Objective To study the clinical characteristics and the effect of enzyme replacement therapy for late-onset glycogen storage disease typeⅡ(GSDⅡ). Methods The clinical, laboratory data and the result of genetic testing were retrospectively analyzed in a GSDⅡchild, the effect of enzyme replacement therapy was followed up and the relevant literature was reviewed. Results The patient had motor regression after 1 year old, the serum creatine kinase level is from 675 to 1286 U/L. The EMG test showed myopathic change, acid alpha-glucosidase activity is 12.0 nmol/(g·min), next generation sequencing of genetic muscle diseases panel found the GAA compound heterozygous mutations, both were tiny variations, and muscle biopsies showed the typical pathological features of GSD. The patient was given human recombinant of alpha glucoside enzyme 20 mg per kilogram of body weight, once every other week for 1 year. The weakness of the patient’s muscle strength had no obvious aggravation. Conclusions Early and adequate enzyme replacement therapy is the only possible treatment for GSDⅡ.

[Objective] To evalvate the influence of total knee replacement(TKR)on the lipid peroxdation in erythrocytes,and the prophylactic treatment of Vitamin E and fructose 1,6-diphosphate(FDP)on it.[Method]Totally 60 patients of knee osteoarthritis were divided into control group,Vitamin E group,Fructose 1,6-diphosphate(FDP)group and Vitamin E added FDP group.Blood samples were taken for biochemical determination of MDA and Cu-Zn-SOD before and after the operation at 1,3,5 and 7 days.[Result]MDA level in erythrocytes increased singnificantly after TKR compared with that before operation(P

Objective To investigate the distribution and drug resistance of pathogenic bacteria in hospital ICU patients with lower respiratory tract infection , and provide scientific reference for clinical rational drug use . Methods The strains were identified by VITEK -32 automatic bacterial identification instrument , the bacterial sensitivity was determined by K -B disk diffusion method , and the statistical analysis was performed by WHONET 5.4 software.Results The total isolated pathogenic bacteria of lower respiratory tract infection in ICU patients was 453 strains.332 strains of gram negative bacteria accounted for 73.3%,and the former three ones were Pseudomonas aeruginosa (115 strains,accounted for 25.4%),Klebsiella pneumonia (90 strains,accounted for 19.8%),Acineto-bacter baumannii (38 strains,accounted for 8.4%).102 strains of gram positive bacteria accounted for 22.5%,and the top three were Staphylococcus aureus (31 strains,accounted for 6.8%),coagulase negative Staphylococcus (22 strains,accounted for 4.9%),Enterococcus (18 strains,accounted for 4%).Meropenem,imipenem (Stenotroph-omonas maltophilia was excepted ) ,Cefoperazone /sulbactam and Amikacin were most sensitive against gram negative bacteria;Teicoplanin and vancomycin were highly sensitive against gram positive bacteria .Conclusion The main pathogenic bacteria of respiratory tract infection in ICU patients was gram negative bacilli ,which were seriously resist-ant to commonly used antimicrobial drugs .So strengthening the infection management of ICU and the control for risk factors,and rationally using of antimicrobial drugs has great significance in reducing the drug resistance of pathogenic bacteria rate .

Objective:To investigate the analysis of features and differential diagnosis of ultrasonic image for patient with endometrial lesions.Methods: The imaging data of 80 patients with endometrial lesions were selected as random number table. The relevant ultrasound manifestations and sonographic features of patients were further analyzed, and then these data were compared with the pathological diagnosis by using operation.Results: In the 80 patients with endometrial lesions, 43 cases were endometrial polyps, and in the 43 cases, there were 41 cases were consistent with the pathological diagnosis and the diagnostic accordance rate was 95.35%, while there were 2 cases were not consistent between the two methods. 33 cases of the 80 patients were submucous myoma of uterus, and there were 29 cases of them were consistent with the pathological diagnosis and the diagnostic accordance rate was 87.88%, while there were 4 cases were not inconformity. And 4 cases of the 80 patients were endometrial carcinoma, the result was completely consistent with the pathological diagnosis and the diagnostic accordance rate was 100%. In the 6 misdiagnose cases, the ratios of atypical cases and (or) typical cases were 1:16, 5:36 and 1:8, respectively. All of these endometrial lesions has the distinctive manifestations on the ultrasonoscopy of color Doppler.Conclusion: For the detection of endometrial lesions, transvaginal colar Doppler ultrasonic detection has unique feature of ultrasonogram compared with other detection methods. In the contrast, there is a regular congruent relationship between the ultrasonogram of typical disease and section of pathological specimen by using operation in a certain degree, and the ultrasonogram of typical disease has definite diagnosis value.

