Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.

Objective:To analyze the clinical features and prognosis of acute B lymphoblastic leukemia (B-ALL) children carrying a TCF3: : PBX1 fusion gene and to evaluate the prognostic value of this gene.Methods:Retrospective cohort study.A total of 2 164 B-ALL children aged 0-18 years diagnosed and treated at 19 pediatric centers from October 2016 to June 2022 were enrolled.They were divided into the positive group and the negative group according to whether they carried a TCF3: : PBX1 fusion gene.The clinical characteristics, treatment response, adverse reactions, and prognosis of the 2 groups of patients were analyzed.The rank sum and Kruskal-Wallis tests were used to compare two and more than two groups of numerical variables, respectively.Fisher′s exact test was used to compare categorical variables.Results:Among the 2 164 patients, 116 (5.4%) were TCF3: : PBX1 positive, of which 70 patients were female, accounting for 60.3%.There were 840 female patients in the TCF3: : PBX1-negative group, accounting for 41.0%.There was a significant difference in the ratio of females between the TCF3: : PBX1-positive and TCF3: : PBX1-negative groups ( P<0.001).No significant difference was observed in age of onset between the two groups( P>0.05).The proportion of bone marrow naive cells [54.00 (14.00, 76.50)% vs.29.00 (3.00, 68.00)%], white blood cell counts [25.30 (10.46, 60.94)×10 9/L vs.9.03 (4.38, 30.73)×10 9/L] and hemoglobin counts [82.00(63.00, 101.00) g/L vs.74.00(60.00, 90.00) g/L] in the TCF3: : PBX1-positive group were significantly higher than those in the negative group at the onset (all P<0.05).In terms of treatment response, the proportion of peripheral blood naive cells on Day 8 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group [2.00 (0, 9.00)% vs.0 (0, 2.00)%, P<0.001].The proportion of minimal residual disease <0.1% on Day 15 in the TCF3: : PBX1-positive group was significantly higher than that in the negative group ( P=0.038).There were no significant differences in cumulative recurrence rate, treatment-related mortality (TRM), and overall survival (OS) between the TCF3: : PBX1-positive group and TCF3: : PBX1-negative group (all P>0.05).The cumulative recurrence risk of TCF3: : PBX1-positive patients was 9.646 times higher than that of ETV6: : RUNX1-positive patients with better prognosis( HR=9.646, 95% CI: 1.026-90.700, P=0.047).There were no significant differences in TRM and OS between TCF3: : PBX1-positive and ETV6: : RUNX1-positive patients (all P>0.05).A significant enrichment of PAX5 mutations was detected in TCF3: : PBX1-positive patients.Among the 7 high-risk TCF3: : PBX1-positive patients in a single center, 4 patients had PAX5 mutations, and this proportion was significantly higher than that in other patients ( P<0.001). Conclusions:B-ALL children carrying a TCF3: : PBX1 fusion gene have a high remission rate and good long-term prognosis after intensive chemotherapy.It is suggesting that TCF3: : PBX1-positive B-ALL patients should be rated at intermediate risk to receive intensive chemotherapy.

Parkinson′s disease (PD) is a common neurodegenerative disease. Its main pathological feature is the progressive loss of substantia nigra and other catecholaminergic neurons containing neuromelanin (NM). Therefore, NM may be closely related to the pathogenesis of PD. The content of NM can be detected by NM magnetic sensitive sequence imaging, and then it can be applied to the study of the neuropathological mechanism of NM and PD. This paper focuses on the physiological significance of NM, its role in the pathogenesis of PD and the prospect of NM as a biomarker to assist diagnosis and disease monitoring of PD.

OBJECTIVE: To analyze the prognosis of fetuses with cystic hygroma (CH) or nuchal translucency (NT) or nuchal fold (NF) thickening detected by prenatal echography. METHODS: From January 2014 to December 2015, 124 fetuses with CH and NT/NF thickening on prenatal echography were enrolled from Women's Hospital of Zhejiang University School of Medicine. The basic clinical information, ultrasonic results, pregnancy outcomes and newborn follow-ups were analyzed. The cases were grouped by prognosis and the factors affecting prognosis were analyzed with logistic regression. RESULTS: There were 85 cases of labor induction including one stillbirth and 39 cases delivered. Except one infant who died after birth, all live births survived with good prognosis. Univariate analysis showed that the gestational age at diagnosis of poor prognosis group was earlier than that of good prognosis group (<0.01); and the former group also had higher hydrops fetalis rate and additional structural anomalies rate (all <0.01). Multivariate regression analysis showed that hydrops fetalis (=90.105, <0.05) and additional structural anomalies (=61.854, <0.05) were risk factors of poor prognosis in fetuses with CH and NT/NF thickening. CONCLUSIONS Fetuses with diagnosed CH or NT/NF thickening on prenatal ultrasonography are likely to be associated with chromosomal abnormality. Early gestational weeks, hydrops fetalis and additional structural anomalies may indicate poor prognosis.
Female ; Fetus ; Humans ; Hydrops Fetalis ; etiology ; Infant, Newborn ; Lymphangioma, Cystic ; complications ; diagnosis ; Nuchal Translucency Measurement ; Pregnancy ; Pregnancy Outcome ; Prognosis ; Ultrasonography, Prenatal

Spinocerebellar ataxia type 2 (SCA2) is an autosomal dominant degenerative disease. The main clinical manifestation of SCA2 is progressive cerebellar syndrome, but a wide range of extracerebellar and non-motor symptoms can be observed clinically. The non-motor symptoms and extra-cerebellar signs in SCA2 patients are reviewed to provide a better understanding in cognition, diagnosis and treatment of the disease.

