Objective To explore the clinical features of hepatolenticular degeneration (HLD) with hemolytic anemia as the first manifestation, and its relationship with ichthyosis.Method The clinical manifestations, course, diagnosis, and treatment of HLD combined with ichthyosis in one child who presented with hemolytic anemia as the ifrst manifestation, were retrospectively analyzed.Results In ten-year-old girl, the ifrst symptom was fatigue and jaundice, and then progressed to abdominal distension 5 days after admission. The reticulocyte percentage was 8.5%. Coombs test was negative. The K-F rings appeared on the cornea. The CT scan of Abdomen showed pleural and peritoneal effusion, abnormal substance deposition in liver and splenomegaly. There were mutations inATP7B andTGM1 genes, both of which were located on fourteenth of chromosomes. The diagnosis of HLD combined with ichthyosis was conifrmed. After symptomatic and supportive treatments such as removal of copper, prevention of the copper absorption and supplement of vitamin A, the disease was brought to remission.Conclusion HLD with hemolytic anemia as the ifrst manifestation, combined with ichthyosis was extremely rare.

Hypoxia-inducible factors 1α (HIF-1α) is the key cellular oxygen-sensitive transcription factors that could activate diverse pathways in regulating cellular metabolism,angiogenesis,proliferation and migration,enabling a cell to generate adaptive responses to a low oxygen or hypoxic environment.HIF-1 α has been shown to play an important role in the pathogenesis of multiple liver diseases.This review explores the impact of HIF1α on liver ischemia-reperfusion injury and liver transplantation as well as its mechanism.

Objective To evaluate the role of O-GlcNAc protein modification in attenuation of brain damage by glutamine in septic rats.Methods Sixty male SD rata weighing 180-240 g were randomly divided into 4 groups:sham operation group(group S,n =12),sepsis group(group CLP,n =16),glutamine group(group G,n =16),an inhibitor of O-linked-N-acetyl glucosamine transferase Alloxan + glutamine group(group G + A,n =16).Rats were submitted to sepsis by cecal ligation and perforation(CLP).Glutamine(Gln)0.75 g/kg was injected iv after CLP in group G.Gln 0.75 g/kg was injected iv and Alloxan 90 mg/kg was injected ip after CLP in group G + A.Equal volume of normal saline was given in group S and group CLP.A1 24 h afler CLP,the neural reflex score was evaluated,then rat was sacrificed.The brain was removed for measurement of brain water content,observation of histopathology and determination of O-GlcNAc-modified protein expression.Results Compared with group S,neural reflex score and brain water content were significantly increased in groups CLP,G and G + A(P ＜ 0.05).Compared with group CLP,neural reflex score and brain water content were significantly decreased in groups G and G + A(P ＜ 0.05),and the expression of O-GlcNAc-modified protein was upregulated in group G(P ＜ 0.05),Compared with groups G,neural reflex score and brain water content were significantly increased,and the expression of O-GlcNAc-modified protein downregulated in group G + A(P ＜ 0.05).There was no significant difference in O-GlcNAc-modified protein expression among groups S,CLP and G + A.Conclusion Glutamine attenuates brain damage through O-GlcNAc protein modification in septic rats.

Objective To observe the expression levels of CD174, cyclin D1 and cyclin E in gastrointestinal tumor tissues, analyze the correlation among CD174, cyclin D1 and cyclin E, and evaluate the expression level of CD174 for diagnosis and prognosis of gastrointestinal tumors.Methods Flow cytometry was used to determine the expression of CD174, cyclin D1 and cyclin E on surface of the cells which were collected from the tumor tissues and distant part beside the same tumor and suspended. The expression and topography of CD174 in gastrointestinal tissue were investigated with immunohistochemical staining.Results The expression of CD174 in tumor tissues was obviously lower than that in distal noncancerous tissues (P

Objective To understand the eco-hydraulics characteristics of Biomphalaria straminea,the intermediate host of Schistosoma mansoni. Methods The drainage method and settlement tube method were applied to measure B. straminea's den-sity and hydrostatic settling velocity respectively. Results The density of B. straminea was 1.04-1.16 g/cm3,and the average value was 1.08 g/cm3. The hydrostatic settling velocity was 2.32-12.92 cm/s. Conclusions The eco-hydraulics characteristics of B. straminea is different from Oncomelania hupensis,and more attention should be paid to the hydraulic measures for the con-trol of B. straminea.

Serum B7-H4 protein levels were measured by enzyme-link immunosorbent assay (ELISA) in 62 patients with epithelial ovarian cancer, 20 patients with benign ovarian diseases, and 30healthy subjects.Serum B7-H4 protein levels in epithelial ovarian cancer, benign ovarian diseases and normal controls were 0.72(0.32,1.88 )μg/L, (0.45±0.21)μg/L,(0.38 ± 0.19 ) μg/L respectively (P＜0.01).The serum level of B7-H4 protein in epithelial ovarian carcinoma was correlated with FIGO stages, lymph node metastasis, histological grade and histological types ( all P ＜ 0.01 ), but not with menopause and ascites (P＞0.05).The receiver operating characteristics curve analysis revealed that B7-H4protein had a sensitivity and specificity of 69% (43/62)and 86%(43/50) for diagnosis of epithelial ovarian cancer.

