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Wang J.,Shanghai JiaoTong University | Luan Z.,Naval General Hospital of PLA | Jiang H.,Guangzhou Women and Childrens Medical Center | Fang J.,Sun Yat Sen University | And 3 more authors.
Biology of Blood and Marrow Transplantation | Year: 2016

We investigated the efficacy of allogeneic hematopoietic stem cell transplantation (alloHSCT) in pediatric patients with mucopolysaccharidosis (MPS). A retrospective analysis of transplantation data from 34 cases of MPS from the China Children Transplant Group, treated between December 2004 and September 2015, was conducted. Among the 34 cases, 12 cases were type I, 12 were type II, 4 were type IV, 4 were type VI, and 2 were of an unknown type. The median age at transplantation was 3.75 years (range, 1 to 7 years); the median follow-up time was 14 months (range, 2 to 119 months). Eleven patients underwent unrelated cord blood transplantation and 23 underwent peripheral blood stem cell transplantation (4 cases with an HLA-matched sibling donor, 2 cases with an HLA-mismatched related donor, and 17 cases with an unrelated donor). A busulfan-based myeloablative regimen was used as a conditioning regimen. The estimated overall survival at 3 years was 84.8% ± 6.3% and 91.2% of the patients (31 of 34) achieved full donor chimerism. Twenty-seven children were evaluable and all but 1 (carrier sibling donor; enzyme level improved but failed to reach normal) achieved normal enzyme level after transplantation. The incidence of grades II to IV acute graft-versus-host disease (aGVHD) was 41.1% (14 of 34), wherein the incidence of grades III and IV aGVHD was 11.8% (4 of 34). The incidence of moderate-to-severe chronic graft-versus-host disease was 5.9% (2 of 34). There was a significant difference in the survival rate between children who received transplantation before 2009 and those after 2009 (55.6% versus 95.7%, P = .002); the survival rate was lower in patients with pneumonia before transplantation than in those with no active infection before transplantation (66.7% versus 95.5%, P = .019), and no significant differences in survival rates were observed among children with different disease types, ages at transplantation, donor/graft source, and conditioning regimens. After transplantation, upper-airway obstruction, hepatosplenomegaly, and corneal clouding were significantly improved; hearing and motor function were improved to a certain extent; valvular heart disease was improved in some patients but progressed in others; and short stature and speech skills showed little improvement. AlloHSCT may save the lives of patients with MPS I, II, IV or VI and could improve quality of life. Pretransplantation pneumonia affects transplantation outcomes. Advances in transplantation protocols and techniques help to improve patient prognosis. Well-matched unrelated donors can also be an ideal donor source. Standardized follow-up and a multidisciplinary team contribute to accurate evaluation of long-term post-transplantation outcome and further improve the quality of life of MPS patients. © 2016 The American Society for Blood and Marrow Transplantation


PubMed | Shanghai JiaoTong University, The Prince of Wales Hospital, Naval General Hospital of PLA, Sun Yat Sen University and 2 more.
Type: Journal Article | Journal: Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation | Year: 2016