Objective: We planned to evaluate the effectiveness of moving epidemic method (MEM) in calculating influenza epidemic threshold of 7 climatic zones in China mainland. Methods: The positive rate of influenza virus was obtained from the National Influenza Surveillance Network System from 2010/2011 to 2017/2018. We divided the 31 provinces into 7 climatic zones according to previous literatures and applied MEM to calculate the influenza epidemic threshold of 2018/2019 influenza season for these climatic zones. Sensitivity, specificity, positive predictive value and negative predictive value were calculated to evaluate the effectiveness of MEM. Results: Pre-epidemic threshold (the positive rate of influenza virus) varied from 9.66% (temperate zone) to 16.36% (subtropical zone) for 2018/2019 influenza season. The gap between pre-epidemic and post-epidemic thresholds was less than 5% except for plateau zone. The sensitivity was 86.16% (95CI:66.81%-98.23%), the specificity was 94.92% (95CI: 91.13%-98.41%), the positive predictive value was 89.87% (95%CI: 84.39%-94.38%), the negative predictive value was 92.96% (95%CI: 84.46%-99.17%). Conclusion Overall, moving epidemic Method performs well in calculating influenza epidemic threshold in China, much better than the previous study.

The annual deaths associated with influenza is estimated to be between 290 000 and 650 000,which caused substantial burden to the society.Children have the highest incidence of influenza among all age groups,which can cause overloaded medical visits and a significant increase of hospitalization risk.The severe economic burden includes not only the direct medical costs due to outpatients and hospitalization,but also the indirect burden of school absence of children and work absence of their family members.Annual vaccination is the best measure to prevent influenza,however,influenza vaccination coverage among children in China is very low,and influenza vaccination has not yet been included in the National Immunization Program.Now,the disease burden of influenza and the vaccine usage in children in China were reviewed,and in order to provide evidence for influenza control and prevention.

Embryo is regarded as a semi-allograft for carrying paternal genetic information. It can escape the attack from maternal immune system and successfully implant into the uterus, which mainly relies on the establishment of immune tolerance at the maternal-fetal interface. The maternal-fetal interface is the basis for the connection and material exchange between the mother and fetus. The mechanisms of immune re-sponses at this interface are the key to the maintenance of normal pregnancy. Immunomodulatory molecules expressed at the maternal-fetal interface are vital for immune tolerance. Studies have shown that sialicacid-binding immunoglobulin-like lectins (Siglecs) are abundantly expressed at the maternal-fetal interface and play an important role in immune regulation. Siglecs are important members of the typeⅠimmunoglobulin-like superfamily. By binding with the sialic acid residues on the side chains of glycoproteins or glycolipids, Siglecs involve in immune regulation, the activation and proliferation of immune cells and immune cell-medi-ated physiological and pathological processes. Present research on the expression of Siglecs in the maternal-fetal interface is mainly focused on Siglec-6 and Siglec-10, while other Siglecs are less studied. Siglecs, such as Siglec-6 and Siglec-10, might involve in the regulation of immune tolerance at the maternal-fetal in-terface through binding to different ligands. This article briefly reviewed the expression of Siglecs and their ligands at the maternal-fetal interface and their roles in immune tolerance.

Objective We planned to evaluate the effectiveness of moving epidemic method (MEM) in calculating influenza epidemic threshold of 7 climatic zones in China mainland. Methods The positive rate of influenza virus was obtained from the National Influenza Surveillance Network System from 2010/2011 to 2017/2018. We divided the 31 provinces into 7 climatic zones according to previous literatures and applied MEM to calculate the influenza epidemic threshold of 2018/2019 influenza season for these climatic zones. Sensitivity, specificity, positive predictive value and negative predictive value were calculated to evaluate the effectiveness of MEM. Results Pre?epidemic threshold (the positive rate of influenza virus) varied from 9.66% (temperate zone) to 16.36% (subtropical zone) for 2018/2019 influenza season. The gap between pre?epidemic and post?epidemic thresholds was less than 5% except for plateau zone. The sensitivity was 86.16% (95CI: 66.81% - 98.23%), the specificity was 94.92% (95CI:91.13%-98.41%), the positive predictive value was 89.87% (95% CI : 84.39%-94.38%), the negative predictive value was 92.96% (95%CI: 84.46%-99.17%). Conclusion Overall, moving epidemic Method performs well in calculating influenza epidemic threshold in China, much better than the previous study.

Objective We planned to evaluate the effectiveness of moving epidemic method (MEM) in calculating influenza epidemic threshold of 7 climatic zones in China mainland. Methods The positive rate of influenza virus was obtained from the National Influenza Surveillance Network System from 2010/2011 to 2017/2018. We divided the 31 provinces into 7 climatic zones according to previous literatures and applied MEM to calculate the influenza epidemic threshold of 2018/2019 influenza season for these climatic zones. Sensitivity, specificity, positive predictive value and negative predictive value were calculated to evaluate the effectiveness of MEM. Results Pre?epidemic threshold (the positive rate of influenza virus) varied from 9.66% (temperate zone) to 16.36% (subtropical zone) for 2018/2019 influenza season. The gap between pre?epidemic and post?epidemic thresholds was less than 5% except for plateau zone. The sensitivity was 86.16% (95CI: 66.81% - 98.23%), the specificity was 94.92% (95CI:91.13%-98.41%), the positive predictive value was 89.87% (95% CI : 84.39%-94.38%), the negative predictive value was 92.96% (95%CI: 84.46%-99.17%). Conclusion Overall, moving epidemic Method performs well in calculating influenza epidemic threshold in China, much better than the previous study.