular tissues than in leaves. Histochemical GUS staining demonstrated that 35S-Px was strongly active in the xylem and phloem. Thus, fusion of 35S and Px might considerably enhance the strength of Px and increase its vascular specificity. In addition to confirming that 35S enhances the activity of a low-level tissue-specific promoter, these findings provide information for further improving the activity of such promoters, which might be useful for engineering new types of resistant genes against vascular infections.

11.
Zhongguo Shi Yan Xue Ye Xue Za Zhi ; 26(3): 642-646, 2018 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-29950197

RESUMO

OBJECTIVE: To explore the HER22 expression in children with ETV6/RUNX1 (E/R)-positive acute lymphoblastic leukemia(ALL) and to investigate the relationship between the HER2 expression and clinical features. METHODS: Thirty-seven newly diagnosed E/R-positive ALL children and 6 controls (4 cases of ITP and 2 healthy children) were selected in Institute of hematology and blood disease hospital. The 37 patients were divided into standard risk (SR), intermediate risk(IR), high risk(HR) groups according to risk stratification; and they were divided into relapse and non-relapse groups according to follow-up result. The CD10+CD19+ cells were sorted by flow cytometry. The mRNA was extracted from these cells. Real-time fluorescent quantitative PCR was used to detect the expression level of HER2. RESULTS: Among the 37 cases, 51.35% (n=19) were boys and 48.65% (n=18) were girls and their median age was 4.72 (1.72-11.99) years old. Among the 6 controls, 50% (n=3) were boys and 50% (n=3) were girls and the median age was 5.24 (1.53-13.17) years old. The expression level of HER2 in E/R-positive ALL patients were lower than that in controls (P<0.05). Although the difference of HER2 expression level between the 2 groups failed to achieve statistical significance, the expression level of HER2 in relapse patients were significantly lower than that in non-relapse patients, and the HER2 expression in HR group patients were lower than that in SR and IR groups. In addition, there was no significant correlation between the expression level of HER2 and the sex, age, initial white blood cell count, blast cell percentage and the level of LDH (P>0.05). CONCLUSION: The expression level of HER2 in E/R+ ALL patients is lower than that in controls, and in relapse group lower than that in non-relapse patient. Thus, HER2 may play important roles in the pathogenesis and relapse mechanism of pediatric E/R-positive ALL patients.


Assuntos
Leucemia-Linfoma Linfoblástico de Células Precursoras , Adolescente , Criança , Pré-Escolar , Subunidade alfa 2 de Fator de Ligação ao Core , Feminino , Citometria de Fluxo , Humanos , Lactente , Masculino , Prognóstico , Proteínas Proto-Oncogênicas c-ets , Receptor ErbB-2 , Recidiva , Proteínas Repressoras
12.
Compr Psychiatry ; 84: 47-53, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29684660

RESUMO

OBJECTIVE: The present study aims to provide a comprehensive profile of the primary family caregivers of schizophrenia individuals in rural China. METHOD: A cross-sectional study was conducted with a sample of 327 primary family caregivers of schizophrenia individuals recruited through a one-stage cluster sampling in Ningxiang County of Hunan province, China. The social demographic and psychological profiles of primary caregivers were measured using standard scales and self-designed scales. RESULTS: The typical caregiver profile consists of a 58-year old married first degree relative (mostly parents or spouses) with a low socio-economic position. Most of them have been caregiving for over 10 years (74.3%) and have some physical illness (67.0%). The major caregiving activities were medicine management (71.6%) and hospital visit (69.4%), yet there is still 17.1% primary caregivers involved with neither of the care. Most (84%) of caregivers reported some kind of burden, with anxiety in 45.9% of caregivers and depression in 45.4%. Family caregivers also reported positive aspects of caregiving including a well-functional family (51.0%) and rewarding feelings (58.3%). CONCLUSION: The findings of the present study have brought attention to a special group of family caregivers for schizophrenia, with implications for intervention on them in the future.


Assuntos
Cuidadores/psicologia , Família/psicologia , População Rural , Esquizofrenia/terapia , Psicologia do Esquizofrênico , Adulto , Idoso , Ansiedade/epidemiologia , Ansiedade/psicologia , Ansiedade/terapia , Cuidadores/economia , China/epidemiologia , Efeitos Psicossociais da Doença , Estudos Transversais , Depressão/epidemiologia , Depressão/psicologia , Depressão/terapia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pais/psicologia , Esquizofrenia/epidemiologia , Fatores Socioeconômicos , Inquéritos e Questionários
13.
Biomed Pharmacother ; 98: 88-94, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29247951