We investigated the efficacy of allogeneic hematopoietic stem cell transplantation (alloHSCT) in pediatric patients with mucopolysaccharidosis (MPS). A retrospective analysis of transplantation data from 34 cases of MPS from the China Children Transplant Group, treated between December 2004 and September 2015, was conducted. Among the 34 cases, 12 cases were type I, 12 were type II, 4 were type IV, 4 were type VI, and 2 were of an unknown type. The median age at transplantation was 3.75 years (range, 1 to 7 years); the median follow-up time was 14 months (range, 2 to 119 months). Eleven patients underwent unrelated cord blood transplantation and 23 underwent peripheral blood stem cell transplantation (4 cases with an HLA-matched sibling donor, 2 cases with an HLA-mismatched related donor, and 17 cases with an unrelated donor). A busulfan-based myeloablative regimen was used as a conditioning regimen. The estimated overall survival at 3 years was 84.8%6.3% and 91.2% of the patients (31 of 34) achieved full donor chimerism. Twenty-seven children were evaluable and all but 1 (carrier sibling donor; enzyme level improved but failed to reach normal) achieved normal enzyme level after transplantation. The incidence of grades II to IV acute graft-versus-host disease (aGVHD) was 41.1% (14 of 34), wherein the incidence of grades III and IV aGVHD was 11.8% (4 of 34). The incidence of moderate-to-severe chronic graft-versus-host disease was 5.9% (2 of 34). There was a significant difference in the survival rate between children who received transplantation before 2009 and those after 2009 (55.6% versus 95.7%, P=.002); the survival rate was lower in patients with pneumonia before transplantation than in those with no active infection before transplantation (66.7% versus 95.5%, P=.019), and no significant differences in survival rates were observed among children with different disease types, ages at transplantation, donor/graft source, and conditioning regimens. After transplantation, upper-airway obstruction, hepatosplenomegaly, and corneal clouding were significantly improved; hearing and motor function were improved to a certain extent; valvular heart disease was improved in some patients but progressed in others; and short stature and speech skills showed little improvement. AlloHSCT may save the lives of patients with MPS I, II, IV or VI and could improve quality of life. Pretransplantation pneumonia affects transplantation outcomes. Advances in transplantation protocols and techniques help to improve patient prognosis. Well-matched unrelated donors can also be an ideal donor source. Standardized follow-up and a multidisciplinary team contribute to accurate evaluation of long-term post-transplantation outcome and further improve the quality of life of MPS patients.


PubMed | National University of Defense Technology, Naval General Hospital of PLA and Southern Medical University
Type: Journal Article | Journal: Journal of dentistry | Year: 2014

Currently used fibre-reinforced composite (FRC) intracanal posts possess low flexural strength which usually causes post fracture when restoring teeth with extensive loss. To improve the flexural strength of FRC, we aimed to apply a high-performance fibre, poly p-phenylene-2, 6-benzobisoxazole (PBO), to FRCs to develop a new intracanal post material.To improve the interfacial adhesion strength, the PBO fibre was treated with coupling agent (Z-6040), argon plasma, or a combination of above two methods. The effects of the surface modifications on PBO fibre were characterised by determining the single fibre tensile strength and interfacial shear strength (IFSS). The mechanical properties of PBO FRCs were characterised by flexural strength and flexural modulus. The cytotoxicity of PBO FRC was evaluated by the MTT assay.Fibres treated with a combination of Z-6040 and argon plasma possessed a significantly higher IFSS than untreated fibres. Fibre treated with the combination of Z-6040-argon-plasma FRC had the best flexural strength (531.51 26.43MPa) among all treated fibre FRCs and had sufficient flexural strength and appropriate flexural moduli to be used as intracanal post material. Furthermore, an in vitro cytotoxicity assay confirmed that PBO FRCs possessed an acceptable level of cytotoxicity.In summary, our study verified the feasibility of using PBO FRC composites as new intracanal post material. Although the mechanical property of PBO FRC still has room for improvement, our study provides a new avenue for intracanal post material development in the future.To our knowledge, this is the first study to verify the feasibility of using PBO FRC composites as new intracanal post material. Our study provided a new option for intracanal post material development.


PubMed | Chinese Academy of Sciences, Naval General Hospital of PLA, The General Hospital of the Chinese Peoples Armed Police Force and University of Sichuan
Type: Journal Article | Journal: Oncology reports | Year: 2013

