Objective To investigate the clinical features of X-linked agammaglobulinemia (XLA)in children,and to improve the early diagnostic rate.Methods The medical records of 11 children with XLA between Dec 2003 and Nov 2011 were reviewed.Results The smallest age of 11 cases with XLA presenting the first symptoms was 0.4 years old and the oldest age was 4 years old with a mean of 2.4 years old.The first diagnosis time was at the age of 3.5 to 13 years old,with a mean of 7 years old.The age at first diagnosis in 63.6％ ( 7/11 ) of patients was more than 7 years old.Two patients ( 18.2％ ) had the family history of the similar disease.Two patients were died from the infection and 1 patient was missed.The other 8 patients were survived.Respiratory infections occurred in 100％ of the 11 patients.54.5％ (6/11 )of the patients were suffered with otitis media and digestive infections were seen in 36.4％ (4/11 ) of the patients.The levels of lgA,IgM and IgG in the serum of the pauents were all decreased.The levels of IgG in the serum of 9 patients were less than 2 g/L and in the serum of the other 2 patients were less than 2.4 g/L.The levels of CD19 in the serum were all less than 1％ and an inversed ratio of CD4/CD8 was observed in 9 patients.Eight patients were diagnosed as XLA by gene detection.Conclusion The age presenting first symptoms in children with XLA in this study is earlier than the reported data and the age at first diagnosis is later.Respiratory infection is the most common manifestation.High prevalence of otitis media and digestive infections are common.The patients with XLA rarely have the family history.The early diagosis and long-term treatment with the intravenous immunoglobulin may improve the prognosis of XLA.

Objective To investigate the impact of compliance and quality of life of psychological intervention for cancer patients and their primary caregivers,as well as the correlation between the psychological issues of patients and their primary caregivers.Methods The enrolled patients were randomly divided into intervention group and control group.The patients in intervention group were given to standardize anti-tumor therapy,while the patients and their primary caregivers were given psychological intervention once a week.The patients in the control group only received standard anti-tumor therapy.By TDL determination of quality of life,anxiety rating scale (SAS) and self-rating depression scale (SDS),in front of the psychological intervention after 8 weeks of intervention,the two groups of patients and their primary caregivers were questionnaired,and recorded the completion of the treatment plan.By SPSS 12.0 software,the statistics were completed.Results 51 cases in intervention group and 38 cases in control group were able to complete the number of people expected to treat there was a statistically significant (P ＜ 0.05).TDL determination and quality of life scores in intervention group patients and their primary caregivers were significantly higher (P ＜ 0.05).SAS and SDS score in intervention group patients and their primary caregivers were significantly lower than control group (P ＜ 0.05).Conclusion The effective psychological intervention to cancer patients and their primary caregivers during the treatment of patients could improve the compliance of cancer patients,the quality of life of cancer patients and their primary caregivers.The psychological problems between the patients and primary caregivers are positive correlation.

Objective: To study the compatible stability of cyclophosphamide (CTX) and Mesna admixture for injection in 4 commonly used infusions (5% glucose injection,0.9% sodium chloride injection,fructose injection and xylitol injection) at different temperatures.Methods: An HPLC method was adopted to determine the contents of CTX for injection in 24h respectively under 5℃ and 25℃.The appearance and pH value of the admixture were investigated.Results: No significant changes of appearance, pH value or content of the admixture were found out in 24h under 5℃ and 25℃.Conclusion: CTX and Mesna admixture for injection is compatible with the 4 commonly used infusions under 5℃ and 25℃ for clinical use in 24 h.

Objective To approach the effective treatment for simple bone cyst in adult. Methods Forty-eight cases were divided into two groups by visiting order, 20 cases (steroid group) were treated by prednisolone acetate and 28 cases (open resection and allograft group) were treated by open resection and allograft. The curative effect were contrasted between two groups. Results The follow-up time was 5-46(26.58 ± 10.81) months. Aecording to the Chigira's healing criteria for simple bone cyst, grade Ⅰ , Ⅱ , Ⅲ,V were 1, 2, 10 and 7 cases respectively in steroid group, contrasting to 0, 3, 5 and 20 cases respectively in open resection and allograft group. The recovery rate was 85.0%(17/20) in steroid group and 89.3%(25/28) in open resection and allograft group, which was no statistically significant difference between two groups (P＞0.05). Conclusions Although the two remedies are no obvious difference in curative effect,simple bone cyst at lower extremity of weight-bearing with obvious osteolysis in adult is recommended to open resection and allograft, otherwise or at upper extremity to steroid injection.

