Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent inherited kidney disease,its prevalence ranges from 1 in 1 000 to 1 in 400.Two genes responsible for ADPKD, PKD 1 and PKD 2,were cloned in 1994 and 1996 respectively. Many researches have been done and great progress has been made on ADPKD.Current hot issues in this area included the structures and functions of polycystin 1 and polycystin 2,the role of cilia on polycystic kidney disease(PKD),the relationship between polycystins and the vascular abnormalities,the image evaluation of PKD,the effects of blocking RAS on slowing down the PKD progression and the treatment prospects for ADPKD. This paper focused on some issues and their implications for the diagnosis and treatment of PKD.

Objective To study the expression of extracellular matrix and polycystin-1 in ADPKD and their relation to cyst formation. Methods The expression of polycystin-1, fibronectin, laminin, type Ⅰ collagen, and type Ⅳ collagen were analysed in the normal kidney, fetal kidney and polycystic renal tissue by using immunohistochemical technique. Results The expression Of fibronectin, laminin, type Ⅰ collagen, and type Ⅳ collagen increased in polycystic renal tissue compared with normal kidney. The basement membrane lining cysts was markedly thickened. Type Ⅰ collagen was detected in the interstitium between cysts. Laminin, fibronectin and type Ⅳ collagen were localized in cyst basement membrane. The expression of polycystin-1 increased in polycystic renal tissue. The expression of extracellular matrix had significant correlation with the expression of polycystin-1. Conclusion The abnormal expressions of extracellular matrix and polycystin-1 exist in ADPKD. Abnormal expression of polycystin-1 may result in the alterations of extracellular matrix that is related to cyst formation.

Objective To explore the autocrine mechanism of hepatocyte growth factor (HGF) and its receptor c-MET distribution in autosomal dominant polycystic kidney disease (ADPKD) cyst-lining epithelial cells. Methods The concentration of HGF was examined with ELISA in ADPKD patients' cystic fluid, serum and cultured media of cyst-lining epithelial cells. The expression of HGF and c-MET mRNA and protein in cyst-lining epithelial cells was detected by RT-PCR, in-situ hybridization, Western blotting, immunohistochemical analysis and computer image analysis. Results The concentration of HGF in ADPKD non-dialyzed patients'cystic fluid was much higher than that in ADPKD patients' serum [(8. 61?0. 07)ng/ml vs (0.26?0.05) ng/ml, P

Objective: To study the influence of recombinant human hepatocyte growth factor (rhHGF) on proliferation and extracellular matrix synthesis of autosomal dominant polycystic kidney disease (ADPKD) cyst lining epithelial cells in vitro . Methods: The effects of different concentration rhHGF (0.5,1,2.5,5 ng/ml)in 48 h and the optimal concentration rhHGF of different time (24,48,72 h ) on proliferation of ADPKD cyst lining epithelial cell lines were observed by the incorporation of 3H TdR, and synthesis of collagen and laminin were respectively observed by the incorporation of 3H proline and radioimmunoassay. Results: rhHGF stimulated the proliferation of ADPKD cyst lining epithelial cells and synthesis of collagen and laminin,the optimal concentration and time of rhHGF were 1 ng/ml and 48 h. Conclusion: rhHGF can significantly stimulate ADPKD cyst lining epithelial cells proliferation and extracellular matrix synthesis in vitro . [

Objective: To study the effect of N (4 hydroxyphenyl) retinamide (4 HPR) on apoptosis of cyst lining epithelial cells in autosomal dominant polycystic kidney disease (ADPKD).Methods: The proliferation of ADPKD cyst lining epithelial cells was detected by MTT assay after stimulated by 1,2.5,5 and 10 ?mol/L 4 HPR for 24,48,72 and 96 h respectively.The effect of 4 HPR on the survival rate of cells stimulated by HGF was analyzed with trypan blue staining.The effect of 4 HPR on the apoptotic rate of cyst lining epithelial cells stimulated by HGF was detected with DNA laddering and cell staining with fluorescent dye Hoechst 33342.The expression of HGF and c Myc protein was examined by immunohistochemical staining and semi quantitative analysis in cyst lining epithelial cells stimulated by 4 HPR.Results: Compared with control group,4 HPR inhibited the proliferation of ADPKD cyst lining epithelial cells in a dose and time dependent manner.The most remarkable inhibition effect was observed by 5 or 10 ?mol/L 4 HPR for 96 h ( P

