Acquired aplastic anemia is a refractory blood disease for children,and severe acquired aplastic anemia can be a great threaten for life.China is a high incidengce area,children at a period during which this disease can mostly occur.In China,except some congenital aplastic anemia,most children who have this diease is acquired aplastic anemia.In 2009,the Britain society of hematology recommended Guidelines for the diagnosis and management of aplastic anaemia,which represented the latest diagnosis and treatment principle and mainstream view of the clinical methodology about aplastic anemia.They make it clear that the main treatment for the disease are allogeneic hematopoietic stem cell transplantation(allo-HSCT) and immunosuppressive therapy(IST).This article collects the documents in recent 5 years,and provides a overview about the latest advance of the allo-HSCT and IST in the word.

Objective To investigate the expression of thiredoxin domain containing 5 (TXNDC5) in the synovial tissues and blood samples of various arthritic conditions and autoimmune diseases to further confirm the previous findings, investigate the relations between the expression level of TXNDC5 and clinical parameters of RA. Methods The expression of TXNDC5 in the synovium was quantitatively analyzed by immunohistochemistry, real-time quantitative PCR and Western blotting. The levels of TXNDC5 in blood and synovial fluid was determined using sandwich ELISA in patients with RA, osteoarthritis (OA), systemic lupus erythematosus (SLE), ankylosing spondylitis (AS) and normal controls. One-way ANOVA, LSD test and Spearmen' s correlation were used for statistical analysis. Results Immunohistochemistry indicated that TXNDC5 expression was significantly higher in the synovial tissues of RA (100%, 40±9) than in those of OA and AS(200%,4±4). Real time PCR and western blotting confirmed the above findings (P<0.01). Sandwich-ELISA indicated significantly elevated level of TXNDC5 in the blood and synovial fluid of patients with RA (A=1.31±0.37), but not in those of OA, SLE, and AS, the healthy controls (P<0.01). The level of TXNDC5 in the blood of RA patients (A=0.8185±0.299) was positively correlated with the level of anti-CCP (r=0.350, P =0.027). Conclusion The results suggest that the pronounced increase of TXNDC5 expression may stimulate synovial pannus formation in the hypoxic environment of RA.

ObjectiveTo investigate the expression of ATA1 in the synovial tissue from patients with ankylosing spondylitis (AS) and to localize expression of ATA1 in AS synovial membranes.In addition,tag SNPs were genotyped to determine the possible association of this gene with AS risk.MethodsWestern blotting analysis was applied to determine the expression of ATA1 in the synovial tissues by comparing the expression profiles of AS(n=8),rheumatoid arthritis(RA,n=9) and osteoarthritis(OA,n=9) samples.Immuno-histochemistry was used to localize the expression of ATA1 in the synovial membrane.The levels of ATA1 in the synovial fluid of patients with AS were determined using ELISA with OA and RA as controls.Taqman method was used to genotype tag SNPs (rs2753934,rs2749531 and rs6575424) in 56 AS cases,260RA cases and 160 healthy controls.ANOVA,LSD test andx2 test were used for statistical analysis.Results Increased expression of ATA1 in synovial membranes of AS was found when compared with samples from RA and OA.ELISA results showed significantly elevated level of ATA1 in the synovial fluid of patients with AS (1.6+0.6),but not in samples of RA(1.4±0.5) and OA (1.2±0.5)(P＜0.05).Haplotype analysis did not reveal a haplotype association in AS or RA(P＞0.05).ConclusionThe current findings suggest that upregulation of ATA1 may play an important role in the pathogenesis of AS.

Objective To analyze the risk factors of laryngeal carcinoma.Methods The data of laryngeal carcinoma inpatients of our hospital from October 2005 to November 2007 were reviewed retrospectively by a case control analysis.Results The males who have smoking addiction,alcohol consumption,family history of malignant tumor and have more risks to suffer from laryngeal carcinoma.For The family history,OR is 2.01 in the single factor analysis,and in the multivariate analysis the OR is 2.27.Conclusion Gender,smoking,alcohol and family history of malignant tumor have great effect on the risk of laryngeal carcinoma.Family history of malignant tumor is an independent risk factor.