Objective To compare the applicability of the Beijing Version of the Montreal Cognitive Assessment (MoCA) and the Mini Mental State Examination (MMSE) in screening for cognitive impairment in patients with acute ischemic stroke for 2-3 weeks.Methods MoCA and MMSE were conducted in 201 patients with acute ischemic stroke within 2 to 3 weeks after the onset of stroke.With MoCA＜23 and MMSE ＜26 as the cut off value,we assessed the clinic effect of the MoCA and MMSE and explored the correlation between two instruments.Results The average scores of MoCA and MMSE scale were (20.5±4.3) and (25.4±3.5) points.The prevalence of cognitive impairment evaluated with MoCA and MMSE were 57.2％and 43.3％,respectively.MoCA showed significant correlation with MMSE score (Pearson's correlation coefficient=0.833,P＜0.001),and an agreement with Kappa values of 0.532 (P＜0.01) in screening for cognitive impairment.Conclusions The prevalence of cognitive impairment assessed with MoCA is higher than that of with MMSE when using MoCA＜23 and MMSE＜26 as the cut off values.Both instruments show a good agreement for screening cognitive impairment in acute ischemic stroke within 2 to 3 weeks following the disease onset.

ObjectiveTo study the effect of Ghrelin on activation of UCP3 and phosphorylation of ACC under the high fat environment in rat myoblast,and to support the study on prevention of type 2 diabetes.MethodsThe rat myoblast were exposed to 0.3 mmol/L palmitic acid for 12 h,at the same time,10-11 mol/L,10-9 mol/L,10-7 mol/L Ghrelin were added.The expression of UCP3 mRNA was determined by RT-PCR.The protein levels of UCP3 and p-ACC were measured by western blotting.ResultsCompared with the control group,UCP3 mRNA and the protein levels of UCP3 and p-ACC were obviously decreased in 0.3 mmol/L PA group(UCP3 mRNA:P =0.000,UCP3 protein levels:P =0.000,p-ACC protein levels:P =0.003).Compared with the 0.3 mmol/L PA group,with the increasing concentration of Ghrelin,UCP3 mRNA and the protein levels of UCP3 and p-ACC were elevated.The effects of 10-7 mol/L Ghrelin were significant(UCP3 mRNA:P =0.017,UCP3 protein levels:P =0.000,p-ACC protein levels:P =0.007).ConclusionsGhrelin could enhance activation of UCP3 and phosphorylation of ACC under high fat environment in rat myoblast.

Objective To investigate the clinical efficiency of Isophane Protamine Biosynthetic Human Insulin Injection (pre‐mixed 30R)(Novolin 30R) combined with insulin pump therapy in patients with diabetes mellitus complicated with pulmonary in‐fection ,and to analyze the detection indexes .Methods From Jan .2010 to Feb .2013 ,90 cases of patients with diabetes mellitus com‐plicated with pulmonary infection were selected and divided into the observation group and control group .The control group re‐ceived Novolin 30R treatment and conventional treatment ,the observation group were given insulin pump therapy based on the treatment of control group .The clinical efficiency and detection indexes were compared and analyzed .Results The cure rate and to‐tal effective rate were 51 .11% and 82 .22% respectively in the control group ,and were 84 .44% and 100 .00% in the observation group respectively .There were significant differences of both cure rate and total effective rate between the two groups (P<0 .05) . There were statistically significant differences of hospitalization time ,the time for reaching target of glucose ,insulin dose ,recovery time of body temperature ,hospital costs ,incidence rate of complications ,serum levels of sodium ,potassium and urea nitrogen ,os‐molality and PH value between the two groups(P<0 .05) .Conclusion The combination of Novolin 30R and insulin pump therapy could provide satisfactory clinical efficiency in patients with diabetes mellitus complicated with pulmonary infection .

Objective To investigate the changes of the cell cycle regulators ATM, Chk2 and p53 and cell cycle arrest in HeLa cells after cisplatin therapy. Methods The proliferation-inhibiting rates of HeLa cells induced by eisplatin of different concentrations were measured by MTT assays. The mRNA and protein expressions of ATM, Chk2 and p53 of HeLa cells with and withont cisplatin were detected by RT-PCR and Western blot, respectively. The cell cycle analysis was conducted by flow cytometric analysis. Results Cisplatin inhibited the proliferation of HeLa cells in a dose- and time-dependent manner. The mRNA and protein expressions of ATM, Chk2 and p53 were increased in HeLa cells treated with cisplatin. The cell cycle was arrested in G2/M phase in HeLa cells treated with cisplatin. Conclusion Activation of ATM, Chk2 and p53 might be critical in determining whether cells survive or undergo apoptesis. Targeting ATM, Chk2 and p53 pathway might he a promising strategy for reversing chemoresistance to clsplatin in cervical cancer.

