Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Male ; Mucopolysaccharidosis II ; genetics ; Pedigree

Objective:To investigate the effect of free fatty acid on leptin receptor in rats.Methods:High plasma free fatty acid rat model was created by intravenous injection of free fatty acid. Western blot was used to assess the protein level of leptin-receptor in rat liver, muscle and pancreas tissues. The RNA expression level of leptin-receptor was examined by RT-PCR .The tyrosine phosphorylation of leptin-receptor was examined by immuno-precipitation. Results:The protein and RNA levels of leptin-receptor in liver,muscle and pancreas tissues of high plasma free fatty acid rats were similar to those of normal group(P>0.05).But the tyrosine phosphorylation of leptin-receptor significantly downregulated in liver and muscle tissues as compared with control group(P<0.05). Conclusions:High plasma free fatty acid may affect the tyrosine phosphorylation of leptin-receptor in rat liver and muscle.

Objective To investigate the mechanism of Chinese herb in preventing recurrence of ulcerative colitis. Methods One hundred and three UC patients were divided into treatment group and control group randomly. The treatment group was treated by Chinese herb and the control group was treated by Sulfasalazine. After treated for three months, fifty three full remission patients were followed-up. CD4+, CD8+ and CD3+ cell count were performed and influence of Chinese herb on recurrence was observed. Results Either the cell count of CD3+, CD4+ and CD8+ or the percentage of CD8+ in the peripheral blood was significantly higher in recurrence group than those in the control (P

The culture conditions of Saccharomyces cerevisiae sp.strain by 1.1 b were optimized for the production of D-(-)-mandelate dehydrogenase which is useful for the asymmetric bioreduction of benzoylformate to form D-(-)-mandelate.The optimum medium(per liter)consistes of 60 g peptone,30 g maltose, 0.5 g MgSO_4,0.01 g ZnSO_4,1.0 g KCl.After optimization of the culture medium,the enzyme production in shake flasks is enhanced from 2.56 to 20.21 U/L.The optimum fermentation conditions were determined as follows:medium volume 100 mL(i.e.,40%for a 250-mL shake flask),pH 6.5,inoculum size 10%,temperature 30℃,and cultivation time 25 h.

Objective To investigate the emotional problem of attention deficit and hyperactivity disorder(ADHD).Methods(Seventy) ADHD children and 45 controls were evaluated with the screen for child anxiety related emotional disorders(SCARED) and depression self rating scale for children(DSRSC).Results Children with ADHD showed significantly higher somatization/panic,general(anxiety),separated anxiety,scholasstic phobia and anxiety total score,depression total score(all P

There are some problems with this course of medical laboratory special English, such as reference materials, teaching contents, teaching ways, the evaluation forms, etc. The department of medical laboratory of North Sichuan Medical College did exploratory reforms including 1+n teaching mode (one major teacher and several co-operational teachers in discussion section), Flipped classroom and interactive teaching, new formative assessment forms (usual performance combing final shows grade), etc. They used innovative teaching way with the purpose of establishing new teaching way , cultivating abilities of au-tonomous learning and comprehensive application.

Objective To determine whether normal-appearing cervical spinal cord in patients with amyotrophic lateral sclerosis (ALS) has abnormal changes based on the quantitative measurement in healthy volunteers. Methods Conventional MRI and axial DTI were obtained in 16 patients with ALS (ALS group) and 16 age-matched control subjects (normal group) . ADC, fractional anisotropy (FA) and relative anisotropy(RA)imagcs were obtained on workstation (AW4. 2). ROIs (5 mm × 5 mm) were placed in anterior funicalus, posterior funiculus, and bilateral lateral corticospinal tracts (LCTs), respectively, at the same slice (C3). Independent-sample t test was used for comparison of parameters between the two groups. Correlations between DTI parameters of ALS and ALS course, Norris score, and ALSFRS were carried out separately by Pearson correlation analysis. Results FA and RA values of bilateral LCTs were decreased significantly. FA/RA values of left LCT were 0.762±0.089 and 0.762±0.107 in ALS group, while they were 0.863±0.098 and 0.890±0.105 in control group, respectively. FA/RA values of right LCT were 0.751±0.065 and 0.772±0.082 in ALS group, and they were 0.843±0.118 and 0.863±0.134 in control group, respectively, they were decreased significantly (t = 2.575、4.195、2.246、2.218, P < 0.05). There were no significant differences (t = - 1.319, - 1.087, P > 0.05) between ADC values of left and right CSTs in ALS group [(0.744±0.162) × 10~(-3), (0.767±0.141) × 10-~(3) mm~2/s] and control group [(0.640±0.149) ×10~(-3), (0.643±0.168) ×10~(-3) mm~2/s)]. FA, RA and ADC values of ALS patients in anterior funiculus were 0.637±0.113, 0.622±0.138, (0.950±0.354)×10~(-3) mm~2/s, in control group they were 0.670±0.117, 0.656±0.136, (0.865±0.238) × 10~(-3) mm~2/s, there were no significant differences (t = 0.854, - 0.704, - 1.155,P > 0.05). FA, RA and ADC values of ALS patients in posterior funieulus were 0.886±0.073, 0.920±0.100, (0.613±0.137)×10~(-3) mm~2/s, in control group they were 0.906±0.078, 0.914±0.135, (0.636±0.224) × 10~(-3) mm~2/s, there were no significant differences (t = 1.655, - 0.148, - 1.360; P > 0.05). No significant correlation existed between FA and RA values and disease course, Norris and ALSFRS score (P > 0.05), in left and right LCTs. Conclusion DTI with SE-EPI technique is simple and sensitive to detect the pathological changes of the cervical spinal cord in ALS patients. DTI can reveal the abnormalities which are "normal appearing" on conventional T_2WI.

