SUMMARY Lysosomal storage diseases are a group of genetic disorders that result from the defect in lysosomal function. Signs and symptoms are variable, it is difficult to diagnose this group of disease merely by the clinical manifestation. The diagnosis usually is made by measuring the activity of the corresponding enzyme. Gene mutational analysis is useful for the diagnosis of some of the lysosome storage diseases. The treatment has focused on the replacement of the defective enzyme responsible for the disease and the hematopoietic stem cell transplantation. Both of them have achieved exciting outcomes in some of the diseases.

Objective: To study psychosocial factors and immunity of patients with generalized anxiety disorder Method: 30 patients with generalized anxiety disorder (GAD), 25 patients with neurotic depression and 32 normal controls were collected and assessed with LES (life event scale), SCSQ (simplified coping style questionnaire) and EPQ (Eysenck personality questionnaire) The blood level of IL-2, and rates of NK cell and CD3+ cell of them were also measured Result: The number of negative life events and LEU of negative life events of GAD group were higher that of normal controls, but lower than that of neurotic depression group Their total life events were also more than that of normal controls They had less positive coping than normal controls, more negative coping than both normal controls and patients with neurotic depression Their N score of EPQ was higher than that of normal control and had no significant different to that of patients with neurotic depression They also had higher levels of IL-2 than that of the other two groups There was no significant difference in rates of NK cell and CD3+ cell among all three groups The level of IL-2 was negatively correlated to the number of total life events and L score of EPQ, positively correlated to the rate of NK cell Conclusion: Patients with GAD have psychosocial stress in their life, they incline to negative coping, having unstable mood and impaired immune function Psychosocial stress has influence on immunity

Objective To explore the specific features and value of spiral CT delay repeated scanning in diagnosis of cerebral schistosomiasis granuloma.Methods The spiral CT dynamil scanning material of 49 cases of intracerebral schistomiasis proved by pathology and clinic were retrospectively analyzed.Results There was no focus enhancement within ninety seconds post contialed;Focus showed a beginning enhancement was begun within the second to the fifith minutes and the peak enhancement was within the fifth to the fifteenth minutes mixed together like a ball gradually ;Focus enhancement decreased with time within the fifteenth to the twentieth minutes and disappeared after the twenty-fifth minutes.Conclusion Spiral CT delay repeated scanning had specific features in diagnosis of cerebral schistosomiasis granuloma,the diagnosis accuracy rates was 100%.

Objective To evaluate the influence of SP600125,a selective c-Jun N-terminal kinase (JNK) inhibitor,on the levels of serum total bile salt (TBA) and Bsep,Ntcp expression in the hepatic tissue of rats with ethinylestradiol induced intrahepatic cholestasis.Methods Rats pregnant for 15days were administered the subcutaneous injection of 17- b -estradiol propylene ( EE ) to modulate the ICP animal models,and be SP600125 to intervene.Testing the level of serum TBA and the expression of c-Jun,Bsep,Ntcp in the hepalatic tissue.Results The average gray values of c-Jun in the group of ICP models were significantly lower than the normal control group ( 101.05 ± 5.20 vs 118.99 ± 5.95,P ＜ 0.05 ).After the intervention of SP600125,comparing with the group of ICP models,the expression of Bsep,Ntcp in the group of SP600125 intervention were significantly higher,and this change in the high dose of SP600125 intervention group was more obvious ( low dose intervention group Bsep:0.452 ±0.031 vs 0.291 ±0.043,Ntcp:0.462 ± 0.015 vs 0.285 ± 0.021,P ＜ 0.05 ; high dose intervention group Bsep:0.568 ± 0.038 vs 0.291 ±0.043,Ntcp:0.605±0.020 vs 0.285 ±0.021,P ＜0.05),while the level of TBA in the serum was significantly lower.Conclusions Treatment with SP600125 can down-regulate the level of c-Jun/AP-1,and it may participate in the lower expression of Bsep、Ntcp in the ICP rats which were induced by 17-bestradiol.

