Wilson’s disease is an autosomal recessive disorder of copper metabolism. The resultant accumulation of copper primarily damages the liver and brain, resulting in hepatic, neurological and psychiatric symptoms. There have been many recent studies advancing the understanding of Wilson’s disease in Asia. There are indications that the incidence of Wilson’s disease in parts of Asia may be relatively high. Many genetic studies have identifi ed various hot spots in theATP7B gene in a variety of the Asian populations. Screening of these hotspot mutations may thus be useful in confi rming the diagnosis. Despite the advances in treatment, lack of familiarity by the health care profession resulting in late diagnosis, and poor access to treatment particularly among those from the developing economies remain areas of major concern.

Background Corneal alkali burn is a common cause of corneal neovascularization(CNV).Researches demonstrated that aquaporin 1(AQP1) plays key role in neovascularization of tumor tissue.So it is speculated that AQP1 participates in pathogenesis and development of CNV.ObjectivePresent study aims to investigate the expression of aquaporin 1(AQP1) in cornea after alkali burn and explore its role in corneal neovascularization.MethodsCornea neovascularization (CNV) models were established by putting a filter paper (the size of 3 mm in diameter immersed 1mol/L NaOH solution) on cornea for 20 seconds in the left eyes of 25 adult Sprague Dawley rats.The fellow eyes were treated using the filter paper with normal saline solution as normal control group.Corneal new blood vessel was evaluated under the slim lamp biomicroscopy once per day,and area of new vessel was calculated.The animals were sacrificed and corneal samples were obtained in day 1,4,7,14,21 after corneal alkali burn.The expression of AQP1 and VEGF in corneas were detected by immunochemistry,and the relative expression level of AQP1 mRNA in rat cornea was determined using RT-PCR.ResultsThe corneal neovascularization grew at the second day and reached the largest area at the fourteenth day after corneal alkali burn.The expression of VEGF protein (gray value) in corneal sample was significantly elevated from the first day through the fourteenth day after corneal alkali burn in comparison with control group(t_(1d)=12.410,t_(4d)=29.428,t_(7d)=16.030,t_(14d)=13.321,P＜0.05),and then the expression level declined till twenty-one day without obvious difference in comparison with control group(t_(21d)=1.587,P＞0.05).The expression of AQP1 protein showed the same tendency with that of VEGF protein(t_(1d)=7.623,t_(4d)=15.293,t_(7d)=18.291,t_(14d)=14.483,t_(21d)=6.381,P＜0.05).The dynamic change expressions of AQP1 and VEGF showed a significant positive correlation(r=0.834,P＜0.05).The expression of AQP1 mRNA (A value)was weaker in control group but considerably increased after alkali burn with the peak value in the seventh day,showing a significantly difference between day 1,4,7,14 and control group(t_(1d)=3.491,t_(4d)=10.690,t_(7d)=12.936,t_(14d)=10.767,t_(21d)=8.594,P＜0.05).ConclusionThe level of AQP1 expression is markedly related to inflammatory CNV in rat cornea after alkali burn.

To clarify the effects of zinc on the proliferation and apoptosis of cultured human retinal pigment epithelia (RPE) and the expression of caspase-3 in RPE cells. The effect of Zinc on the proliferation of RPE were examined with MTT method. TUNEL method was used to detect the apoptosis of RPE cells. Caspase-3 was detected by immunohistochemistry. A concentration of zinc higher than 0.001 microM could inhibit the proliferation of RPE. And the relationship between concentration of zinc higher than 10 microM and growth prohibition rate of RPE cells was dose-dependent. All concentrations of zinc including 0.001 microM enhanced the expression of caspase-3 of RPE. But only the concentration of zinc higher than 0.01 microM could induce apoptosis of RPE. It is concluded that zinc could enhance the expression of caspase-3 of RPE cells and induce apoptosis of RPE cells. Caution should be taken when using zinc supplements for the treatment of ARMD patients without deficiency of zinc.
Apoptosis/*drug effects ; Caspase 3 ; Caspases/metabolism ; Cell Division/drug effects ; Cells, Cultured ; Dose-Response Relationship, Drug ; Pigment Epithelium of Eye/*cytology ; Zinc/*pharmacology

Air Pollutants, Occupational ; analysis ; Chromatography, Gas ; methods ; Methyl Ethers ; analysis ; Workplace

Objective To explore the effect of mirror therapy in conjunction with task-oriented training on the upper extremity function of post-stroke patients and the possible mechanisms. Methods Twenty-one post-stroke patients were randomly assigned to a treatment group (10 patients) or a control group (ll patients).The patients in the treatment group were given conventional rehabilitation therapy and mirror therapy in conjunction with task-oriented training for 4 weeks.The control group was given only the same conventional rehabilitation treatment as the treatment group and upper extremity task-oriented training for 4 weeks.Both groups trained 1 h/d,5 d/week.Upper-extremity motor ability was assessed using the Fugl-Meyer assessment (FMA) and the Hong Kong version of the functional test for the hemiplegic upper extremity (FTHUE-HK).Ability in the activities of daily living (ADL) was assessed using the Barthel Index (BI) pre-treatment and after 2 and 4 weeks of treatment. Results At the 2nd week and 4th week evaluations,the FMA,FTHUE-HK and BI scores in both groups had all significantly increased compared with pretreatment.The average FMA score in the treatment group increased significantly more than in the control group at week 2,but by the 4th week both FMA and FTHUE-HK scores in the treatment group significantly exceeded those in the control group.There was no significant difference between the two groups in the improvement of their average BI scores. Conclusions Implementing mirror therapy in conjunction with task-oriented training is efficacious.It may promote upper-extremity motor ability in patients with hemiparesis soon after stroke,but there is no definite improvement in ADL ability.