Objective To investigate the association between PARK16 gene polymorphism and Parkinson's disease(PD)susceptibility in Chinese Han population.and to analyze its single-nucleotide polymorphism(SNP)genotypes,frequencies and odds ratios(OR)of different genotypes.Methods The association between two SNP loci in PARK16 gene(Rs947211,Rs823128)and PD susceptibility was investigated by TaqMan quantitative polymerase chain reaction(PCR)in 226 PD patients and 362 healthy controls.Allele and genotype frequencies were calculated by the Chi-square test,and the clinical data were also analyzed.Results Three genotypes of Rs947211(GG,AG and AA)account for 34.1%(77/226),46.0%(104/226),19.9%(45/226)in the PD group,and 23.8%(86/362),53.0%(192/362),23.2%(84/362)in the control group,respectively.There was significant difference between two groups (P<0.05).Setting the GG genotype as the reference,OR values of AG and AA genotype were 0.57(95%CI0.38-0.85,P=0.006)and 0.55(95%CI 0.34-0.85,P=0.015),while the OR value for exposure to the A allele(AA+AG)was 0.56(95%CI0.38-0.82,P=0.003).Genotypes of Iate-onset PD were also significantly different from the controls(OR valne of AG=0.46,95%CI 0.27-0.78,P=0.004:OR value of AA=0.35.95%CI 0.18-0.68,P=0.002).And there was no diffefence in clinical features among the 3 genotypes. The frequency of Rs823128, another locus, in PD group was not significantly different from the control group( AA genotype as the reference, OR value of AG was 1. 12, 95% CI 0. 75-1.68, P = 0.568; OR value of GG was 0.99, 95% CI 0.35-2.76, P = 0.994). Conclusion Polymorphism of PARK 16 locus Rs947211 is associated with PD patients in Chinese Han population.

Objective To determine the frequency of depression in patients with Parkinson' s disease(PD) and healthy controls over 50 years of age and to analyze the characteristics and influencing factors of PD with depression(PDD) in Nanjing.Methods One hundred and twenty-six PD patients were diagnosed and assessed using Self-rating Depression Scale (SDS) and Hamilton Depression Scale (HAMD).The frequency,characteristics and influencing factors of depression were statistically analyzed,and the factor analysis of HAMD was carried out.Also,one hundred and twenty-four healthy subjects over the age of 50 were selected as the control group.Ressults The incidence of depression in PD group was 48.4％ ( 61/126):15.1％ (19/126) for mild depression,27.8％ (35/126) for moderate depression,5.6％ (7/126) for severe depression.The incidence of depression in the control group was 9.7％ (12/124):5.7％ (7/124) for mild depression,2.4％ (3/124) for moderate depression,1.6％ (2/124) for severe depression.There was a significant difference between these two groups( x2 =45.36,P ＜ 0.01 ).Univariate and Logistic regression analysis revealed that a high frequency of depression occurred in patients with long PD duration,high H-Y stage and UPDRS Ⅲ.According to each factor analysis of HAMD,the scores of cognitive impairment,tardiness,anxiety and sleep disturbances of the PD patients with depressive syndromes were higher than that of the control group.Conclusion Depression is a relatively common complication of PD in Nanjing which is associated with long PD duration,severity of motor disturbances and increasing H-Y stage.

Objective To investigate the change and clinical significance of the serum level of human epididymis secretory protein 4 (HE4) in elderly patients with epithelial ovarian cancer (EOC).Methods The serum levels of HE4 were determined by ELISA in EOC patients (n= 33), benign ovarian disease patients ( n = 17) and healthy women ( n = 20), all of them aged 60 years or over. And there were other 31 EOC patients and 20 healthy women aged less than 60 years at the same period as controls. Results In healthy people, the serum level of HE4 was higher in the group with age ≥60 years than with age ＜60 years [(32.25± 13. 15) pmol/L vs. (24.59±8.60) pmol/L]. The serum HE4 level had a positive correlation with age (r=0. 525, P＜0.01). The median level of HE4 was significantly higher in elderly EOC patients than in benign ovarian disease patients (81.50 pmol/L vs.45.60 pmol L, U=168.5, P＜0.05) and healthy women (81.50 pmol L vs. 33.30 pmol L, U=76.5, P＜0. 01). The HE4 had a significantly higher area under ROC curve when compared with CA125 (0. 799 vs. 0. 782), while the cut-off value was set 65.43 pmol L with a sensibility and specificity of 66.67% and 86.49% respectively. The diagnostic efficacy of HE4 was higher than CA125. There was no significantly statistical difference between elderly and non-elderly EOC patients in serum HE4 levels (U= 101.2, P＞0. 05). Conclusions Age is an important factor which can affect human serum level of HE4. HE4 determination is helpful in the diagnosis of ovarian epithelial carcinoma in the elderly patients, and the combined detection of HE4 and CA125 is an useful adjuvant diagnostic measure.

Objective To evaluate the cost-effectiveness and -benefit of the World Bank Loan Project on Schistosomiasis Control in Qianjiang City. Methods According to the national scheme the cost-effectiveness and cost-benefit of the project were analysed. Results After the project was carried out for 9 years, poputation schistosome infections rate decreased by 56.48%; cattle infections rate decreased by 48.08%; snail areas reduced 1224 hm 2(42.76%). Infected snail areas reduced 273 hm 2(17.13%). Snail infections rate decreased by 97.04%. The investment for infections rate decreased by 1% in 100 people was 1384.47 yuan. The investment for infections rate decreased by 1% in 100 head of cattle was 3386.96 yuan. The investment to reduce 1 hm 2 snail ares was 8935.63 yuan. Conclusion For one yuan investment, the benefit was 7.96 yuan and the total of the net benefit was 106784700 yuan in 9 years.