RESUMO

Cervical cancer is one of the most common malignant carcinomas in the female reproductive system. Long non-coding RNAs (lncRNAs) have been verified to participate in the tumorigenesis of cervical cancer. In present study, we investigate the role of lncRNA XLOC_008466 in the occurrence and progression of cervical cancer. Results showed that XLOC_008466 expression was up-regulated in cervical cancer tissue and cells compared to normal controls. In vitro functional experiments, CCK-8 assay and colony formation assay showed that XLOC_008466 knockdown suppressed the proliferation of cervical cancer cells. Flow cytometry and transwell assay showed that XLOC_008466 knockdown induced G0/G1 phase arrest and aggravated the apoptosis. In vivo, XLOC_008466 knockdown inhibited the tumor growth. Bioinformatics analysis revealed that XLOC_008466 sponged miR-216b with the complementary binding sites at 3'-UTR. Overall, our study reveals the tumor promoting role of XLOC_008466 in cervical cancer carcinogenesis, providing a novel molecular mechanism and therapeutic target for cervical cancer.


Assuntos
Carcinogênese/genética , RNA Longo não Codificante/genética , Neoplasias do Colo do Útero/genética , Neoplasias do Colo do Útero/patologia , Regiões 3' não Traduzidas/genética , Animais , Apoptose/genética , Pontos de Checagem do Ciclo Celular/genética , Linhagem Celular Tumoral , Proliferação de Células/genética , Feminino , Fase G1/genética , Células HeLa , Humanos , Camundongos , Camundongos Nus , Fase de Repouso do Ciclo Celular/genética , Regulação para Cima/genética
14.
Oncotarget ; 8(17): 28052-28062, 2017 Apr 25.
Artigo em Inglês | MEDLINE | ID: mdl-28427208

RESUMO

Previous studies have shown that mammalian cardiac tissue has a regenerative capacity. Remarkably, neonatal mice can regenerate their cardiac tissue for up to 6 days after birth, but this capacity is lost by day 7. In this study, we aimed to explore the expression pattern of long noncoding RNA (lncRNA) during this period and examine the mechanisms underlying this process. We found that 685 lncRNAs and 1833 mRNAs were differentially expressed at P1 and P7 by the next-generation high-throughput RNA sequencing. The coding genes associated with differentially expressed lncRNAs were mainly involved in metabolic processes and cell proliferation, and also were potentially associated with several key regeneration signalling pathways, including PI3K-Akt, MAPK, Hippo and Wnt. In addition, we identified some correlated targets of highly-dysregulated lncRNAs such as Igfbp3, Trnp1, Itgb6, and Pim3 by the coding-noncoding gene co-expression network. These data may offer a reference resource for further investigation about the mechanisms by which lncRNAs regulate cardiac regeneration.


Assuntos
Regulação da Expressão Gênica , Miocárdio/metabolismo , RNA Longo não Codificante/genética , Regeneração/genética , Animais , Animais Recém-Nascidos , Apoptose/genética , Biologia Computacional/métodos , Perfilação da Expressão Gênica , Ontologia Genética , Redes Reguladoras de Genes , Genômica/métodos , Sequenciamento de Nucleotídeos em Larga Escala , Camundongos , Camundongos Endogâmicos C57BL , Reprodutibilidade dos Testes , Análise de Sequência de RNA
15.
Zhongguo Dang Dai Er Ke Za Zhi ; 19(1): 27-33, 2017 Jan.
Artigo em Chinês | MEDLINE | ID: mdl-28100318

RESUMO

OBJECTIVE: To evaluate the clinical characteristics and risk factors of clonal evolution after immunosuppressive therapy (IST) in children with severe/very severe aplastic anemia (SAA/VSAA). METHODS: The clinical data of 231 children with newly-diagnosed SAA/VSAA who received IST were retrospectively studied. The incidence and risk factors of clonal evolution after IST were analyzed. RESULTS: The 5-year overall survival rate of the 231 patients was 82.7%. Except for 18 cases of early deaths, 213 patients were evaluated for IST efficacy. Among the 231 patients, cytogenetic abnormalities for at least two chromosome metaphase were detectable in 14 (7.4%) patients, and PNH clones were detectable in either peripheral red blood cells or neutrophils for 95 patients. Among the 213 patients evaluated for IST efficacy, 15 patients experienced clonal evolution after IST. Five patients had PNH and trisomy 8 which were defined as favorable progressions, and ten patients experienced monosomy 7 and MDS/AML as unfavorable progressions. The 5-year accumulative incidence of favorable and unfavorable progression were (2.2±2.2)% and (4.8±3.3)%, respectively. Until the last follow-up, 100% (5/5) of patients with favorable progressions and 50% (5/10) of patients with unfavorable progressions survived. WBC>3.5×109/L, CD3+T cell percentage>80%, dosage of antithymocyte globulin >3.0 mg/(kg·d) and no response to IST were related to unfavorable progressions by univariate analysis. Cox multivariate analysis revealed that an increased CD3+T cell percentage (>80%) and no response to IST were independent risk factors for unfavorable progressions. CONCLUSIONS: The children with SAA/VSAA who have an increased CD3+T cell percentage at diagnosis or have no response to IST are in high risks of unfavorable progressions.