Objective To investigate the characteristics of Wegener granulomatosis in children and to improve its recognition. Methods Ten pediatric patients with Wegener granulomatosis were studied retrospectively by clinical manifestations, serum examination, pathological data, treatment, etc. Results All children had clinical evidence of upper respiratory tract and lungs involvement; kidney lesion occurred in 6 cases(6/10); joints, skin, eyes and nervous system were also involved with different degree, cANCA (PR3) showed positive in 8 cases; Pathological examination (1 kidney sample, 2 nasal mucosa samples, 2 skin samples) showed granulomatous vasculitis and crescents were found in renal biopsy; 7 cases were treated with corticosteroid plus cyclophosphamide, 1 case was treated with corticosteroid plus methotrexate, and improvement were seen in all of them. Conclusions Wegener granulomatosis in children have diverse clinical manifestations and clinical diagnosis is difficult. The upper and lower respiratory tract and kidney are most commonly involved organs. ANCA inspection is characteristic. Treatment with corticosteroid and immunosuppressive can achieve good outcome.

Objective To study the relation between phlegm-damp retention type of metabolism syndrome and microcosmic indices in order to find out the objective evidence for TCM syndrome differentiation.Methods Totally 49 cases of metabolism syndrome were observed,25 of them were phlegm-damp retention type in differentiation and 24 were non-phlegm-damp retention type.The height, body weight,waistline and blood pressure of two groups were measured,the serum leptin,insulin and blood lipid were determined, and the insulin resistance index(IRI)and body mass index(BMI)were calculated.Results The leptin,BMI,systolic pressure,FINS and IRI of the phlegm-damp retention group were all higher than those of the non-phlegm-damp retention group.Conclusion BMI, leptin,insulin,and IRI may be taken as the the index of syndrome differentiation in metabolism syndrome.

Objective To analyze the clinical and pathological characteristics of children with lupus nephritis(LN).Methods The data of 116 inpatients from Mar.2000 to Nov.2008 with LN were retrospectively analyzed.The clinical,immunochemical and pathological data were recorded.Renal tissue was observed by light microscopy and electron microscopy after HE,PAS,Masson and PASM staining according to WHO standards.Follow-up results showed complete remission,partial remission,disease activity,renal insufficiency and death.Results Of the 116 cases of LN,there were 27 male and 89 female with a ratio of male to female 1.03.3,and the mean age was(12.0?2.2) years.The incidence of nephrotic syndrome was 63.8 %(74 cases),and acute nephritis was 21.5%(25 cases).Class Ⅳ LN was the most frequent type(14 cases,50%) and classⅢ was next(25 cases,21.5%).In view of the outcome,rapidly progressive glomerulonephritis and class Ⅳ LN were the worst.LN was initially controlled in 96.5% of the patients.Relapses of LN were most caused by the intermittent treatment.Totally 32 cases showed different renal injury,and 6 cases progressed to death.Conclusions Renal biopsy is important to diagnosis,treatment and prognosis evaluation of LN.Long and regular treatment is important for children with LN.

Background The main mechanism of diabetic retinopathy (DR) is the formation of retinal neovascularization. Vascular endothelial growth factors (VEGF) is primarily factor for DR. Objective The goal of this study was to investigate the effects of angelicae sinensis decoction for supplementing blood ( ASD), a kind of traditional prescription of Chinese herbal medicine, on vascular endothelial growth factor (VEGF) level in vitreous and serum of STZ-induced type 2 diabetes mellitus. Methods Thirty-four SPF male SD rats were fed using high fat diet for 8 weeks and then the streptozocin of 25 mg/kg was intraperitoneally injected to create the type 2 diabetic models. ASD (3.6 g/kg) and 10 mg/kg Gli of 5 mi were administered intragastrically from the 4th day of high fat die though 8 weeks respectively. Other 10 matched rats were used as normal control group. The body weight, blood glucose level ,triglycerides (TG) and high density lipoprotein (HDL) were detected in 4 and 8 weeks after usage of drug. The vitreous and serum samples were obtained in the 8 th weeks for the VEGF detection by enzyme-linked immunosorbent assay (ELISA). This experiment comlied with the Regulations for the Administration of Affair Concerning Experimental Animals by State Science and Technology Commission. Results The body weight and HDL in DM group were significantly lower than those of normal control group in 4 or 8 weeks ( P＜0. 01 ), but in ASD and Gli groups ,the body weight and HDL were higher than those of the DM group (P＜0. 05 ). the blood glucose level and TG were elevated in DM group compared with normal control group (P＜0. 01 ), but those in ASD group and Gli group were decreased in comparison with DM group ( P＜0.05 ). EIISA revealed that no significant differences were found in VEGF levels between normal control group and ASD group or Gli group (P＞0. 05 ), but in DM group, the serum VEGF level (147.65±4. 55 ng/L) was reduced and vitreous VEGF level (673.45± 100. 85 ng/L) was raised,showing the considerable differences (P＜ 0.05 ). No any correlation was found between serum VEGF level and vitreous VEGF level (r=0.314,P＞0. 05). Conclusion ASD can decrease the VEGF level in vitreous,implying that ASD postpone the occurrence of diabetic retinopathy in DM rat.

Plasma exchange is a commonly used blood purification method, h includes plasma exchange,double filtration plasmapheresis and immunoadsorption.Many nervous system diseases have the antibody. Guillian-Barre syndrome is caused by antibodies of the nervous system demyelinafing disease; Myasthenia grads is caused by the acetylcholinc receptor antibodies. CIDP also has autoimmune-mediated mechanism. Plasma exchange can be apphed effectively to remove antibodies, and to quickly improve the patient' s clinical symptoms and shorten the course of disease.