Objective The present study investigated the expression of the citrullinated proteins in the synovium and serum of rheumatoid arthritis(RA)patients.Methods The expression of the citrullinated proteins in the synovium and serum of RA patients was analyzed by two-dimensional western blotting analysis (2-D WB),mass spectrometry MALDI-TOF/TOF MS,western blotting,immunohistochemistry and ELISA.Then we analyzed the data with one-way ANOVA,LSD test,Kruskal-Walls test and Spearman correlation analysis.Results Alpha-1-antitrypsin(A1AT),fibrinogen beta chain(FIBB),keratin type Ⅱ cuticular Hb4(KRT84),tubulin beta chain(TBB)and vimentin(VIME)were detected in RA serum and anti-citrulline antibody could be detected using 2-D WB.A1AT,KRT84 and TBB were expressed significantly in the synovial membranes and synovial fluids of RA patients.Furthermore,high levels of autoantibodies against KRT84 were detected in the blood of RA patients when compared with samples from the healthy controls.Conclusion Current study has identified novel autoantigens in RA,including A1AT,FIBB,KRT84,TBB and VIME using 2-D WB with purified RA sera and anti-citrulline antibody.FIBB and VIME have been confirmed to be autoantigens in the literature,this demonstrates the feasibility of our protocol and the reliability of our study results.

AIM: To investigate the potential role of 14-3-3 tau in trophoblast cells on invasiveness. METHODS: 14-3-3 tau expression was detected in first-trimester villi, deciduas and human trophoblastic cell line (BeWo) by immunohistochemistry. Small interference RNA (siRNA) targeting 14-3-3 tau was transfected into BeWo cells. The effects of down-regulated 14-3-3 tau on invasion of human trophoblasts cell line BeWo were examined by matrigel invasion assay, and the transcription, translation of E-cadherin and snail were estimated by RT-PCR or Western blotting. U0126 was used to detect the extracellular-signal related kinase 1/2 (ERK1/2) function on down-regulation of 14-3-3 tau induced cell invasion. RESULTS: 14-3-3 tau was detected in the invasive trophoblastic cells in the first trimester villi and that invaded to the deciduas. BeWo cells also expressed 14-3-3 tau. Down-regulation of 14-3-3 tau increased the invasive cell-number of BeWo, as well as the expression of snail, and inhibited E-cadherin. U0126 inhibited the enhanced invasiveness in these cells induced by the down-regulation of 14-3-3 tau. CONCLUSION: 14-3-3 tau may regulate the invasiveness of human trophoblastic cells through ERK1/2 signaling pathway.

OBJECTIVE To investigate the reasons and preventive measures of recurrent laryngeal nerve injury during operation in patients with benign thyroid neoplasm. METHODS The clinical data of 2243 cases with benign thyroid neoplasm were reviewed. The relations among different neoplasm regions, different procedures and injury of recurrent laryngeal nerve were analyzed. RESULTS Injury incidence of recurrent laryngeal nerve in this study was 3.0 %. In cases with nodular goiter on the dorsum of thyroid and recurrent laryngeal nerve exposed during operation, injury incidence of recurrent laryngeal nerve was 1.0 %. And there was no recurrent laryngeal nerve injury in cases with nodular goiter on other region of the thyroid. In cases used locality protection during operation, injury incidence of recurrent laryngeal nerve was 7.6 % when benign lesions on the dorsum of thyroid and was 0.17 % when benign lesions on other region of the thyroid. CONCLUSION In cases with nodular goiter or adenoma on the dorsum of thyroid, exposing recurrent laryngeal nerve is necessary during operation and in cases with benign lesions on other region of thyroid, locality protection is useful. There are three major routes for recurrent laryngeal nerve exposure.

