Objective To study the curative efficacy and safety of single-unit umbilical cord blood transplantation (sUCBT) for malignant hematologic diseases,which is provided by China's public cord blood bank.Methods We retrospectively analyzed 409 cases of malignant hematologic diseases who accepted myeloablative single-unit unrelated donor UCBT without ATG at our center between May 2008 and December 2016.A comparative analysis was made on the total nuclear cells (TNC) of the umbilical cord blood before freezing and after thawing,the cells of CD34+,the recovery rate of cells and the clinical effect of UCBT.Result 409 units of umbilical cord blood used in UCBT respectively came from eight China's public cord blood banks.The average TNC of 409 units of umbilical cord blood before freezing and after the tubular recovery were respectively 18.5 × 108 and 16.34 × 108 (p =0.000).The average recovery rate of the tubular recovery was 88.5％,and there was significant difference among cord blood banks (P =0.000).The average TNC of umbilical cord blood before freezing and transfusion were respectively 18.5 × 108 and 15.86 × 108 (p =0.000).The average recovery rate of umbilical cord blood transfusion was 85.9％,with the difference being significant among cord blood banks (P =0.000).The average number of CD34+ cells before freezing and after the tubular recovery was 11.18 × 106and 8.68 × 106 (p =0.000).The average recovery rate of CD34+ cells after the tubular recovery was 80.75 ％,with the difference being significant among the cord blood banks (P =0.000).At 42nd day after UCBT,the cumulative incidence of neutrophil engraftment was 95.4％,and the median time of the engraftment was 17 days (11-38 days).The cumulative incidence of platelet engraftment at 120th day was 84.6％,and the median time of the engraftment was 36 days (14-93 days).The cumulative incidence of erythrocyte engraftment at 60th day was 92％,and the median time of engraftment was 22 days (9d-60 days).After the umbilical cord blood provided by each bank was used in UCBT,it got the difference in cumulative incidence of engraftment.The P values for cumulative incidence of neutrophil,platelet and erythrocyte engraftment were respectively 0.004,0.01 and 0.000 2,with the differences being statistically significant.At 100th day after UCBT,the cumulative incidence of Ⅱ-Ⅳ and Ⅲ-Ⅳ degrees of acute graft-versus-host disease (aGVHD) was respectively 28.63％ and 15.7％.After umbilical cord blood provided by each bank was used in UCBT,it got the difference in cumulative incidence of aGVHD.There was no significant difference between Ⅱ-Ⅳ and Ⅲ-Ⅳ degrees (P =0.809 and 0.68 respectively).At 3rd year after UCBT,the cumulative incidence of relapse was 15.89％.After umbilical cord blood provided by each bank was used in UCBT,there was no significant difference in the cumulative incidence of relapse (P =0.898).At 3rd year after UCBT,the overall survival (OS) rate and disease free survival (DFS) rate were respectively 66.7％ and 59％.After umbilical cord blood provided by each bank was used in UCBT,it got the difference in OS and DFS.There was no significant difference in OS and DFS (P =0.566 and 0.703 respectively).At 3rd year after sUCBT,the rate of graft-versus-host diseases/relapse-free survival (GRFS) was 54.3％.After umbilical cord blood provided by each bank was used in UCBT,there was no significant difference in the rate of GRFS (P =0.449).Conclusion The umbilical cord blood provided by China's public cord blood bank was used in UCBT.It has a high safety and good efficacy in treating malignant hematologic diseases.But it needs to set up the standardized and normalized quality-control system of umbilical cord blood for China's public cord blood bank.

Objective To explore stress changes of articular surface of knees after unicompartmental knee arthroplasty (UKA) using the finite element analysis,so as to make clear the advantages and disadvantages of such a surgery.Methods Dynamic finite element analysis was performed for 6 patients after UKA and 6 healthy subjects,and compared with previous analysis of patients undergoing total knee arthroplasty (TKA).Results The stress of medial compartment was significantly bigger than that of the lateral of normal knees when bending to 30° (P＜0.05).It was the same case for postoperative knees of TKA in the whole flexion movement and those of UKA at the bending angle of 0°,60° and 90° (P＜ 0.05 for both).At the bending angle of 60° and 90°,the stress of the medial compartment of postoperative UKA knees was significantly bigger than that of the TKA knees and in the whole flexion movement,the stress of the lateral compartment of the former was significantly bigger than the latter,The stress of lateral compartment of postoperative UKA knees was bigger than the normal controls at the bending angle of 0°,30°,60° and 120° (P＜0.05).Conclusion UKA may increase the stress of knee joints and accelerate the development of osteoarthritis of the contralateral compartment,leading to poor long-term effect.However,the surgery still has advantages if strictly choosing surgery indications,optimizing the surgical operations,properly improving prosthesis and reasonably guiding patients rehabilitation.

Objective : To observe the dynamic expression of recombinant lysyl oxidase like protein 1 ( LOXL1) in the lysine oxidase family in the liver of C57BL/6 mice infected with Schistosomajaponicum and explore its role in hepatic fibrosis． Methods: Mice were infected subcutaneously with cercariae of S．japonicum，and sacrificed with euthanasia in 6，9 and 12 weeks after infection．The sera and liver tissues were collected．The levels of liver fibrosis in mice was dynamically evaluated by HE and Sirius red staining，and the serum transaminases were detected．The dynamic expression levels of collagen type Ⅰ ( Col1) ，LOXL1 and α-smooth muscle actin( α-SMA) in liver tissues were determined respectively by Western blot and qPCR. Finally，the dynamic levels of soluble and insoluble collagens were detected. Results: The result of HE and Sirius red staining showed that hepatic fibrosis levels increased at 6 weeks，peaked at 9 week，and decreased at 12 week in response to S．japonicum infection．Western blot and q-PCR showed that the expression levels of LOXL1，Col1 α1，Col3 α1 and α-SMA was significantly up regulated and reached maximum at the 9th week in response to S．japonicum infection．Soluble collagen protein levels reached maximum at the 9th week．and decreased at 12 week，however insoluble collagen protein levels continued to increase． Conclusion There may be a correlation between LOXL1 and fiber cross-linking in the process of hepatic fibrosis in S．japonicum，and it plays a role in promoting hepatic fibrosis.

OBJECTIVE: To identify pathogenic mutation in a Chinese family affected with hereditary spastic paraplegia (HSP) through genetic testing and a follow-up survey. METHODS: Whole exome sequencing was performed on DNA samples of two patients and one unaffected member to screen candidate mutations. Sanger sequencing was used to validate the suspected mutations in all ten family members. RESULTS: Four patients and three asymptomatic members (under 25 years old) carried a c.1771T>C mutation of the KIAA0196, while the other three asymptomatic members (over 40 years old) did not carry the mutation. The mutation was predicted to be "affect protein function", "probably damaging" and "disease causing" by SIFT, PolyPhen-2 and Mutation Taster, respectively. Three asymptomatic carriers were followed up and one of them developed HSP one year later, while the other two had no signs of the disease yet. CONCLUSION The clinical phenotype of the c.1771T>C mutation of KIAA0196 has a considerable heterogeneity and this mutation may be a common pathogenic site of KIAA0196 mutations among Chinese patients with hereditary spastic paraplegia.
Adult ; Asian Continental Ancestry Group ; Heterozygote ; Humans ; Mutation ; Pedigree ; Phenotype ; Proteins ; genetics ; Spastic Paraplegia, Hereditary ; genetics