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.

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.

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

Objective To investigate the relationship between serum adipocyte fatty acid binding protein (FABP4),insulin resistance and maternal complications in patients with gestational diabetes mellitus (GDM) with satisfactory glucose control.Methods 60 cases of pregnancy between 24 and 28 weeks of OGTT diagnosed GDM pregnant women were selected.30 cases with satisfactory blood sugar control were selected as satisfactory blood sugar control group,30 cases with unsatisfied blood sugar control were selected as unsatisfied blood glucose control group.Healthy control group was selected from 24 to 28 weeks of healthy pregnancy in 30 patients.Fasting serum FABP4,fasting blood glucose(FPG) and fasting insulin(FINS) were measured in three groups of pregnant women after 24 to 28 weeks and pregnant term.To calculate the insulin resistance index (HOMA-IR) of the steady state model,and record and observe the complications of the three groups.Results In the satisfactory blood glucose control group,the incidence rates of pregnant hypertensive disorders,excessive amniotic fluid,premature rupture of membranes,postpartum hemorrhage,cesarean section,macrosomia,neonatal asphyxia,neonatal hypoglycemia,hyperbilirubinemia of newborn were 10.0％,6.7％,13.3％,6.7％,23.3％,16.7％,10.0％,16.7％,10.0％,which were significantly lower than those in the unsatisfied blood glucose control group (23.3％,20.0％,23.3％,16.7％,33.3％,33.3％,20.0％,30.0％,36.7％),the two groups had significant differences(x2 =4.33,6.12,6.01,3.97,7.41,5.46,10.02,4.79,9.22,all P ＜ 0.05).The incidence rates of pregnant hypertensive disorders,excessive amniotic fluid,macrosomia between the satisfactory blood glucose control group (10.0％,6.7％,16.7％) and healthy control group(3.3％,0.0％,6.7％) had statistically significant differences (x2 =7.45,8.46,4.69,all P ＜ 0.05).The incidence rates of postpartum hemorrhage,premature rupture of membranes,cesarean section,neonatal asphyxia,neonatal hypoglycemia,neonatal hyperbilirubinemia between the satisfactory blood glucose control group (6.7％,13.3％,23.3％,10.0％,16.7％,10.0％) and healthy control group(10.0％,10.0％,20.0％,6.7％,13.3％,3.3％) had no statistically significant differences (all P ＞ 0.05).Pregnant 24 ～ 28 weeks,FABP4,FINS,FPG and HOMA-IR of the satisfactory blood glucose control group were (1.78 ± 0.33) ng/mL,(12.35 ± 0.48) mIU/L,(5.51 ± 0.96) rmmol/L,(3.88 ± 0.55),which of the unsatisfied blood glucose control group were (2.36 ± 0.08) ng/mL,(13.92 ± 1.17) mIU/L,(5.46 ±0.74)mmol/L,(3.95 ± 1.17),the differences between the two groups were not statistically significant (all P ＞ 0.05),and compared with the healthy control group,the differences were statistically significant(t =15.32,10.36,11.54,7.34,all P ＜ 0.05).After term pregnancy,the FINS,HOMA-IR of the satisfactory blood glucose control group were still higher than those in the healthy control group,the differences were statistically significant(all P ＜ 0.05).However,FABP4,FPG between the satisfactory blood glucose control group and healthy control group had no statistically significant differences (all P ＞ 0.05).Conclusion With the control of blood glucose levels,decreased in patients with GDM FABP4,fasting blood glucose,glycosylated hemoglobin level,but high insulin resistance still persists and glycemic control satisfaction does not completely reduced the occurrence of the complications in both mothers and neonates.

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.

Objective To analyze the correlation between 18F-FDG SUV and immunohistochemical index including GLUT1, Ki-67, MVD, survivin and cyclinA in non-snall-cell lung cancer. Methods Thirty-three patients with NSCLC underwent preoperative PET/CT examination and surgical operation. All patients were divided into two groups according to the size of tumor (cutoff=3 cm), metastasis of mediastinal or hilar lymph nodes or not, and histological types of the cancer, respectively. The expression of GLUT1, Ki-67, MVD, survivin and cyclinA were estimated with SP immunohistochemical technique, and were analyzed statistically to reveal the correlation to FDG SUV. Results The rate of positive expression of GLUT1, Ki-67 and CD34 were 66.67%, 72.73% and 100%, respectively. The mean value of CD34 in all 33 patients was 12.6±2.9 (12-56). The rate of positive expression of survivin was 84.85%, and the corresponding data of cyclinA was 27.27%. Conclusion There is linear correlation between FDG PET SUV and GLUT1, but not between FDG PET SUV and Ki-67, MVD, survivin and cyclinA. The expressions of GLUT1, Ki-67, MVD, survivin and cyclinA are not related with the size of tumor, nor metastasis of lymph nodes. The expression of GLUT1 and Ki-67 is related with histological types of the cancer, but not with MVD, survivin and cyclinA.