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 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 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 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.

Purpose To investigate the association of multi-slice spiral CT (MSCT) appearances and ischemia time as well as ischemia degree through both unenhanced and contrast-enhanced MSCT scans, then to investigate the value of MSCT in diagnosing acute mesenteric ischemia (AMI) by comparing the MSCT findings with pathologic examinations after establishing canine's AMI model. Materials and Methods 18 healthy hybrid canines were successfully punctured into the right femoral artery through seldinger's technique and injected absolute ethanol into the distal branches of superior mesenteric artery (SMA) for embolization via a 5F Cobra catheter. All experimental canines were underwent both unenhanced MSCT and enhanced CT scans no matter before nor after embolization. Every 3 canines were put to death randly each hour and ischemia bowel biopsies were examined. Results The AMI's models were suceessfully established in all 18 canines. The MSCT appearances were not same in different periods. Mesenteric stranding, bowel-wall thickening and luminal dilatation occurred in early periods and ascites, intramural gas and portal venous gas occurred lately. The contrast-enhanced degrees of abnormal bowel walls after embolization were declined than that of preoperation. The canines of three-hour to six-hour ischemia groups developed microscopic and gross changes of bowel ischemia. Conclusion MSCT can make the diagnosis of AMI accurately and the appearances of CT were gradually more variety along with the ischemia time longer. The contrast-enhanced degrees of abnormal bowel walls were negative correlation with ischemia time as well as ischemia degree.

Norovirus ; chemistry ; genetics ; physiology ; ultrastructure ; Open Reading Frames ; Virus Replication

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 observe the anti-tumor action of Fuzheng Yuliu Tablet(FYT).Methods Mice received subcutaneous injection of S180 into right axilla.Then the tumor-implanted mice were randomized into 6 groups: model group,FYT groups(at the dose of 2.4 and 12.4g/kg respectively),cisplatin group(at the dose of 8?g/d),and cisplatin+FYT groups.The treatment lasted 10 days.The tumor-inhibitory rate and the life-prolongation rate were observed after treatment.The cultured ovarian cancer cell strains of SKOV3 were randomized into 4 groups:model group,two FYT groups and cisplatin group.Apoptosis of SKOV3 was detected by enzyme-linked immunosorbent assay(ELISA)after culturing with the medicine for 48 hours.Results The tumor growth was inhibited to different extends in all of the treatment groups,the decrease of tumor mass weight was obvious in cisplatin group and FYT groups,and the body weight was increased in FYT groups and cisplatin+FYT groups(P

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.