Objective:To evaluate the efficacy and safety of cord blood stem cell transplantation (CBSCT) in pediatric recipients with mucopolysaccharidosis type Ⅱ (MPS Ⅱ, Hunter syndrome).Methods:Clinical data of five male children with MPS Ⅱ who underwent CBSCT at the Department of Hematology, Children's Hospital of Soochow University between March 2018 and July 2023 were retrospectively analyzed. Post-transplantation clinical outcomes and enzymatic activity were observed. Literature was searched in the China National Knowledge Infrastructure (CNKI), Wanfang, and PubMed databases using the keywords "mucopolysaccharidosis type Ⅱ" "MPS Ⅱ" "IDS gene" and "Hunter syndrome" in both English and Chinese. Articles describing clinical manifestations, genetic diagnosis, and hematopoietic stem cell transplantation (HSCT) in MPS II were screened.Results:All five patients were male, with a median age at diagnosis of 4.3(2.5-5.5) years and a median age at transplantation of 4.6(2.8-6.5) years. At diagnosis, all exhibited coarse facial features, hepatosplenomegaly, skeletal deformities or abnormalities, abnormal head MRI findings, and Mongolian spots; four had joint stiffness, three had valvular heart disease, and two had airway obstruction, short stature, and intellectual disability. Three recipients received single-unit cord blood, and two received double-unit cord blood. Myeloablative conditioning regimens consisted of busulfan, cyclophosphamide, anti-thymocyte globulin ± fludarabine. The median neutrophil engraftment and platelet engraftment times were 19(14-21) days and 26(15-44) days, respectively. Complete donor chimerism was achieved at 1 month post-transplantation. Complications included peri-engraftment syndrome in 5 cases, acute graft-versus-host disease (GVHD) in 2 cases (1 with grade Ⅳ skin and grade Ⅱ intestinal involvement; 1 with grade Ⅱ skin involvement), limited chronic GVHD in 1 case (moderate intestinal involvement), cytomegalovirus (CMV) infection in 3 cases, Epstein-Barr virus (EBV) infection in 1 case, and capillary leak syndrome in 1 case; all were successfully managed. At the last follow-up in December 2023, all patients were alive, and enzyme activity had normalized by 3 months post-transplantation. Most clinical symptoms and signs improved; however, neurocognitive function showed no significant improvement, and some recipients exhibited progressive brain parenchymal changes on MRI. Literature review included 7 English and 5 Chinese studies, indicating that CBSCT and other HSCT modalities can improve multi-system clinical manifestations in MPS Ⅱ children, including restoration of enzyme activity, organ function improvement (such as liver and spleen shrinkage, adenoid reduction), enhanced motor function, and stabilization of neurocognitive function. Some studies suggest superior efficacy compared with enzyme replacement therapy, particularly in delaying disease progression and improving daily living abilities.Conclusion:CBSCT effectively restores enzymatic activity and improves multi-system manifestations in children with MPS Ⅱ, although its effect on neurological symptoms remains controversial. It is a safe and feasible therapeutic option for this condition.

Objective To sort out the clinical research evidence on the promotion of wound healing after anal fistula surgery by traditional Chinese medicine in the past decade,and understand the evidence distribution in this field,the evidence map system was used.Methods Computer retrieval of CNKI,VIP,Wanfang,PubMed,Web of Science,EMbase,The Cochrane Library and other databases were searched.Interventional studies,observational studies,systematic review/meta-analysis on the treatment of postoperative anal fistula wounds by traditional Chinese medicine in the past decade were included.The evidence was analyzed and presented in the form of text and graphics.And the quality of clinical RCT was evaluated using Cochrane systematic review manual.AMSTAR was used to evaluate the quality of meta-analysis,and the JBI quality evaluation tool was used to evaluate the methodological quality of retrospective studies.Results A total of 544 articles were included.In recent 10 years,the number of published documents showed an increasing trend.The intervention measures of TCM to promote wound healing after anal fistula operation included single intervention mode and combined intervention mode.The outcome indexes mainly included clinical efficacy,basic wound condition,TCM syndrome score,adverse reactions,quality of life,long-term efficacy and so on.The overall quality of clinical RCT,retrospective analysis and systematic review/meta-analysis is low.Most studies showed that Chinese medicine had advantages in the treatment of vertigo,and more high-quality studies were still lacking.Conclusion More large-sample,multi-center RCTS should be carried out in the future,and the quality of relevant systematic review/meta-analysis should be improved.The advantages of TCM in promoting wound-heal after anal fistula surgery can be fully explored,which provide strong support for the effectiveness and safety of traditional Chinese medicine in improving wound management.