Assuntos
Anemia Aplástica/tratamento farmacológico , Evolução Clonal , Imunossupressores/uso terapêutico , Adolescente , Anemia Aplástica/genética , Anemia Aplástica/imunologia , Anemia Aplástica/mortalidade , Criança , Pré-Escolar , Aberrações Cromossômicas , Feminino , Humanos , Lactente , Masculino , Modelos de Riscos Proporcionais , Estudos Retrospectivos
16.
BMC Pediatr ; 16(1): 207, 2016 12 08.
Artigo em Inglês | MEDLINE | ID: mdl-27931195

RESUMO

BACKGROUND: Ventricular septal defect (VSD) is a highly prevalent fetal congenital heart defect, which can become spontaneously closed during infancy. The current study aims to characterize fetal VSDs that were subsequently spontaneously closed in the first 2 years of life in eastern China. METHODS: Between January 2011 and December 2013, 257 fetal patients diagnosed with isolated VSD by fetal echocardiography at Nanjing Maternity and Child Health Care Hospital, China, were enrolled in the study. Subjects were divided into three groups: group 1 = persistent VSD; group 2 = closed after birth; group 3 = closed during gestation. Fetal echocardiography data, physical features at birth and follow-up outcomes for 2 years were compared to identify factors contributing to spontaneous closure (SC) of VSD. A predictive formula was applied to patients admitted to hospital in the first quarter of 2014 (n = 23) for validation. RESULTS: SC occurred in 42.8% patients. Birth weight (3.095 ± 0.774, 3.174 ± 0.535, 3.499 ± 0.532 kg in groups 1, 2 and 3, respectively) and defect diameter (3.422 ± 0.972, 2.426 ± 0.599, 2.292 ± 0.479 mm, in groups 1, 2 and 3, respectively) showed statistically significant differences between the three groups (P = 0.004 and P = 0.000, respectively). Receiver operating characteristic (ROC) curves identified cut-off value for the defect diameter as 2.55 mm, and logistic regression analysis identified the SC probability = (1 + exp -[-2.151 - 0.716*birth weight + 1.393*diameter])-1. Results indicated that male fetuses, full-term birth, muscular VSD, and defects without blood flow crossing the septum, have higher incidence of SC. CONCLUSIONS: The major determinants of SC of isolated VSD are birth weight and diameter of the defect. In addition, VSD location may also affect the SC incidence.


Assuntos
Comunicação Interventricular/diagnóstico , Pré-Escolar , Técnicas de Apoio para a Decisão , Ecocardiografia , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Gravidez , Prognóstico , Curva ROC , Remissão Espontânea , Estudos Retrospectivos , Ultrassonografia Pré-Natal
17.
Yi Chuan ; 38(10): 894-901, 2016 10 20.
Artigo em Inglês | MEDLINE | ID: mdl-27806930

RESUMO

Antibiotic resistance has become a serious concern in treatment of bacterial infections. Overexpression of efflux pump is one of the important mechanisms in antibiotic resistance. In Gram negative bacteria, RND (Resistance-nodulation-cell division) superfamily efflux pump plays a vital important role in antibiotics resistance. Recent research progress unveils an intriguing interrelationship between RND efflux pump and the bacterial quorum sensing system, whose regulation is dependent on small signal molecules. This article reviews the latest findings on the structure and transport mechanism of RND efflux pump, as well as the general features and regulatory mechanisms of quorum sensing, with a special focus on the role and mechanism of quorum sensing system in regulation of RND efflux pump, and the influence of efflux pump on quorum sensing signal transportation. Further investigation of the interrelationship between RND efflux pumps and the bacterial quorum sensing systems is critical for elucidation of the regulatory mechanisms that govern the expression of the RND efflux pumps genes, and may also provide useful clues to overcome the efflux pump mediated antibiotic resistance.