The therapeutic potential of membrane complement regulatory protein (mCRP)-neutralizing antibodies is unsatisfactory, which perhaps lies in the complex role of mCRPs in tumor occurrence and development. As a member of the mCRPs, CD46 is a transmembrane protein with a cytoplasmic domain and is implicated more in the control of the alternative complement pathway than of the classical complement pathway. Growing evidence has revealed that both the CD46 signaling pathway and microRNAs (miRNAs) play an important role in the development and progression of hepatocellular carcinoma (HCC). In the present study, we analyzed mCRP expression in different tumor tissues by employing western blotting and qPCR. To address the potential role of miRNAs in CD46 signaling, we set out to profile miRNA expression in CD46-overexpressed and -silenced HepG2 cell lines. Furthermore, bioinformatic analysis was performed to identify downstream targets of CD46 signaling. We found that the levels of CD46 expression in HCC tissues were significantly higher compared to that in the adjacent normal tissues. After complement-related gene expression profiling and unsupervised hierarchical clustering analysis of 10 HCC tissues, a total of 37 miRNAs showed significantly different expression levels before and after CD46 expression change. By bioinformatic analysis, we identified let-7b and miR-17 as downstream targets of CD46 signaling, and that the expression levels of let-7b and miR-17 were negatively correlated with that of CD46 in HepG2 cells. The present study suggests that CD46 plays an important role in HCC carcinogenesis by regulating let-7b and miR-17.


Lu Z.,Naval General Hospital of PLA | Song Q.,General Hospital of Chinese Peoples Armed Police Force | Yang J.,University of Sichuan | Zhao X.,Naval General Hospital of PLA | And 3 more authors.
Cellular Physiology and Biochemistry | Year: 2014

Background: Periplocin is used for treatment of rheumatoid arthritis, reinforcement of bones and tendons, palpitations or shortness of breath and lower extremity edema in traditional medicine. Our previous findings suggested that periplocin could inhibit the growth of lung cancer both in vitro and in vivo. But the biological processes and molecular pathways by which periplocin induces these beneficial effects remain largely undefined. Methods: To explore the molecular mechanisms of periplocin involved in anti-cancer activity, in the present study the protein profile changes of human lung cancer cell lines A549 in response to periplocin treatment were investigated using the proteomics approaches (2-DE combined with MS/MS). Western blot was employed to verify the changed proteins. Interactions between changed proteins were analyzed by STRING. Results: 29 down-regulated protein species named GTP-binding nuclear protein Ran (RAN), Rho GDP-dissociation inhibitor 1 (ARHGDIA), eukaryotic translation initiation factor 5A-1 (EIF5A) and Profilin-1(PFN1), and 10 up-regulated protein species named Heat shock cognate 71 kDa protein (HSPA8),10 kDa heat shock protein (HSPE1), and Cofilin-1(CFL-1) were identified. Among them, GTP-binding nuclear protein Ran (RAN) and Rho GDP-dissociation inhibitor 1 (ARHGDIA) were the most significantly changed (over tenfold). The proteasome subunit beta type-6 (PSMB6), ATP synthase ecto-α-subunit (ATP5A1), Aldehyde dehydrogenase 1 (ALDH1) and EIF5A were verified by immunoblot assays to be dramatically down-regulated. By STRING bioinformatics analysis revealing interactions and signaling networks it became apparent that the proteins changed they are primarily involved in transcription and proteolysis. Conclusion: Periplocin inhibited growth of lung cancer by down-regulating proteins, such as ATP5A1, EIF5A, ALDH1 and PSMB6. These findings may improve our understanding of the molecular mechanisms underlying the anti-cancer effects of periplocin on lung cancer cells. © 2014 S. Karger AG, Basel.


Wang F.,Naval General Hospital of PLA | Lu H.-X.,Naval General Hospital of PLA | Lin S.-S.,Naval General Hospital of PLA
Chinese Journal of Tissue Engineering Research | Year: 2013