Objective To sort out the clinical research evidence on the promotion of wound healing after anal fistula surgery by traditional Chinese medicine in the past decade,and understand the evidence distribution in this field,the evidence map system was used.Methods Computer retrieval of CNKI,VIP,Wanfang,PubMed,Web of Science,EMbase,The Cochrane Library and other databases were searched.Interventional studies,observational studies,systematic review/meta-analysis on the treatment of postoperative anal fistula wounds by traditional Chinese medicine in the past decade were included.The evidence was analyzed and presented in the form of text and graphics.And the quality of clinical RCT was evaluated using Cochrane systematic review manual.AMSTAR was used to evaluate the quality of meta-analysis,and the JBI quality evaluation tool was used to evaluate the methodological quality of retrospective studies.Results A total of 544 articles were included.In recent 10 years,the number of published documents showed an increasing trend.The intervention measures of TCM to promote wound healing after anal fistula operation included single intervention mode and combined intervention mode.The outcome indexes mainly included clinical efficacy,basic wound condition,TCM syndrome score,adverse reactions,quality of life,long-term efficacy and so on.The overall quality of clinical RCT,retrospective analysis and systematic review/meta-analysis is low.Most studies showed that Chinese medicine had advantages in the treatment of vertigo,and more high-quality studies were still lacking.Conclusion More large-sample,multi-center RCTS should be carried out in the future,and the quality of relevant systematic review/meta-analysis should be improved.The advantages of TCM in promoting wound-heal after anal fistula surgery can be fully explored,which provide strong support for the effectiveness and safety of traditional Chinese medicine in improving wound management.

Objective:To evaluate the efficacy and safety of cord blood stem cell transplantation (CBSCT) in pediatric recipients with mucopolysaccharidosis type Ⅱ (MPS Ⅱ, Hunter syndrome).Methods:Clinical data of five male children with MPS Ⅱ who underwent CBSCT at the Department of Hematology, Children's Hospital of Soochow University between March 2018 and July 2023 were retrospectively analyzed. Post-transplantation clinical outcomes and enzymatic activity were observed. Literature was searched in the China National Knowledge Infrastructure (CNKI), Wanfang, and PubMed databases using the keywords "mucopolysaccharidosis type Ⅱ" "MPS Ⅱ" "IDS gene" and "Hunter syndrome" in both English and Chinese. Articles describing clinical manifestations, genetic diagnosis, and hematopoietic stem cell transplantation (HSCT) in MPS II were screened.Results:All five patients were male, with a median age at diagnosis of 4.3(2.5-5.5) years and a median age at transplantation of 4.6(2.8-6.5) years. At diagnosis, all exhibited coarse facial features, hepatosplenomegaly, skeletal deformities or abnormalities, abnormal head MRI findings, and Mongolian spots; four had joint stiffness, three had valvular heart disease, and two had airway obstruction, short stature, and intellectual disability. Three recipients received single-unit cord blood, and two received double-unit cord blood. Myeloablative conditioning regimens consisted of busulfan, cyclophosphamide, anti-thymocyte globulin ± fludarabine. The median neutrophil engraftment and platelet engraftment times were 19(14-21) days and 26(15-44) days, respectively. Complete donor chimerism was achieved at 1 month post-transplantation. Complications included peri-engraftment syndrome in 5 cases, acute graft-versus-host disease (GVHD) in 2 cases (1 with grade Ⅳ skin and grade Ⅱ intestinal involvement; 1 with grade Ⅱ skin involvement), limited chronic GVHD in 1 case (moderate intestinal involvement), cytomegalovirus (CMV) infection in 3 cases, Epstein-Barr virus (EBV) infection in 1 case, and capillary leak syndrome in 1 case; all were successfully managed. At the last follow-up in December 2023, all patients were alive, and enzyme activity had normalized by 3 months post-transplantation. Most clinical symptoms and signs improved; however, neurocognitive function showed no significant improvement, and some recipients exhibited progressive brain parenchymal changes on MRI. Literature review included 7 English and 5 Chinese studies, indicating that CBSCT and other HSCT modalities can improve multi-system clinical manifestations in MPS Ⅱ children, including restoration of enzyme activity, organ function improvement (such as liver and spleen shrinkage, adenoid reduction), enhanced motor function, and stabilization of neurocognitive function. Some studies suggest superior efficacy compared with enzyme replacement therapy, particularly in delaying disease progression and improving daily living abilities.Conclusion:CBSCT effectively restores enzymatic activity and improves multi-system manifestations in children with MPS Ⅱ, although its effect on neurological symptoms remains controversial. It is a safe and feasible therapeutic option for this condition.

