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OBJECTIVES@#To investigate the effectiveness of high-dose chemotherapy combined with autologous hematopoietic stem cell transplantation (ASCT) in the treatment of children with high-risk neuroblastoma (NB).@*METHODS@#A retrospective analysis was performed on 29 children with high-risk NB who were admitted to Shanghai Children's Hospital and were treated with high-dose chemotherapy combined with ASCT from January 2013 to December 2021, and their clinical features and prognosis were analyzed.@*RESULTS@#Among the 29 children treated by high-dose chemotherapy combined with ASCT, there were 18 boys (62%) and 11 girls (38%), with a median age of onset of 36 (27, 59) months. According to the International Neuroblastoma Staging System, 6 children (21%) had stage III NB and 23 children (79%) had stage IV NB, and the common metastatic sites at initial diagnosis were bone in 22 children (76%), bone marrow in 21 children (72%), and intracalvarium in 4 children (14%). All 29 children achieved reconstruction of hematopoietic function after ASCT. After being followed up for a median time of 25 (17, 45) months, 21 children (72%) had continuous complete remission and 8 (28%) experienced recurrence. The 3-year overall survival rate and event-free survival rate were 68.9%±16.1% and 61.4%±14.4%, respectively. Presence of bone marrow metastasis, neuron-specific enolase ≥370 ng/mL and positive bone marrow immunophenotyping might reduce the 3-year event-free survival rate (P<0.05).@*CONCLUSIONS@#Children with high-risk NB who have bone marrow metastasis at initial diagnosis tend to have a poor prognosis. ASCT combined with high-dose chemotherapy can effectively improve the prognosis of children with NB with a favorable safety profile.
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Preescolar , Femenino , Humanos , Masculino , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Neoplasias de la Médula Ósea/tratamiento farmacológico , China , Terapia Combinada , Supervivencia sin Enfermedad , Trasplante de Células Madre Hematopoyéticas , Neuroblastoma/patología , Pronóstico , Estudios Retrospectivos , Trasplante de Células Madre , Trasplante AutólogoRESUMEN
Objective: To analyze the clinical characteristics and long-term outcomes with multicenter study for acute lymphoblastic leukemia (ALL) in children over 10 years old and adolescents. Method: Newly diagnosed ALL patients aged from 10 to 18 years old in three hospitals were included in the study from May 1(st) 2005 to April 30(th) 2015. They were received ALL-2005/2009 protocol following up to December 31(st) 2016. The clinical characteristics, outcomes and the prognostic analysis were evaluated between the two protocols. Results: Totally, 237 patients were involved in the study, 76 cases for ALL-2005 and 161 cases for ALL-2009 protocol. Complete remission (CR) after induction therapy was 94.5%. 64 (28.6%) patients relapsed with a median time of 14.5 months and 70 (29.5%) patients passed away during the following time. In long-term follow-up, the 5-year event-free survival (EFS) and 5-year overall survival (OS) of ALL patients were (63.1±3.3)% and (68.4±3.2)%. The 7-year EFS and OS were (61.0±3.5)% and (67.6±3.3)%.The 5-year EFS of intermediate risk group in ALL-2005 and ALL-2009 protocol were (73.6±6.1)% and (71.7±4.3)% with no difference (χ(2)=0.064, P=0.801). The 5-year EFS of high risk group in two protocols were (27.6±9.6)% and (33.9±9.3)%, showing no significant difference (χ(2)=0.296, P=0.586). Five years relapsed rate of two protocols were (33.8±5.7)% and (32.6±4.1)% with no difference (χ(2)=0.055, P=0.815). The mortalities were 36.8% and 29.8% separately (χ(2)=2.869, P=0.090). Univariate analysis indicated that age, male, risk, BCR/ABL translocation/t(9;22) and resistant to induction were risk prognostic factors in long-term survival (χ(2)=4.764, 4.796, 46.410, 9.560, 25.450; P=0.029, 0.029, <0.001, 0.049, <0.001). Cox multivariate analysis showed male, risk and resistant to induction were independent risk prognostic factors (RR=1.790, 2.727, 2.719; P=0.021, 0.000, 0.012). Conclusion: Protocol ALL-2009 enhanced the chemotherapy intensity in intermediate risk group with no benefit of survival. BCR-ABL fusion or t(9;22) translocation was still the risk factor of prognosis. TKI inhibitor used in these patients could improve survival. EFS rate was increased a little and death rate was decreased in ALL-2009 protocol with no significant lower relapsed rate comparing with ALL-2005 protocol.