Chemistry Techniques, Analytical ; instrumentation ; Herbicides ; analysis ; Humans ; Paraquat ; analysis

Objective To study the clinical effect of compound Yisui Shengxue Keli(YSSXKL) on treating ?-thalassemia. Methods By using self-control study method,30 cases of major and intermediate ?-thalassemia had been first treated with placebo for three months,then were treated with YSSXKL in the followed three months in Guangxi province,the high incidence area of the disease. The clinical symptoms,traditional Chinese medicine symptoms and dynamic changes of the blood levels of Hb,RBC,Ret and HbF were observed before and after each treatment. The CRF tables were filled in according to the traditional Chinese medicine symptoms of the patients and the side effects recorded. Results During period of placebo treatment,the main curtive effective indexes Hb and RBC were not obviously raised,while the RBC levels were inversely degraded after the first month of taking placebo orally(P

Objective To investigate the survival situation of thalassemia cases treated with Bushen Yisui Method. Methods The survival situation of 197 cases were investigated by telephone. Results The total survival rate of 197 cases is 87.3%, among which the thalassemia major is 65.71%, the thalassemia intermedia is 99.2%, the ?-thalassemia is 100%, the ?-thalassemia is 85.0%. 158 of 197 cases have been analyzed genotype, among which 63.16% is the survival rate of homozygote, 89.16% of the heterogenic duplex heterozygote, 100% of the heterozygote. To ?-thalassemia, the survival rate has these trends:the major

Objective The molecular basis for opiate tolerance and dependence remains poorly understood despite extensive investigation in several preparations, including the hippocampus. Recent studies have implicated that the hippocampus played a central role in opiate tolerance, dependence and withdrawal. The current study is to explore the change in guanine nucleotide binding protein-inhabitant (Gi_2) protein in primary cultured hippocampal neurons with morphine treament. Methods The hippocampus was harvested from newborn Sprague-Dawley rats. Primary hippocampal neuronal cultures of 7 days in vitro were used and divided randomly into six groups (n=6), i.e. morphine treatment 4h group (M4), 8h group (M8), 16h group (M16), 24h group (M24), 48h group (M48) and control group (C). All morphine treatment groups were treated with morphine (10?mol/L). C group was treated with saline. The G protein levels were determined with immunofluorscence and laser scanning confocal microscope (LSCM) imaging techniques. Results Gi_2 protein levels in M16, M24 and M48 groups decreased significantly compared with that in C group (P0.05). Among M16, M24 and M48 groups, Gi_2 protein level was lowest in the M48 group. Conclusion The results indicated that Gi_2 protein levels decreased significantly in primary cultured hippocampal neurons with morphine treatment, which might be a potential molecular mechanism of opioid tolerance and dependence.

Objective To investigate the changes in inhibitory guanine nucleotide binding protein Gi_2 in five brain regions of morphine addicted rats: ventral tegmental area, nucleus accumbens, prefrontal cortex, hippocampus and locus caeruleus. Methods 36 adult male SD rats were randomly divided into six groups (n=6): acute morphine dependent group, acute abstinence group, acute control group, chronic morphine dependent group, chronic abstinent group and chronic control group. Morphine dependent models were reproduced. Withdrawal syndrome was induced with naloxone 5mg/kg for 30min in rats of abstinence group. All rats were sacrificed by decapitation. Frozen sections of coronal plane of respective brain regions (ventral tegmental area, nucleus accumbens, prefrontal cortex, locus coeruleus, hippocampus) were prepared. The relative concentrations of Gi_2 protein were determined with immunohistochemical methods. Results Gi_2 proteins in acute morphine dependent group and acute abstinence group were significantly decreased compared with that of acute control group in nucleus accumbens (P

Objective To study the effect of preoperative immunonutrition on nutritional status and immunity for the patients with gastrointestinal cancer, and to evaluate whether the administration of immunonutrition could reduce the rate of postoperative complication and thus shorten the days of postoperative hospitalization. Methods Sixty patients with different gastrointestinal cancer were randomized to receive either immunonutrition (containing Arginine, RNA and ?-3 polyunsaturated fatty acids) for 7 days before surgery (EN, n=30) or a conventional diet (CONT, n=30). Variables of nutritional status and immunity, postoperative complications, infection and the days of postoperative hospitalization were measured. Results There were no significant differences in the immunological and nutritional variables between two groups before surgery. The serum concentrations of prealbumin(PALB) and transferrin(TRF) were lower than those before surgery in the CONT group on postoperative day 3 (P