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 improve the early diagnosis of Wiskott-Aldrich syndrome (WAS),through summing up and analyzing the clinical characteristics of children with WAS.Methods The clinical and laboratory characteristics of 18 children with WAS,including the cases from Suzhou Jiulong Hospital,Shanghai Jiaotong University School of Medicine,and Tongji Hospital Affiliated to Tongji University,and the domestic reports in recent 6 years,were induced and analyzed.Results All patients were males;the average of incidence age was about 1.9 months and the average age at diagnosis was 10.8 months.All cases had the clinical characteristics of WAS including eczema,infection tendency and thrombocytopenia with small platelet size.Moreover,10/18 cases (55.56％) WAS patients progressed to severe cases (scores reached to 4 or 5 points)at diagnosis,and with the increase of age of the patients at diagnosis,the ratio of severe cases would be increased.The conventional immune function indexes showed no specific diagnostic value,and the typical clinical features and the WAS gene mutation detection were the key diagnostic basis.Seven cases used to be misdiagnosed as idiopathic thrombocytopenia,and received corticosteroids and intravenous immunoglobulin therapy.In a total of 18 cases,4 cases received allogeneic hematopoietic stem cell transplantation,of which,β cases were cured and 1 case died of transplantation-related interstitial pneumonia,while the remaining 14 cases are unknown for their with follow-up treatment and prognosis.Conclusions The typical clinical features and the WAS gene mutation detection were the key diagnostic basis.In order to improve the rate of early diagnosis and avoid misdiagnosis,it's a great need to improve the clinical understanding of WAS.

Objective:To study the changes of endothelin-1(ET-1) and malondialdehyde (MDA) in Kawasaki disease (KD) and to probe the relationship between the changes of ET-1, MDA and coronary artery pathological changes. Methods: KD group included 42 patients ( male 25, female 17), with a mean age of (3.25?0.75) years old; control group included 30 normal children (male 19, female 11), with a mean age of (3.58?0.55) years old. Serum contents of ET-1 and MDA were measured by radioimmunology and modified thiabarbituricaid(TBA) method in 2 groups, and the inner diameters of coronary artery and aorta were measured by color echocardiography. KD group was subdivided into dilated-coronary artery group and undilated group for comparison.Results: Serum contents of ET-1 and MDA in KD group were apparently higher than those in control group (-[76.63?18.36-] vs -[41.55?16.68-] pg/ml and -[3.18?0.60-] vs -[1.52?0.24-] ?mol/L,respectively;P

OBJECTIVE Explore the waysof cochlear implantation(CI)surgery technique for bilateral profound sensorineural hearing loss patients with otitis media and mastoiditis. METHODS CI was performed in 29 patients with different of otitis media and mastioditis,26 of them underwent a single-stage operation,and 3 had a two-stage operation,according to the degree and extent of lesion. For patients with mild inflammation,cochlear implantation was performed after complete eradication of inflammation at the same protocol the patients with severe inflammation underwent an intact canal wall tympanomastoidectomy or reconstructed it initially,cochlear implantation was performed after the initial procedures. RESULTS One- stage or tow-stage operations of CI were carried out for 29 patients with otitis media and mastoiditis. All electrodes were implanted successfully,in which the CI went normally and electrode array were protected well. None of the cases showed recurrence of infection during an average follow-up period of 2-8 years. CONCLUSION The CI could be performed in otits media and mastoiditis patients after reversionary surgery . Individual management based on the degree and extent of the local lesion was emphasized.

Objective To find out the endoscopic characteristics of peptic ulcer disease in Monglian area.Methods 15826 cases in hospital within past 17 years were retrospectively analyzed.Results In all endoscopic cases,2298 cases of peptic ulcer were found,the detecting rate was 14.52%.Among them,58.98%(1376 cases) were of duodenal ulcer,32.16%(739 cases) were of gastric ulcer,7.96%(183 cases) were of complex ulcer.The detecting rates of male and female patients were 18.53% and 8.06% respectively,Monglian and Han nationalities were 18.43% and 13.15% respectively.Conclusions The detecting rate of peptic ulcer in Monglian nationality is higher than in Han nationality at Monglian area,has singnificant difference(P

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.

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.

