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.

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

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

OBJECTIVETo improve the diagnosis and treatment of penile metastasis from rectal carcinoma.

METHODSWe reported a case of penile metastasis secondary to rectal adenocarcinoma, reviewed the relevant literature, and discussed the common origins, clinical features, pathogenic mechanisms, diagnosis and treatment of this disease.

RESULTSThe patient was a 54-year-old male, with metastatic penile tumors secondary to rectal adenocarcinoma, with serious adhesion to the surrounding tissue and metastasis to the liver. As treatment, we performed colostomy to relieve voiding difficulty, followed by combination chemotherapy with oxaliplatin, 5-fluorouracil, and levofolinate. The patient died 10 months later as a result of systemic failure.

CONCLUSIONPenile metastatic malignancy has a poor prognosis. Early diagnosis and combined and individualized therapies may improve the quality of life, relieve pain and prolong the life of the patient.

Adenocarcinoma ; secondary ; therapy ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Combined Modality Therapy ; methods ; Fluorouracil ; administration & dosage ; Humans ; Liver Neoplasms ; secondary ; therapy ; Male ; Middle Aged ; Organoplatinum Compounds ; administration & dosage ; Penile Neoplasms ; secondary ; therapy ; Quality of Life ; Rectal Neoplasms ; pathology

The incidence of systemic fungal infections have increased dramatically, moreover, drug resistance including either primary (intrinsic) or secondary (acquired) resistance, becomes one of the main reasons accounting for the failure of treating invasive fungal infections in the past decades. Nowadays, clinically available antifungal drugs are limited and their combination in antifungal therapy was not effective. It is expected to be a new strategy to synergistically sensitize antifungal drugs against drug-resistant fungi by using new small molecules. Based on the study in our research group and the reported work of others, we reviewed the research of the natural products which have synergistic effect with the antifungal agents against drug-resistant fungi. This review focused on the resource, structure, pharmacological activity, and action mechanism of the compounds, as well as somewhat in common, and would provide theoretical base for seeking new drug against drug-resistance fungi.
Antifungal Agents ; chemistry ; pharmacology ; Biological Products ; chemistry ; pharmacology ; Drug Synergism ; Fungi ; drug effects

OBJECTIVETo compare the efficacy between eccentric fixation and internal fixation for treatment of intertrochanteric fractures of femur,to provide a theoretical basis for the selection of the treatment method of the intertrochanteric fractures of femur.

METHODSFrom February 2007 to January 2010,82 patients with femoral intertrochanteric fracture were treated by internal fixation including 39 cases of eccentric fixation involving 23 males and 6 females, aged from 41 to 81 years old with an average of (62.68±10.69), using the DHS or proximal femoral locking plate; 43 cases of intramedullary fixation involving 15 males, 28 females,aged from 43 to 78 years old with an average of (62.60±8.37),using PFN or PFNA fixed. The surgical incision length, operative time, blood loss and postoperative Harris score between two groups were compared.

RESULTSThe wound of two groups were primary healing without operative complications. All cases received follow-up for an average time of 18.3 months (12 to 28 months). The incision length, operative time and blood loss had a statistically significant difference between two groups (P<0.05). Harris scores of hip joint function at 1 month after operation had statistically significant difference between two groups (P<0.05), and Harris scores at 12 months after operation had no statistical significance difference between two groups. The rate of excellent and good was 89.7% in eccentric fixation group and 90.7% in intramedullary fixation group,the difference was not statistically significant (t=0.0613, P>0.05). In eccentric fixation group, there was 1 case of fracture nonunion with DHS loose and ensuing hip varus deformity. In intramedullary nail fixation group, there was no anti-rotation out,distal intramedullary nail of femoral refracture occurred in 1 case.

CONCLUSIONTwo treatment methods for the treatment of femoral fractures had a good therapeutic effect,but the intramedullary fixation had shorter operative time and less blood loss than the eccentric fixation,it prior to apply to osteoporosis and unstable femoral intertrochanteric fractures.

Adult ; Aged ; Aged, 80 and over ; Female ; Fracture Fixation, Intramedullary ; methods ; Fracture Healing ; Hip Fractures ; surgery ; Humans ; Male ; Middle Aged ; Operative Time

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.

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.

Anemia, Dyserythropoietic, Congenital ; pathology ; Child ; Humans ; Male ; Siblings