Objective:To study the effects of recombinant human hepatocyte growth factor (rhHGF) on the synthesis of extracellular matrix (ECM) and matrix metalloproteinases and tissue inhibitor of metalloproteinases in autosomal dominant polycystic kidney disease (ADPKD) cyst lining epithelial cells.Methods:The synthesis of laminin (LN) and aminoterminal propeptide of type Ⅲ procollagen (PⅢNP) in ADPKD cyst lining epithelial cells stimulated by rhHGF was examined with radioimmunoassay.The synthesis of type Ⅳ collagen (ColⅣ) was analyzed with ELISA.The mRNA expression of transforming growth factor ? 1 (TGF? 1),matrix metalloproteinases 2 (MMP 2),tissue inhibitor of metalloproteinases 1 (TIMP 1) and TIMP 2 in rhHGF stimulated cyst lining epithelial cells were detected by RT PCR.MMP 2 protein expression was examined with Western blotting and MMP 2 activity was analyzed by gelatin zymography in supernatant of cyst lining epithelial cells stimulated by rhHGF.Results:In rhHGF group and rhHGF+ anti TGF? 1 antibody group,the synthesis of LN and ColⅣ were markedly increased.There was no significant difference in the synthesis of LN and ColⅣ between the 2 groups.Among control group,rhHGF group,rhHGF+ anti HGF antibody group and rhHGF+ anti TGF? 1 antibody group,no significant difference in the synthesis of PⅢNP was found.No significant difference was found in the expression level of TGF? 1 mRNA in cyst lining epithelial cells among control group,rhHGF group and rhHGF+anti HGF antibody group.Compared with control group,MMP 2 mRNA expression in ADPKD cyst lining epithelial cells was significantly increased and TIMP 1 mRNA and TIMP 2 mRNA expression were significantly decreased in rhHGF group.Furthermore,MMP 2 protein expression and MMP 2 activity in supernatant of cyst lining epithelial cells also greatly increased.Conclusion:HGF stimulates the synthesis of LN and ColⅣ.HGF up regulates MMP 2 expression while HGF down regulates TIMP 1 and TIMP 2 expression in ADPKD cyst lining epithelial cells.All these changes may involve in the initiation and progression of ADPKD cysts.

Objective:To investigate the effect of mycophenolate mofetil(MMF)on proliferation and apoptosis of cyst-lining epithelial cells in patients with autosomal dominant polycystic kidney disease(ADPKD),and to compare its effect with that of rapamycin(RAPA)in vitro.Methods: Primary cultured cyst-lining epithelial cells were treated with MMF and RAPA at different concentrations(0,0.005,0.05,0.5,5 ?g/ml)for 48 h or 72 h.The inhibitory effects of them on the cells were evaluated by MTT assay;the cell cycle distribution and apoptotic ratio were determined by flow cytometry.The morphological changes of cyst-lining epithelial cells were observed under transmission electron microscope.Results: Both MMF and RAPA significantly inhibited the proliferation of cyst-lining epithelial cells in a dose-and time-dependent manner.After 48 h treatment,the cells were blocked at S phase by MMF and at G0/G1 phase by RAPA.Both drugs induced cell apoptosis,with the maximal apoptotic rate being(5.53?0.27)% for MMF and(4.36?0.10)% for PAPA.Typical morphological changes of apoptotic cells were observed under electron microscope.Conclusion: MMF can effectively inhibit proliferation and induce apoptosis of cyst-lining epithelial cells,but its inhibitory effect is weaker than that of RAPA.

Peripheral arterial disease(PAD)is a common manifestation of systemic atherosclerosis associated with significant morbidity and mortality.The goals of management for patients with PAD are to relieve exertional symptoms,improve walking capacity,improve quality of life,and prevent and retard the progression of systemic atherosclerosis.These patients should be candidates for aggressive secondary prevention strategies including tobacco cessation,physical activity,dietary modification,weight maintenance,blood pressure control,cholesterol control,antiplatelet therapy and angiotensin-converting enzyme inhibitor therapy.This article reviews the current non-intervention treatment of patients with PAD.

Objective To investigate the correlation of serum sFasL level with carotid arterial intima- media thickness(IMT) and some known markers of inflammation, nutritional status in uremic patients with cardiovascular disease(CVD). Methods 134 uremic patients on hemodialysis were divided into two groups, CVD group (n=103) and non-CVD group (n=31). The serum level of sFasL, C-reactive protein (CRP), IL-6, TNF-? and albumin were measured by enzyme-linked immunosorbent assays (ELISA). The expression of Fas and FasL in radial arterial endothelial cells were observed both by immuohistochemical stain and by reverse transcription- polymerase chain reaction (RT-PCR). The Carotid arterial IMTs were measured by Color doppler ultrasonography. Results Compared with the non-CVD group, CVD group showed significantly increased serum sFasL, CRP, IL-6, and TNF-?, and decreased serum albumin(P

Objective: To study the application of glucose infusion test (GIT) in assaying the vascular access recirculation rate in hemodialysis patients. Methods: Access recirculation was assayed by both urea test (UT) and GIT in 82 hemodialysis patients, and 17 patients were also examined by Doppler ultrosonics method. The results of the Doppler ultrosonics were compared with those of UT and GIT. Results: Thirty-seven (45.12%) patients showed positive results with GIT and 29 patients (35.36%) with UT.All 17 patients had recirculation confirmed by Doppler ultrosonics and all had positive results by GIT (100%), but only 9 (52.94%) of the 17 patients had positive results by UT. Conclusion: Comapared with UT, GIT is more sensitive, more ecnomical,and simpler in determining vascular access recirculation, and can be used as a new method for screening vascular access recirculation.