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 screen the proteins with decreased expression in the synovial tissues of rheumatoid arthritis (RA) patients by comparing their expression profiles with that of osteoarthritis (OA) and ankylosing spondylitis (AS) patients by a proteomic approach,and to explore the association of reduced expression with disease susceptibility by a single nucleotide polymorphism (SNP) analysis.Methods Proteins extracted from the synovial membranes (n=10 for each disease) were separated by 2-D electrophoresis.The proteins with significantly decreased expression in the RA samples were subjected to MALDI-TOF-MS.The results were verified using Western blotting.Tag SNPs located in the targeted gene were assessed using the Taqman assay in a cohort of 267 Chinese patients with RA and 160 healthy controls.The genotyping results were confirmed in a large cohort of 389 patients with RA and 371 healthy controls.SPSS 11.5 software package was used for one way ANOVA and Fisher's exact test.Results The expression of vitamin D-binding protein (VDBP) in the synovial membranes from patients with RA was significantly decreased when examined by proteomic approach.This result was confirmed by Western blotting analysis.The rs2282679 was significantly associated with RA (P=0.026 794).This result was confirmed in a large cohort of RA( OR=0.678 639,95％CI 0.54l 113-0.851 118,P=0.000 776).Conclusion Compared with samples from patients with OA and AS,RA patients' synovial tissues have low VDBP expression when examined by the proteomic method.The tag SNP rs2282679 located in VDBP is significantly associated with RA.The decreased expression and the genetic effect of VDBP in RA suggest that a novel pathogenic pathway,in which vitamin D contributes,may be involved in the arthritis process of RA.

Objective:To investigate the occurrence and influencing factors of perioperative complications after robotic gynecologic surgery.Methods:The clinical data and occurrence of perioperative complications in 1 000 cases robotic surgery completed in the First Affiliated Hospital of Zhengzhou University were retrospectively analyzed.Results:(1) Clinical data: the average age of the patients was (50.2±10.4) years old, and the average body mass index (BMI) was (24.4±3.6) kg/m 2. Among 1 000 cases, 811 cases of them were malignant tumors, including 405 cases of cervical cancer, 279 cases of endometrial carcinoma, 112 cases of epithelial ovarian cancer (EOC), 15 cases of vulvar cancer; 189 cases of them were benign diseases, including 43 cases of uterine prolapse, 57 cases hysterectomy of uterine leiomyoma and adenomyosis of the uterus ≥12 weeks, 84 cases myomectomy of uterine leiomyoma, and 5 cases of fallopian tubal ligation requiring anastomosis. Surgical methods: in patients with malignant tumors, cervical cancer, hysterectomy plus salpingectomy or salpingo-oophorectomy for stage Ⅰa1, and radical hysterectomy plus pelvic lymphatic dissection plus salpingectomy or salpingo-oophorectomy for stage Ⅰa2-Ⅱb. Endometrial carcinoma, performed by staging surgery. Staging surgery for EOC with early stage and cytoreductive surgery with advanced EOC. Vulvar cancer, extensive vulvar resection plus inguinal lymphadenectomy. In patients with benign diseases, uterine prolapse, hysterectomy plus salpingectomy or salpingo-oophorectomy plus sacrocolpopexy. Uterine leiomyoma or adenomyosis with uterus ≥ 12 weeks, hysterectomy plus salpingectomy or salpingo-oophorectomy. Myomectomy for patients requiring uterine preservation with uterine leiomyoma. Tubal anastomosis for patients with fallopian tubal ligation. (2) Surgical complications: intraoperative complications occurred in 25 patients (2.5%, 25/1 000), including 11 patients with vascular laceration, 11 patients with ureteral injury, 2 patients with bladder injury, and 1 patient with intestinal injury. Postoperative complications occurred in 130 patients (13.0%, 130/1 000), including 66 cases of lower limb venous thrombosis, 20 cases of lymphatic cyst, 8 cases of hydronephrosis, 9 cases of ileus, 16 cases with infection, 6 cases with genital fistula, 4 cases with trocar site herniation and 1 case with subcutaneous emphysema. The incidence of intraoperative complications was 3.1% (25/811) in malignant tumors and no case in benign diseases, the incidence rate in malignant tumors was significantly higher than that in benign diseases ( χ2=4.778, P=0.029). The incidence rate in cervical cancer (4.2%, 17/405) and EOC (3.6%, 4/112) were significantly higher than those in endometrial carcinoma (1.4%, 4/279) and vulvar cancer (0/15; P<0.05). The incidence of postoperative complications was 15.2% (123/811) in malignant tumors and 3.7% (7/189) in benign diseases. The incidence rate in malignant tumors was significantly higher than that in benign diseases ( χ2=17.807, P<0.01), but there were no significant difference among different malignant tumors ( χ2=4.318, P=0.229). (3) The correlative factors affecting the occurrence of surgical complications: patient′s age, BMI, previous pelvic or abdominal surgery history, the nature of disease (malignant or benign), operation time, and comorbidities had a significant impact on the incidence of postoperative complications ( P<0.05). Multivariate logistic regression analysis showed that the patient′s age ≥40 years old, BMI ≥25 kg/m 2, previous pelvic or abdominal surgery history, malignant tumors and comorbidities were independent influential factors of the postoperative complications ( P<0.05). Conclusions:Perioperative complications vary according to the type of the surgery. The age, BMI, previous pelvic or abdominal surgery history, malignant tumors, and comorbidities are influential factors of postoperative complications.