Marfan's syndrome(MFS) is a relatively common autosomal dominant hereditary disorder with prominent manifestations in the skeletal, ocular, and cardiovascular systems. MFS has a high penetrance. Recent research on the pathogenesis and diagnosis and therapy of MFS has made a lot of progress. So we reviewed the advance on the relation of molecular genetics and phenotype of MFS.We discussed the molecular hasis, gene mutation and location, diagnosis, clinical features and therapy. Now the detection on the molecular level has been a very important method in diagnose MFS very early or before bom.

Poly(ethylene glycol)(PEG) coated silver nanowires has been hydrothermally prepared at a very slow rate, in which PEG was used as both a mild capping agent and a weak reductant. As-prepared silver nanowires(SNWs) have been characterized using scanning electron microscopy) and transmission electron microscope), which showed that the SNWs are usually longer than 100 μm and their diameters(each SNW has a uniform thickness) vary mainly in the range of 80～300 nm. Surprisingly, some of these silver nanowires connected) by their two ends to form some close-looped silver nanowires, which hitherto has not been reported elsewhere. The existence of close-looped silver nanowires supports the mechanism that nanowires can evolve from fusion of some nanoparticles and/or shorter nanorods. Careful TEM characterization and selected area electron) diffraction revealed that the silver nanowires kept a five-fold twinned structure, which is opposite to some reports) that a long-time incubation will lead to the disappearance of this twin structure. This work demonstrates the situation that deeper investigation is still needed for understanding the structure transformation of silver) nanowires.

Objective To investigate the CT and MRI manifestations and clinical features of liver fluke granuloma.Methods A retrospective analysis was performed for the clinical and imaging data of 5 patients with pathologically confirmed liver fluke granuloma who were hospitalized in The First Affiliated Hospital of Guangxi Medical University from January 2010 to September 2015.Results Liver fluke granuloma had slightly low density on CT plain scan,as well as a slightly low signal on T1 weighted images and a slightly higher signal on T2 weighted images of MRI plain scan.Three-phase contrast-enhanced CT scan showed delayed enhancement with mild dilatation of the intrahepatic bile duct,and normal vessels ran through the lesion.Conclusion Liver fluke granuloma is a rare disease in chnical practice.A history of eating raw fish,delayed enhancement on three-phase contrast-enhanced CT scan,and normal vessels running through the lesion all contribute to the diagnosis of liver fluke granulomas.

Objective To discuss the outcomes of repaired supraptellar bursa for preventing knee joint stiff in condylar comminuted fracture of femer after operation.Methods 25 patients with condylar comminuted fracture of femer were repaired supraptellar bursa after internal fixation.And 16 supraptellar bursa were sutured directly,9 supraptellar bursa were repaired with fat-fascia petal.All the patients were followed up for 6 to 18 months to evaluate the function of knee joints.Results The function of knee joint were evaluated according to merchan system after operation,19 patients were excellent,6 patients were good.Conclusion Repaired supraptellar bursa for preventing knee joint stiff in condylar comminuted fracture of femer after operation is an effective method.

Objective To evaluate clinical application value of DWI and reservation of liver function in patients with chronic liver disease. Methods Thirty cases of healthy control group, and 60 case group with chronic liver disease,including both 30 chronic hepatitis B and 30 cirrhosis. liver function in case group was analysed by venous blood samples. Case groups were divided into three groups according to MELD score: ＜30 group in 27 cases, 30 to 36 group in 17 cases, ＞36 group in 16 cases. All cases underwent liver magnetic resonance DWI. Among the case group, 15 cases were followed-up twice of pre- and aftertreatment. DWI images were read, ADC values of liver parenchyma were measured and standardized with the cephalospinal fluid (CSF) at the same slice. Used SPSS 13.0 for windows to treat the data, group comparison of ADC values were treated by one-factor analysis of variance, interclass comparison each other by SNK method, comparison between pretherapy and post-treatment by paired-samples t test. Results Healthy liver parenchyma of the control group is homogeneous signal on DWI. ADC pseudo-color pictures showed green on the homogeneous areas. Slightly restricted area of chronic hepatitis B showed irregular scattered patchy in DWI images, 25 cases in right and left lobes, 5 cases only in right lobe of the liver. ADC pseudo-color pictures reaveled blue region in proliferation-constrained areas. Restricted areas of the liver parenchymal become more evident, also showed an irregular liver edge in 30 cases of cirrhosis. The standarized ADC average values were: the healthy group (0. 47 ±0. 02) × 10-3 mm2/s, chronic hepatitis B group (0. 37 ±0. 03) × 10-3 mm2/s, cirrhosis group (0. 36 ±0. 04) × 10-3 mm2/s( F =97.05,P ＜0. 05).The difference between healthy group and patients group was statistically significant (P ＜ 0. 05 ). No statistically significant difference between groups of chronic hepatitis B and liver cirrhosis ( P ＞ 0. 05 ).Average ADC values of MELD score among groups of ＜ 30, 30 to 36, ＞ 36 were ( 0. 38 ± 0. 02 ) ×10-3 mm2/s,(0.35 ±0.02) × 10-3 mm2/s, (0.32 ± 0.03) × 10-3 mm2/s respectively. There is a significant difference among the three groups (P ＜0. 05). After treatment, DWI of 15 patients with chronic liver disease showed reduction in restricted areas, and the average ADC value from pre-treatment (0. 33 ±0. 03) × 10-3 mm2/s increased to(0. 38 ±0. 03) × 10-3 mm2/s, MELD score from pre-treatment 36. 01 ±6. 00 reduced to 27. 83 ± 4. 86. Conclusion DWI of chronic liver disease showed patchy diffuse restricted areas; more severe of the liver cell damage was, more worse the liver function was and lower ADC values was. ADC values increased after effective treatment, liver function recoved and diffuse restricted areas of liver parenchyma reduced.