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Adolescente , Niño , Humanos , Masculino , Protocolos de Quimioterapia Combinada Antineoplásica , Supervivencia sin Enfermedad , Leucemia-Linfoma Linfoblástico de Células Precursoras , Pronóstico , Inducción de Remisión , Factores de Riesgo , Translocación GenéticaRESUMEN
<p><b>OBJECTIVE</b>To study the clinical significance of sequentially monitoring minimal residual disease (MRD) in childhood B-cell acute lymphoblastic leukemia (B-ALL).</p><p><b>METHOD</b>Eighty one B-ALL cases were enrolled in the study from January 2004 to December 2009. Leukemia cell markers were detected by flow cytometry at diagnosis, then regularly followed-up.</p><p><b>RESULTS</b>Of 81 cases, 80 achieved complete remission (CR) after induction therapy, 5-year event-free survival (EFS) was (76.80 ± 5.70)%. Among them, the EFS was (89.40 ± 5.90)% in standard risk group and (66.99 ± 13.60)% in intermediate risk group. Eight cases were screened for leukemia markers for MRD monitoring and identified in 68; and 5-year EFS was (79.10 ± 6.20)% and (62.50 ± 15.10)% (P > 0.05, respectively). MRD detection at day 35 in induction therapy showed that 52 of 68 cases were MRD negative (leukemia cells < 0.01%), the 5-year EFS being (88.50 ± 4.90)%, and 16 were MRD positive (leukemia cells ≥ 0.01%), the 5-year EFS being (42.10 ± 20.10)% (P > 0.05). Univariate analysis confirmed that there was a correlation between MRD monitoring and risk stratification. MRD detection at day 55 showed that among the 52 day 35 MRD negative cases, 51 were still negative, 1 positive, among 16 day 35 MRD positive cases, 14 (87.50%) turned negative, 2 still positive. Of the 68 cases, 9 were MRD positive within one year after CR (3 relapsed), 4 MRD positive after one year (2 relapsed) and 55 MRD negative (4 relapsed) (P > 0.05).</p><p><b>CONCLUSIONS</b>Sequential monitoring MRD can find out treatment outcome and adjust therapy in time.</p>
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Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Linfocitos B , Citometría de Flujo , Neoplasia Residual , Diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras , Diagnóstico , Pronóstico , Resultado del TratamientoRESUMEN
<p><b>BACKGROUND</b>Great advances have been made in the diagnosis, molecular pathogenesis and treatment of acute lymphoblastic leukemia (ALL) in the past decade. Due to the lack of large population-based studies, the recent trends in the incidence and geographic variations of ALL in Shanghai, China have not been well documented. To better understand the incidence and epidemiological features of ALL in Shanghai, we conducted a retrospective survey based on the database from the Shanghai Center for Disease Control and Prevention (CDC) and the medical records in all large-scale hospitals in Shanghai, especially those 30 major hospitals with hematology department.</p><p><b>METHODS</b>According to the data from Shanghai CDC, 544 patients, with a median age of 32 years (ranging 1.2 - 89 years), were diagnosed as de novo ALL from January 1, 2002 to December 31, 2006, and they were followed up until December 31, 2007.