Objective To evaluate a somatostaitn analogue (Octreotide) in the treatment of hepatocellular carcinoma (HCC). Methods In this study 62 HCC patients were divided into therapy group (30 cases) and control group (32 cases) based on patients' own will. Patients in treatment group were assigned to receive an average dosage of 339.43±165.53 mg of octreotide. Treatment results and patients' life quality were evaluated on 3rd and 6th month. Result (1) The average live time of the treatment group was (12.89±6.21) months much longer than that of the control group (5.36±6.36) months (P < 0.05). The 6 month, 12 month survival rate of treatment group (73.3% ,50.0% ) was better than that of the control group(40.6% ,9.37% ) (χ2 =4.02 ,χ2 =9.67,all P <0.05). (2) Appetite was improved in 21 patients, body weight increased in 12 patients. Debility ameliorated in 17 patients in therapy group. (3) Tumor grew larger in 8 cases in control group on the sixth month based on liver CT and 6 of them had extrahepatic metastasis. While in therapy group tumor size decreased in 6 cases; did not change in 9 cases; 3 grew larger, and 1 had extrahepatic metastasis. (4) As for side-effects of octreotide therapy, 6 patients had diarrhea at the beginning, as the treatments continued, the symptoms disappeared within one month. Conclusion Octreotide prolongs the surviving time of patients with hepatocellular carcinoma, increases the 6 and 12 months survival rate, causing mild side-effects, and improves the life quality of patients with hepatocellular carcinoma.

Objective To present the development of the Naming test tool ( Huashan Naming Test, HNT)for Chinese cultural and by amnestic mild cognitive impairment ( aMCI ) and mild Alzheimer' s disease (AD) between the detection of memory decline,and to analyze the validity of its trial.Methods 100 normal elders from communities in Shanghai, 100 patients with amnestic mild cognitive impairment (aMCI), and 95 patients with mild Alzheimer's disease (AD) who received an education of junior high school or above and were evaluated by neuropsychological tests including mini mental state examination ( MMSE), auditory verbal memory test, Huashan Naming tests etc.8 cognitive tests.The groups of MCI and AD patients finished cranial MRI.100 items with HNT including 20 animals,10 vegetables,10 fruits ,20 tools ,20 household , 10 vehicles, 10 stationery.Results 1.HNT items to determine: 22 items were excluded due to the completion of the three groups were not significantly different; 8 items were excluded due to the completion of the normal elderly group was lower than 75%; 10 items were excluded from the analysis of variance Fvalue of the minimum value.The remaining 60 items,according to the size of the arrangement and completion rates were divided into two versions of odd and even, respectively HNT-Ⅰ and HNT-Ⅱ.2.HNT characteristics:in normal elderly group age, sex were found to had no significant factors affecting overall scores of HNT-Ⅰ and HNT-Ⅱ but level of education, MMSE score was significantly correlated (P ＜0.05).As cut-off score ≤ 26 for spontaneous naming of HNT,the sensitivities of HNT-Ⅰ for aMCI ,mild AD were 44%, 84% respectively, specificities were all around 84%; the sensitivities of HNT-Ⅱ for aMCI , mild AD were 56% ,83% respectively,specificities were all around 80%.Conclusion HNT is a Chinese cultural background,time-consuming short and good name validity test,and it is worth further promoting the application.

Objective:Since malignant fibrous histiocytoma (MFH) may be taken as an undifferentia-ted pleomorphic sarcoma (UPS), this study was conducted to reassess 33 previously diagnosed MFH cases in the past 10 years based on the latest WHO concept. And then to search for the clinicopathological features, probably tumorigenesis, and the line of differentiation of the remaining MFH/UPS cases.Methods: Thirty-three cases in tissue microarray were studied by immunohistochemistry with panels of neurogenic, myogenic, and lipogenic antibodies. Three expertise pathologists reevaluated the slides separately. Results: Among the 33 cases, 17 cases (51.5%) of MFH had their diagnoses changed, including 5 leiomyosarcomas, 3 malignant peripheral nerve sheath tumors, 1 fibrosarcoma, 1 inflammatory myofibrosarcoma, 1 giant cell tumor and 1 angiomatoid fibrous histiocytoma. The remaining 16 cases (48.5%) were finally diagnosed as MFH/UPS, among which patients were mainly old adults (median age: 63 years; range: 38 to 76 years). The median tumor size was 6.0 cm (range: 3.0 to 14.0 cm), 8 cases (50%) located in lower limb and 5 cases (31.3%) located in thigh. These tumors had marked cytological and nuclear pleomorphism. Immunohistochemistry showed that Vimentin was strongly positive in all 16 MFH/UPS (100%), Muscle-specific actin was variously positive in 8 cases (50%) and 1 case focally expressed Desmin. Eleven cases (68.8%) variously expressed CD68 (KP1) and 7 cases (43.8%) expressed CD68 (PG-M1), which were much higher than leiomyosarcoma, malignant peripheral nerve sheath tumor and liposarcoma with significant difference. Moreover, Ki67 expression rates were from 10% to 100%, including 14 cases more than 50% and 11 cases more than 70%. However, only 2 cases (12.5%) showed P53 positive. Conclusion: MFH/UPS often show marked histological pleomorphism, and the diagnosis must be made by exclusion of other definitive sarcomas, especially myogenic and neurogenic sarcoma. Only Vimentin was always expressed in MFH/UPS, while some of the tumors were positive for myogenic antigen and CD68. It was suggested that MFH/UPS might arise from primary mesenchymal cells, and some cases exhibited fibroblastic and/or myofibroblastic features. In addition, histiocytic phenotypic marker did have more expression in MFH/UPS than in other sarcomas. MFH/UPS still had certain clinicopathological characteristics.