Objective To investigate the clinicopathology characteristics, immunopheotypic of anaplastic large cell lymphoma (ALCL) in order to improve its diagnostic and therapeutic accuracy. Methods The clinical, immunophenotypic and histopathologie features of 19 cases ALCL were retrospectively studied. The expression of CD30, ALK, CD43, CD45RO, EMA and TIA-1 was detected using EnVision immunohistochemical technique. Results All patients were primary systemic ALCL. Among the 19 patients, 11 were males and 8were females. The age ranged from 2 to 71 years, average age 26 years old. It shows a broad morphologic spectrum of the tumor cell and the hallmark cells characterized by sheets of large lymphoid cells withchromatin-poor horseshoe-shaped nuclei containing multiple nucleoli were encountered in all ALCL variants. 16patients (84.2 %) showed B-symptoms, and extranodal involvement was present in 14 (73.7 % ).Immunohistochemical study showed that the tumor cells expressed CD30 (100 %), ALK (78.9 %), EMA(52.6 %), TIA-1 (73.7 %) and 2 cases (10.5 %) neither expressed ALK, nor EMA,TIA-1.17 cases were reached the correct diagnosis according to combination of clinic, histological and immunological features.Conclusion The correct diagnosis of ALCL may be reached by combination of clinic, histological and immunological features according to WHO classification. Immunohistoehemical staining for CD30, ALK, EMA and TIA-1 is useful in making a correct diagnosis.

Objective To review the literature on quality of ife of patients with ovarian cancer. Methods Bibliometric analysis and NoteExpress2 software were used to retrieve China National Knowledge Infrastructure (CNKI) database and Wan Fang database for QOL related literature from Jan.1992 to Aug. 2014. The total of literature, published journals, the research types, the authors and the research content were analyzed. Results A total of 476 papers published in 197 journals were sorted out. The number of papers was on the rise gradually. There were 246 original papers (51.7%). A few researchers were highly productive. The co-authorship rate was 65.1%. The papers focused mainly on QOL, the status of patients′QOL and the improvement of their quality of life. Conclusions The research on QOL of ovarian cancer patients is on the rise, neither comprehensive nor deep. We should pay more attention to the field.

Objective To study the drug resistance situation of Mycobacterium tuberculosis strains isolated from the patients with HIV/AIDS complicating tuberculosis (TB).Methods One hundred and ninety-six inpatients with AIDS complicating TB in our hospital from January 2012 to December 2015 were collected.The drug resistance situation of Mycobacterium tuberculosis strains cultured from sputum/tissue fluid/fiber liquid and pathological tissue submitted samples in the patients with AIDS complicating active TB was analyzed.The BACTEC MGIT960 systems was adopted to perform the bacterial identification.The drug sensitivity test was conducted by using 960 culture testing system.Results One hundred and ninety-six strains of Mycobacterium tuberculosis were cultured,the total drug resistance rate of Mycobacterium tuberculosis was 26.02％.These cases were divided into the ＞100/μL and ≤100/μL groups according to different CD4+ T cells count.The drug resistance situation of Mycobacterium tuberculosis to 4 kinds of first line anti-TB drugs were compared between these two groups.The comparison results found that the resistance rate of Mycobacterium tuberculosis strains to the first line anti-TB drugs had no statistical difference between the two groups (P＞0.05).The resistance rates of first line anti-TB drugs from high to low were isoniazid,rifampicin,streptomycin and ethambutol.The drug resistance rate of Mycobacterium tuberculosis strains to 5 types of drug resistant TB had no statistical difference(P＞0.05).Conclusion The drug resistance rate in the initial patients with HIV complicating TB is consistent with the average initial drugresistance level of TB patients in our country.The TB drug resistance rate in the patients with HIV complicating TB has no correlation with CD4+ T cells count.

Objective To observe the distribution of the neuropeptides in mouse sublingual gland. Method Using immunohistochemical ABC method. Results The striated duct cells in the sublingual gland showed somatostatin and calcitonin gene related peptide immunoreactivity positive which were in the cytoplasma, but the nucleus negative.Conclusion The striated duct cells in mouse sublingual gland also contained neuropetides; which may play an important role in regulating acinar secretion and blood supply.

Purpose　The aim is to modify the pyrogenic pathol ogy mo del induced by injection of saccharomycetet water in rats,and to eliminate the t emperature decline period after injection of saccharomycete water sc.Metho ds　It was measured that the anus temperature of both two groups of rats (one group was injected of incubated saccharomycete water and the other inj ected unincubated saccharomycete water sc) 1,2,3,4,6,8 h after injection respect ively.Results　The anus temperature had no decline period and the temperature rose quickly in the group of injected with incubated saccharomyc ete water (in 34℃thermostasis water).There was significant difference(P＜0.05 or P＜0.01)between incubated group and unincubated group in temperature risin g by t-test.Conclusion　No temperature declining peri od was observed in the pyrogenic pathology model of rat, if those rats were trea ted with saccharomycete water which was incubated at 34℃for 0.5 h.