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 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 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 explore the feature and the optimal treatment of kidney stones in children associ-ated with infant formula which had been aduherated with melamine.Method Total of 47 cases of urinary stone and 31 cases of acute renal failure caused by urinary multiple obstruction in children associated with infant formula which had been adulterated with mlelamine in Beijing Children's Hospital University of Medical Science,form Jan-uary to November,2008 were retrospectively analyzed.Results Infant formula which had been adultemted with melamine may lead to kindey stones in 15 days.The mean age was 11-month and the ratio of male to female was 2.6:1.The kindey stones were usually multiple and mostly located either in the ureteropelvic junction or all through the ureter.In the view of the renal function resuming duration,there was no statistic difference among the acute renal failure group,operation group and the conservative group(P=0.683～0.846).In children with uri-nary obstruction and acute renal failure,hemedialysis or peritoneal dialysis was performed.83.9%were relieved by posting catheter through panedoseope and blood purification.100% of the patients resumed normal renal func-tion.81.3% of the patients without acute renal failure had recovered after conservative therapies such as infusion.Conclusions The patients associated with infant formula which had been adulterated with melamine were Usually little infants.Male were more vulnerable than female.Most of them had recovered smoothly and the renal function resumed normal after effcient therapies.

Objective To investigate the clinical significance of early lactate clearance rate in respect of the prognosis of patients with severe chronic obstructive pulmonary disease (COPD) . Methods A total of 108 COPD patients with respiratory failure were studied prospectively. The patients were divided into survival group ( n = 82) and the death group ( n = 26) , and the survival group further divided into the conventional treatment subgroup ( n = 55 ) and the invasive ventilation subgroup ( n = 27 ) . Arterial blood lactate, arterial gas analysis, APACHE Ⅱ score and the 6-hour clearance rate were measured before and 6 hours after treatment in 3 groups. The high lactate clearance rate group (n =71 ) was defined as a lactate clearance rate ＞ 10% , and the low lactate clearance rate group (n = 37) with a lactate clearance rate ＜ 10%. The correlation between 6-hour lactate clearance rate and prognosis in patients was analyzed. Results Compared the survival patients in invasive ventilation group with the death group, there were no statistical differences in arterial blood lactate, pH value, PaCO2, PaO2, oxygenation index and APACHE Ⅱ score before the treatment. The 6-hour lactate clearance rate in survival patients in conventional treatment group or invasive ventilation group was significantly higher than that in the death group (t =31.2, P＜0. 01; t =34.6, P＜0. 01, respectively), while there were no statistical differences in APACHE Ⅱ score and pH value between these 3 groups. Between the conventional ventilation treatment group and the invasive ventilation treatment group, there was no statistical difference in 6-hour lactate clearance rate ( P ＞ 0. 05 ) . There was no statistical difference in APACHE Ⅱ score between before treatment and after treatment in 3 groups. Mortality in high lactate clearance rate group (4. 2% ) was significantly lower than that in low lactate clearance rate group (62. 2% ) (x2 = 121.9, P ＜ 0. 01 ) .Conclusions The early (6h) lactate clearance rate could be used as a marker for evaluating the severity,therapeutic efficacy and prognosis of patients with severe COPD.

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.

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.