BACKGROUND: Rapid expansion of the dental arch is an effective way to rapidly expanse the jaw. Compared with rapid expansion, the slow expansion has higher stability and less recurrence, but the reports on the long-term stability of quad helix expansion are rare. OBJECTIVE: To retrospectively analyze the clinical effect of quad helix expansion in orthodontics. METHODS: Twenty-two subjects with dental arch stenosis in mixed dentition and early permanent dentition who experienced an expansion of at least 3 mm with quad helix appliance were selected for this study. Plaster dental casts and posteroanterior radiographs were evaluated at the beginning of the treatment (T1), at the completion of phase I quad helix expansion or full treatment (T2), and approximately 2 years following the completion of treatment (T3). The distance between two first molars was measured on the model. J point was drawn on the posteroanterior radiograph in order to measure the distance between the bilateral base bones and the molar inclination, as well as to evaluate the corrective and orthopedic effects of dental arch expansion. RESULTS AND CONCLUSION: Compared with that before expansion, the first permanent molar inclination and the distance between base bones on two sides were significant increased after quad helix expansion; there were no significant differences in the distance between two first permanent molars, first permanent molar inclination and the distance between bilateral base bones on two sides when compared after quad helix expansion and after 2-year follow-up (P > 0.05). The results indicate that the long-term effect of quad helix expansion is stable with orthopedic effect.


Zhang Z.-Y.,Naval General Hospital of PLA | Zhou C.-H.,Naval General Hospital of PLA | Li M.-X.,Naval General Hospital of PLA | Yu Y.-W.,Naval General Hospital of PLA
Experimental and Therapeutic Medicine | Year: 2012

The objective of the present study was to investigate the long-term clinical efficacy of intermittent peritoneal dialysis (IPD) using various doses and to explore the most suitable dialysis dose and practice pattern for patients. A total of 52 inpatients/outpatients who had undergone IPD for more than 5 years were recruited and divided into three groups according to the dialysis dose: 4liters in Group A, 6 liters in GroupB and 8 liters in GroupC. The dwell time was 4h. All patients were fasted overnight. The dialysis adequacy, nutritional status, complication control, blood pressure and intra-abdominal infection were determined and observed among these patients. Barthel index (BI) and Hamilton Depression Scale (HAMD) were employed to measure the activities of daily living (ADL) and degree of depression, respectively. The dialysis adequacy and ultrafiltration volume in Group A were lower than those in Groups B and C, but the residual urine volume was larger than that in the latter two groups. In addition, there was a marked difference in the control of complications between GroupA and Groups B and C. When compared to GroupsA and B, the nutritional status in Group C was significantly decreased, the mean arterial pressure and intra-abdominal infection rates were dramatically increased, and the HAMD scores were also higher (P<0.05). No significant difference was noted in the BI. For patients undergoing long-term IPD, individualized dialysis dose may benefit the dialysis adequacy, nutritional status, control of complications, blood pressure, rate of intraabdominal infection, ADL and depression.


Rui M.,Naval General Hospital of PLA | Duan Y.-Y.,Naval General Hospital of PLA | Zhang X.-H.,Naval General Hospital of PLA | Wang H.-L.,Naval General Hospital of PLA | Wang D.-P.,Naval General Hospital of PLA
Respiration | Year: 2012

Background: Few data are available on the role of neutrophil elastase (NE) and nuclear factor-κB (NF-κB) in the course of seawater drowning-induced acute lung injury (SWD-ALI), and there is no evidence on the value of giving urinary trypsin inhibitor (UTI) in the case of SWD-ALI. Objective: To investigate the role of NF-κB and NE in the pathogenesis of SWD-ALI and whether UTI treatment can attenuate SWD-ALI in rabbits. Methods: Rabbits were randomly assigned to control, seawater drowning, and UTI treatment groups. The rabbits in the control group only suffered from intubation, whereas the rabbits in the seawater drowning group and the UTI treatment group received arterial injection of normal saline without/with 50,000 U/kg body weight of UTI after instillation of seawater into an endotracheal catheter. The activities or contents of NF-κB, MPO, NE, TNF-α, and IL-10 in lung tissue were measured by nonradioactive EMSA, biochemical methods, and ELISA, respectively. Results: After the seawater challenge, all of the rabbits demonstrated immediate drops in arterial PaO 2/FiO 2 and pronounced pulmonary edema and inflammatory cell infiltration with evidence of an increase in the ratio of wet weight to dry weight, lung permeability index, lung injury scores, and the activities or contents of NF-κB, NE, MPO, TNF-α, and IL-10. UTI treatment markedly attenuated lung histopathological changes with evidence of a decrease in all of the parameters, except for upregulation of IL-10. Arterial PaO 2/FiO 2 was significantly improved after 6 h of UTI treatment. Conclusion: These results suggest that NF-κB and NE play an important role in SWD-ALI. UTI protects against SWD-ALI, at least partly, through inhibition of the enhanced local activity of NF-κB, contents of TNF-α and NE, and infiltration of neutrophils and promotion of the level of IL-10. Copyright © 2011 S. Karger AG.