OBJECTIVE:To evaluate the feasibility and initial outcome of reconstructing the defect femur by unicondylar osteoarticular altograft after resection of distal femur tumor.METHODS:Between July 2003 and August 2008,a total of 12 patients with distal femur tumor treated by unicondylar resection and fresh-frozen unicondylar osteoarticular allograft reconstruction of bone defects were retrospectively reviewed at the Department of Orthopedics,General Hospital of Chinese PLA.Of 12 patients,5 medial and 7 lateral femoral condyles were involved.There are 8 patients suffering from giant cell tumor,including 2 patients complicating with pathologic fracture,3patients suffering from osteosamoma and 1 patient suffering from low-grade fibrosarcoma.Before transplantation,MRI and/or CT were used to determine that the tumor was definitely limited in half condyle.Three cycles of chemotherapy preoperatively and six cycles of chemotherapy postoperatively were conducted in patients suffering from osteosarcoma and fibrosarcoma.Patients with giant cell tumor did not receive any intraoperative adjuvant treatment.The surgical margin of resected tumors was evaluated by histological examination.All patients were followed by radiograph or CT.Postoperative function was evaluated by Musculoskeletal Tumor Society (MSTS) scoring system at the time of the latest follow-up.RESULTS:All patients were followed up.The patients were followed up mean 35 months after transplantation.None of the patient died.No local recurrences and metastases were found at the latest follow-up.No evident immune rejection and deep infection were seen.No plate loosening or breakage was observed.All patients had their grafts retained at the time of the latest follow-up.Three patient's allograft showed subchondral bone collapse and articular deterioration,but the function was good.Instability of the knee joint was noted in 3 patients.The mean MSTS functional score (totally 30 points) was 26 points.CONCLUSION:For some patients with giant cell tumor or malignant tumors with clearly defined margins after chemotherapy.When the lesions were limited in half condyle and enough surgical margins can be achieved,reconstruction of bone defect with unicondylar osteoarticular allografts after bone tumors were resected appears to be a reliable alternative.

Objective To investigate the clinical features,diagnosis and treatment of appendix tumor.Methods The clinical data of 58 cases with primary appendiceal tumor were analyzed retrospectively.Results In the 58 cases clinical presentation mimicked acute appendicitis in 22 cases,chronic appendicitis in 15 cases,appendiceal abscess in 12 cases,intra-abdominal mass in 7 cases,and gastrointestinal perforation in 2 cases.Primary appendiceal tumor was diagnosed intraoperatively by intraoperative frozen histopathological examination in 10 cases,and the diagnosis was made by postoperative histopathological examination in the other 48 cases.There was appendiceal carcinoid in 40 cases,adenocarcinoma in 6 cases,mucocele in 2 cases,mucous adenocystoadenoma in 5 cases,pseudomyxoma peritonei in 2 cases,malignant neurilemmoma in 1 case,and malignant lymphoma in 2 cases.Surgical procedures included appendectomy in 6 cases,ileocecal resection in 8 cases,and right hemicolectomy in 44 cases (including right hemicolectomy and intraperitoneal chemotherapy with 5-FU 1 000 mg for pseudomyxoma peritonei in 2 cases).Radical resection was achieved in 55 cases and palliative resection in 3 cases.The 2 cases with pseudomyxoma peritonei died of tumor recurrence at 36 months and 54 months after operation respectively.All the 5 cases of adenocystoadenoma and 2 cases of appendix cyst survived without an evidence of recurrence.7 of 49 cases of malignant appendiceal tumor suffered recurrence postoperatively,the recurrence rate was 14％,which included liver metastasis in 4 cases and intraperitoneal recurrence in 3 cases.The 1,3,5-year survival rates of malignant appendiceal tumors were 100％ (49/49),92％ (35/49) and 80％ (39/49),respectively,which were 100％,98％ and 92％ for carcinoid,and 100％,67％ and 33％ for adenocarcinoma,respectively.Conclusions The preoperative diagnosis of primary appendiceal tumor is very difficult,the intraoperative frozen histopathological examination is helpful for diagnosis,the prognosis of appendiceal carcinoid is fair after resection.