</p><p><b>RESULTS</b>The average annual incidence of ALL in Shanghai was 0.81/100 000. The incidence in men (0.86/100 000) was slightly higher than that in women (0.75/100 000). The age-stratified incidence showed that the incidence was 2.31/100 000 in patients ≥ 17 years old, 0.54/100 000 in those 18 - 34 years old, 0.46/100 000 in those 35 - 59 years old, and 0.94/100 000 in those ≥ 60 years old. Moreover, there were substantial geographic variations in the incidence of ALL, with the incidence in Chongming county, an island in the east of Shanghai city being 0.60/100 000, much lower than those of other districts. Both French-American-British (FAB) and World Health Organization (WHO) classification systems were applied in the present study. Eighty-eight patients were diagnosed as L1 (26.2%), 193 L2 (57.4%), and 55 L3 (16.4%). For 302 patients with immunophenotypic results, 242 were identified as B cell origin (80.1%), 59 as T cell origin (19.5%), and 1 as biphenotype (0.4%). The leukemia cells in 61 patients co-expressed one or two myeloid antigen (20.2%). For 269 patients with cytogenetic results, the incidences of t(9;22) in patients aged < 10, 11 - 17, 18 - 44, 45 - 59 and ≥ 60 years old were 4.2%, 11.4%, 19.2%, 23.1% and 5.3%, respectively.</p><p><b>CONCLUSION</b>Compared with the previous data, the incidence of ALL is increased in Shanghai, and has a geographic distribution characteristic.</p>
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Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , China , Epidemiología , Recolección de Datos , Incidencia , Leucemia-Linfoma Linfoblástico de Células Precursoras , EpidemiologíaRESUMEN
<p><b>OBJECTIVE</b>To analyse the epidemiological data of acute lymphoblastic leukemia (ALL) in Shanghai.</p><p><b>METHODS</b>ALL cases in Shanghai from 2002 to 2006 were retrospectively investigated.</p><p><b>RESULTS</b>All together there were 544 newly diagnozed ALL cases. The yearly incidence of ALL was 0.81/10(5), which was slightly higher in men (0.86/10(5)) than in women (0.75/10(5)). The age-stratified incidence showed 2.31/10(5) in patients (pts) </= 17y, 0.54/10(5) in 18 - 34 y, 0.46/10(5) in 35 - 59 y, and 0.94/10(5) in pts > 60 y. The incidences in Chongming County was 0.60/10(5), being the lowest in all districts. The morphological types of ALL was L(1) (26.2%), L(2) (57.4%) and L(3) (16.4%); the immunophenotype was B (80.1%) and T (19.5%). The incidence of ALL with myeloid antigen expression was 20.2%. Genetic examination revealed that chromosome aberration of t(9;22) was the most common one.</p><p><b>CONCLUSIONS</b>The incidence of ALL in Shanghai is 0.81/10(5). Compared with the national standard (1986 - 1998), the incidence in adolescents is obviously increased. Chongming County has the lowest incidence, indicating a role of environment factor in ALL incidence.</p>
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Humanos , China , Aberraciones Cromosómicas , Inmunofenotipificación , Leucemia-Linfoma Linfoblástico de Células Precursoras , Genética , Encuestas y CuestionariosRESUMEN
Objective To investigate prevalence of erectile dysfunction(ED)in type 2 diabetic male patients and to analyze its related factors.Methods A total of 904 married male patients with type 2 diabetes were involved in this study and they were interviewed with anonymous questionnaire.An international index of erectile function-5(IIEF-5)was used to determine the severity of ED by self-rating score in age,duration of diabetes,serum level of glycosylated hemoglobin A1c(HbA1c),history of alcohol drinking and smoking,blood pressure and anti-hypertensive medication in diabetic ED patients.Relationship between relevant factors and diabetic ED was analyzed.Results ED was diagnosed in 612 diabetics with prevalence of 67.7 percent(612/904)according to their total scores of IIEF-5.Logistic regression analysis showed that increase in duration of diabetes by five years,age by 10 years,serum level of HbAI c by 2%, systolic blood pressure by 30 mm Hg(1 mm Hg = 0.133 kPa),positive history of smoking and alcohol drinking were all independently associated with prevalence of diabetic ED,with OR of 1.96,1.25,2.32, 1.12,1.67(P