Assuntos
Proteínas de Bactérias/metabolismo , Bactérias Gram-Negativas/fisiologia , Proteínas de Membrana Transportadoras/metabolismo , Percepção de Quorum , Antibacterianos/farmacologia , Proteínas de Bactérias/genética , Bactérias Gram-Negativas/efeitos dos fármacos , Bactérias Gram-Negativas/genética
18.
Zhongguo Dang Dai Er Ke Za Zhi ; 18(8): 742-5, 2016 Aug.
Artigo em Chinês | MEDLINE | ID: mdl-27530793

RESUMO

OBJECTIVE: To investigate the association between clinical outcome and gene mutations in children with Fanconi anemia (FA). METHODS: A retrospective analysis was performed for the clinical data of six children with the same severity of FA and receiving the same treatment. At first, single cell gel electrophoresis and chromosome breakage induced by mitomycin C were performed for diagnosis. Then the gene detection kit for congenital bone marrow failure diseases or complementation test was used for genotyping of FA. Finally the association between the clinical outcome at 3, 6, 9, or 12 months after treatment and gene mutation was analyzed. RESULTS: Of all the six FA children, five had FANCA type disease, and one had FANCM type disease; four children carried two or more FA gene mutations. Among the children with the same severity of FA, those with more FA mutations had a younger age of onset and poorer response to medication, and tended to progress to a severe type. CONCLUSIONS: Children carrying more than two FA mutations have a poor clinical outcome, and hematopoietic stem cell transplantation should be performed as soon as possible.


Assuntos
Anemia de Fanconi/genética , Mutação , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Retrospectivos
19.
Cell Physiol Biochem ; 38(5): 1999-2014, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27160009

RESUMO

BACKGROUND: Ventricular septal defect (VSD) is one of the most common congenital heart diseases and to date the role of peptides in human amniotic fluid in the pathogenesis of VSD have been rarely investigated. METHODS: To gain insight into the mechanisms of protein and peptides in cardiovascular development, we constructed a comparative peptidomic profiling of human amniotic fluid between normal and VSD fetuses using a stable isobaric labeling strategy involving tandem mass tag reagents, followed by nano liquid chromatography tandem mass spectrometry. RESULTS: We identified and quantified 692 non-redundant peptides, 183 of which were differentially expressed in the amniotic fluid of healthy and VSD fetuses; 69 peptides were up regulated and 114 peptides were down regulated. These peptides were imported into the Ingenuity Pathway Analysis (IPA) and identified putative roles in cardiovascular system morphogenesis and cardiogenesis. CONCLUSION: We concluded that 35 peptides located within the functional domains of their precursor proteins could be candidate bioactive peptides for VSD. The identified peptide changes in amniotic fluid of VSD fetuses may advance our current understanding of congenital heart disease and these peptides may be involved in the etiology of VSD.


Assuntos
Líquido Amniótico/metabolismo , Comunicação Interventricular/patologia , Peptídeos/análise , Adulto , Estudos de Casos e Controles , Cromatografia Líquida de Alta Pressão , Biologia Computacional , Regulação para Baixo , Ecocardiografia , Feminino , Idade Gestacional , Comunicação Interventricular/diagnóstico por imagem , Comunicação Interventricular/metabolismo , Humanos , Marcação por Isótopo , Redes e Vias Metabólicas , Nanotecnologia , Eletroforese em Gel de Poliacrilamida Nativa , Espectrometria de Massas em Tandem , Regulação para Cima
20.
Zhongguo Dang Dai Er Ke Za Zhi ; 18(4): 287-91, 2016 Apr.
Artigo em Chinês | MEDLINE | ID: mdl-27097569

RESUMO

OBJECTIVE: To identify the incidence of PAX5 deletion in childhood B-lineage acute lymphoblastic leukemia (B-ALL) without reproducible chromosomal abnormalities and to investigate the association between PAX5 abnormalities and prognosis of ALL. METHODS: Multiplex ligation-dependent probe amplification was used to determine the copy numbers of PAX5 gene in children newly diagnosed with B-ALL without reproducible chromosomal abnormalities between April 2008 and April 2013 and controls (children with non-hematologic diseases or tumors). The patients were classifiied into deletion group and non-deletion group based on the presence of PAX5 deletion. RESULTS: Eighteen (21%) out of 86 children with B-ALL had PAX5 deletion. The deletion group had a significantly higher total white blood cell count at diagnosis than the non-deletion group (P=0.001). The Kaplan-Meier analysis demonstrated that the deletion group had a significantly lower disease-free survival (DFS) rate than the non-deletion group (0.69±0.12 vs 0.90±0.04; P=0.017), but there was no significant difference in the overall survival rate between the two groups (P=0.128). The Cox analysis showed that PAX5 deletion was a risk factor for DFS (P=0.03). CONCLUSIONS: PAX5 deletion is an independent risk factor for DFS in B-ALL children without reproducible chromosomal abnormalities.


Assuntos
Deleção de Genes , Fator de Transcrição PAX5/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras B/genética , Doença Aguda , Adolescente , Linhagem da Célula , Criança , Pré-Escolar , Aberrações Cromossômicas , Intervalo Livre de Doença , Feminino , Humanos , Lactente , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras B/mortalidade
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