To study the molecular mechanism for DNA hypomethylation of STAT3 promoter in CD4+ T cells from acute graft-versus-host disease (aGVHD) patients.
 Methods: We collected CD4+ T cells from peripheral blood of 42 patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) from HLA-identical sibling donors. GADD45A expression level in CD4+ T cells was measured by real-time PCR and Western blot. The binding level between HMGB1 and GADD45A in CD4+ T cells was analyzed by co-immunoprecipitation, while the binding levels of HMGB1/GADD45A with STAT3 promoter were detected by chromatin immunoprecipitation-quantitative real-time PCR (ChIP-qPCR). After overexpression of HMGB1 and knockdown of GADD45A in normal CD4+ T cells, STAT3 expression and DNA methylation were measured by Western blot and bisulfite sequencing PCR, respectively.
 Results: GADD45A expression was significantly up-regulated in patients with aGVHD compared with that in the patients without aGVHD. More HMGB1-GADD45A complexes were found in CD4+ T cells from patients with aGVHD compared with that in patients without aGVHD. The bindings of HMGB1/GADD45A with STAT3 promoter were significantly increased, and the binding levels of HMGB1/GADD45A were negatively correlated with STAT3 promoter DNA methylation. The expression of STAT3 was significantly reduced and the DNA methylation of STAT3 promoter was significantly increased in CD4+ T cells with overexpression of HMGB1 and knockdown of GADD45A compared with CD4+ T cells only with overexpression of HMGB1.
 Conclusion: The increased expression of HMGB1/GADD45A plays an importent role in STAT3 promoter DNA hypomethylation, thereby promoting STAT3 expression in CD4+ T cells from aGVHD patients.
CD4-Positive T-Lymphocytes ; Cell Cycle Proteins ; metabolism ; DNA Demethylation ; Gene Expression Regulation ; genetics ; Graft vs Host Disease ; genetics ; HMGB1 Protein ; metabolism ; Hematopoietic Stem Cell Transplantation ; Humans ; Nuclear Proteins ; metabolism ; Promoter Regions, Genetic ; genetics ; STAT3 Transcription Factor ; genetics ; metabolism

To demonstrate the effect of AB serum on terminal erythroid differentiation ex vivo.
 Methods: After separation of CD34+ cells from cord blood, the cells were cultured and divided into a control group and an experimental group. The effects of AB serum were examined by the expressions of different markers (GPA, Band3 and α4-integrin) for erythroblast differentiation and enucleation by flow cytometry. 
 Results: The CD34+ cells were successfully differentiated to enucleated red blood cells. There were evident differences among the expressions of GPA, Band3 and α4-integrin between the 2 groups. The percentage of GPA positive cells in the experimental group was bigger than that in the control group in every time point. The expression of Band3 in the experimental group was higher than that in the control group. The expression of α4-integrin in the experimental group was lower than that in the control group. In addition, the enucleation rate in the experimental group was higher than that in the control group.
 Conclusion: AB serum can promote the cell differentiation and enucleation during terminal erythroid differentiation in vitro.
ABO Blood-Group System ; blood ; physiology ; Anion Exchange Protein 1, Erythrocyte ; metabolism ; Antigens, CD34 ; blood ; Cell Differentiation ; genetics ; physiology ; Cell Nucleus ; Cells, Cultured ; Erythrocytes ; physiology ; ultrastructure ; Erythropoiesis ; genetics ; physiology ; Fetal Blood ; cytology ; physiology ; Flow Cytometry ; Glycophorins ; metabolism ; Humans ; Integrin alpha4beta1 ; metabolism

OBJECTIVE: To investigate the effect of extracts with water and alcohol from Radix Trichosanthis on the cell survival and the expression of hepatitis B surface antigen (HBsAg) and hepatitis B e-antigen (HBeAg) in HepG2.2.15 cell supernatant. METHODS: The cell survival rate of HepG2.2.15 cells was detected by MTT assay. The HBsAg and HBeAg in HepG 2.2.15 cell supernatant were evaluated by enzyme linked immunosorbent assay. RESULTS: The water and alcohol soluble extracts from Radix Trichosanthis significantly inhibited the levels of HBsAg and HBeAg in HepG2.2.15 cells in a time-and-concentration-dependent manner. However, the therapeutic index of extracts with water from Radix Trichosanthis was better than that in the alcohol group. CONCLUSION The activity of water-soluble extract from Radix Trichosanthis is stronger on anti-hepatitis B virus than that of the alcohol-soluble extract.
Antiviral Agents ; pharmacology ; Drugs, Chinese Herbal ; classification ; pharmacology ; Hep G2 Cells ; Hepatitis B Surface Antigens ; biosynthesis ; Hepatitis B e Antigens ; biosynthesis ; Hepatitis B virus ; drug effects ; Humans