PubMed | Naval General Hospital of PLA
Type: Comparative Study | Journal: Respiration; international review of thoracic diseases | Year: 2012

Few data are available on the role of neutrophil elastase (NE) and nuclear factor-B (NF-B) in the course of seawater drowning-induced acute lung injury (SWD-ALI), and there is no evidence on the value of giving urinary trypsin inhibitor (UTI) in the case of SWD-ALI.To investigate the role of NF-B and NE in the pathogenesis of SWD-ALI and whether UTI treatment can attenuate SWD-ALI in rabbits.Rabbits were randomly assigned to control, seawater drowning, and UTI treatment groups. The rabbits in the control group only suffered from intubation, whereas the rabbits in the seawater drowning group and the UTI treatment group received arterial injection of normal saline without/with 50,000 U/kg body weight of UTI after instillation of seawater into an endotracheal catheter. The activities or contents of NF-B, MPO, NE, TNF-, and IL-10 in lung tissue were measured by nonradioactive EMSA, biochemical methods, and ELISA, respectively.After the seawater challenge, all of the rabbits demonstrated immediate drops in arterial PaO(2)/FiO(2) and pronounced pulmonary edema and inflammatory cell infiltration with evidence of an increase in the ratio of wet weight to dry weight, lung permeability index, lung injury scores, and the activities or contents of NF-B, NE, MPO, TNF-, and IL-10. UTI treatment markedly attenuated lung histopathological changes with evidence of a decrease in all of the parameters, except for upregulation of IL-10. Arterial PaO(2)/FiO(2) was significantly improved after 6 h of UTI treatment.These results suggest that NF-B and NE play an important role in SWD-ALI. UTI protects against SWD-ALI, at least partly, through inhibition of the enhanced local activity of NF-B, contents of TNF- and NE, and infiltration of neutrophils and promotion of the level of IL-10.


PubMed | Naval General Hospital of PLA
Type: Journal Article | Journal: Experimental and therapeutic medicine | Year: 2012

The objective of the present study was to investigate the long-term clinical efficacy of intermittent peritoneal dialysis (IPD) using various doses and to explore the most suitable dialysis dose and practice pattern for patients. A total of 52 inpatients/outpatients who had undergone IPD for more than 5 years were recruited and divided into three groups according to the dialysis dose: 4 liters in Group A, 6 liters in Group B and 8 liters in Group C. The dwell time was 4 h. All patients were fasted overnight. The dialysis adequacy, nutritional status, complication control, blood pressure and intra-abdominal infection were determined and observed among these patients. Barthel index (BI) and Hamilton Depression Scale (HAMD) were employed to measure the activities of daily living (ADL) and degree of depression, respectively. The dialysis adequacy and ultrafiltration volume in Group A were lower than those in Groups B and C, but the residual urine volume was larger than that in the latter two groups. In addition, there was a marked difference in the control of complications between Group A and Groups B and C. When compared to Groups A and B, the nutritional status in Group C was significantly decreased, the mean arterial pressure and intra-abdominal infection rates were dramatically increased, and the HAMD scores were also higher (P<0.05). No significant difference was noted in the BI. For patients undergoing long-term IPD, individualized dialysis dose may benefit the dialysis adequacy, nutritional status, control of complications, blood pressure, rate of intra-abdominal infection, ADL and depression.

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