Objective To observe and compare the clinical characteristics of colorectal related multiple primary carcinoma (MPC) and colorectal carcinoma (CRC) for promoting early diagnosis and treatment of colorectal related MPC. Methods Pathological and clinical documents of clearly staged CRC and colorectal related MPC cases from Jul. 1997 to Nov. 2007 were retrospectively analyzed. Results 573 colorectal carcinoma cases were analyzed, including 45 MPC (7.85 %). Parenteral multiple carcinoma originated most frequently from stomach, and then breast, ovary, lung, small intestine and other sites. Among all multiple primary colorectal carcinomas (MPCC), ascending colon carcinoma was most frequent (34.0 %).While among CRC cases, rectal cancer cases was most frequent(36.5 %). Comparing CRC and MPCC, there were no significant difference in terms of tumor family history. Median morbidity age was 57 years and 63 years respectively. Cases with previous colonic polyps accounted for 20.0 % of all MPC cases, while only 0.9 % of all CRC cases. The mOS of CRC and MPC was 93.7 month and 64.8 month respectively. Most frequent pathological type of CRC and MPC were both well-moderately differentiated adenocarcinoma, but more mucinous adenocarcinoma cases were observed in MPC. Conclusion Colorectal related MPC are relatively common among colorectal carcinoma patients. More patients with MPC especially MPCC has colonic polyp. mOS of MPC is shorter than that of CRC, indicating the poor prognosis of MPC compared with CRC.MPCC has multiple colonic polyps, shorter interval of secondary carcinomas, and shorter mOS, worse prognosis than MPC with parenteral tumor.

Objective The present study investigated the expression of heat shock cognate protein 70 (Hsc70) in the synovial tissues and blood samples of patients with rheumatoid arthritis (RA) to determine the pathological role of this protein in the pathogenesis of the disease.Methods The expression of Hsc70 in synovial membranes was quantitatively analyzed by immunohistochemistry,real-time quantitative PCR and western blotting.The samples from osteoarthritis (OA) and ankylosing spondylitis (AS) were used as controls.The levels of Hsc70 in blood of patients with RA were determined using enzyme linked immunosorbent assay (ELISA) with the samples of the healthy subjects as controls.Statistical analysis was conducted with one-way ANOVA,LSD test and Spearmen's correlation.Results Immunohistochemistry showed that Hsc70 had significantly increased expression in synovial tissues of RA than in the samples of OA and AS.Real-time PCR and western blotting confirmed the above findings.ELISA detected significantly elevated level of Hsc70 in blood of patients with RA as compared with samples from the controls (P＜0.01).Conclusion The study suggests that the up-regulation of Hsc70 may be involved in the pathogenic process of RA.

Objective To observe the inhibiting activities of fibronectin (FN) and citrullinated fibronectin (cFN) on disintegrin and metalloproteinase with thrombospondin motifs-4 (ADAMTS-4),and to explore the extracellular regulative mechanisms of ADAMTS-4.Methods FN was incubated with peptidylarginine deaminase type 4 (PADI4).Western blotting analysis was used to verify the citrullination of FN.The binding activity of FN and cFN to ADAMTS-4 were investigated by enzyme-linked immunosorbent assay (ELISA).The proteolytic ability of ADAMTS-4 after binding to FN and cFN were measured with the aggrecanase activity assay kit.One-way ANOVA,LSD-t test and t-test were used for statistical analysis.Results The immunosignal of citrulline was detected in FN after incubated with PADI4,but not in the absence of PADI4.A higher absorbance at 405 nm was detected when the full-length ADAMTS-4 protein was incubated with FN (2.182±0.042) than cFN (0.624±0.033; t=50.522,P＜0.01).Additionally,the recombinant ADAMTS-4 protein with a truncation at the C-terminus displayed low absorbance at 405 nm when the enzyme was incubated with both FN(0.971±0.024) and cFN(0.934±0.012; t=2.388,P＞0.05).Large amounts of ARGxx peptide were detected with full-length ADAMTS-4 in aggrecanase activity assay [(0.908±0.088) nmol/L],but significantly less when in the presence of FN and ADAMTS-4 [(0.573±0.000) nmol/L,P＜0.05].The production of this peptide was more when full-length ADAMTS-4 was incubated with cFN [(0.830±0.020) nmol/L,P＜0.05] than with FN.The reaction containing the truncated ADAMTS-4 without FN or cFN yielded the highest concentration of ARGxx peptide [(36.420±3.673) nmol/L],peptide production was not significantly altered when FN [(41.099±0.101) nmol/L] or eFN [(41.064±0.083) nmol/L] were added to the reaction.Conclusion FN could bind to ADAMTS-4 and inhibit its proteolytic activity.After citrullinated by PADI4,the binding activity of cFN is weakened and less inhibition to ADAMTS-4.