AIM: To investigate whether hepatocyte apoptosis is contributed to liver ischemia-reperfusion (I/R) injury and the relationship between liver caspase-3 activity and hepatocyte apoptosis in cirrhotic rats. METHODS: Liver ischemia-reperfusion is induced by Pringle maneuver. The cirrhotic rats were randomized into two groups: Group A: simple hepatic blood inflow occlusion (HBIO); Group B: HBIO + inhibitor, before HBIO, ZVAD-fmk 15 mg/kg was injected via dorsal penis vein; Group C: healthy rat, simple HBIO. The ischemia time was 30 min in these groups. Serum aspartate aminotransferase(AST), liver caspase-3 activity, and apoptotic hepatocytes were examined in the three groups. RESULTS: After 6 h of reperfusion, the liver caspase-3 activity was markedly elevated and reached its peak, which was statistically higher than that of before I/R ［(18.1±1.8 ) μmol*h-1*g-1 (tissue) vs (6.6±2.0) μmol*h-1*g-1 (tissue), P＜0.01］. The same change occurred in hepatocyte apoptosis between 6 h of reperfusion and before I/R (20.9%±4.9% vs 0.5%±0.3%, P＜0.01). As the reperfusion prolonged, the caspase-3 activity and apoptotic hepatocyte decreased gradually. The 7th-day survival rate was 62.5% in group A. The serum AST, liver caspase-3 activity and apoptotic hepatocytes were significantly higher in group A than those in group B and C, representing the most severe liver injury among the three groups. CONCLUSION: Hepatocyte apoptosis is the major form of cell death in liver ischemia-reperfusion injury in cirrhotic rats. Hepatoctye apoptosis induced by I/R is caspase-3 dependent, and inhibiting caspase-3 can alleviate liver injury. The caspase-3 dependent hepatocyte apoptosis is highly contributed to the pathological phenomenon that the ischemic sensitivity of cirrhotic liver is higher than normal liver.

AIM:To investigate the role of 1,4,5-trisphosphate inositol(IP3)and Fas gene expression in apoptosis of HepG2 cells induced by quercetin.METHODS:HepG2 cells were treated with quercetin at different concentrations(including 20,40,60,80 ?mol/L)for 72 h and treated with 60 ?mol/L quercetin for 6 h,12 h,24 h,48 h and 72 h.IP3,Fas mRNA,Fas protein and apoptosis rate were assayed by IP3-3H Birtrak assay,RT-PCR,Western blotting and flow cytometry,respectively.RESULTS:When HepG2 cells were incubated with different concentrations of quercetin for 72 h,the IP3 content was lower than those in control.Fas mRNA expression,Fas protein expression and the apoptosis rate were higher than those in control.When HepG2 cells were incubated with quercetin for 6 h,12 h,24 h,48 h,72 h,the IP3 contents were lower than those in control incubated with 60 ?mol/L quercetin for 12 h.Fas mRNA expression was higher than that in control incubated with 60 ?mol/L quercetin for 12 h.Fas protein expression was higher than that in control.The apoptosis rate was significantly higher than that in control incubated with 60 ?mol/L quercetin for 24 h(P

AIM: To probe into the role of 1,4,5-trisphosphate inositol(IP3) and bcl-2 gene expression in inhibiting hepatocellular carcinoma of nude mice by genistein.METHODS: Animals with hepatocellular carcinoma were treated with genistein 1 mg?kg-1?d-1(ip) for 3 weeks.The volume and weight of tumaor were measured.IP3,bcl-2 mRNA,Bcl-2 protein were assayed by IP3- Birtrak assay,RT-PCR,Western blotting,respectively.RESULTS: The tumor volume and weight of animals treated with genistein were lower than those in control(42.7mm3?27.8mm3 vs 52.3mm3?26.5mm3,42.7mg?27.8 mg vs 91.3mg?31.4 mg).IP3 content was lower than that in control [(13.4?1.4)nmol/g protein vs(35.3?6.6)nmol/g protein].bcl-2 mRNA expression was lower in group treated with genistein than that in control(RI which was the gray degree multiply area of bcl-2 / the gray degree multiply area of ?-actin 0.48?0.02 vs 0.56?0.15).Bcl-2 protein expression was lower in group treated with genistein than that in control(RI 1.69?0.52 vs 1.37?0.48).CONCLUSION: Genistein inhibits growth of transplanted hepatocellular carcinoma in nude mouse liver by reducing IP3 production and down-regulating bcl-2 gene expression.

AIM: To investigate whether hepatocyte apoptosis is contributed to liver ischemia-reperfusion (I/R) injury and the relationship between liver caspase-3 activity and hepatocyte apoptosis in cirrhotic rats. METHODS: Liver ischemia-reperfusion is induced by Pringle maneuver. The cirrhotic rats were randomized into two groups: Group A: simple hepatic blood inflow occlusion (HBIO); Group B: HBIO + inhibitor, before HBIO, ZVAD-fmk 15 mg/kg was injected via dorsal penis vein; Group C: healthy rat, simple HBIO. The ischemia time was 30 min in these groups. Serum aspartate aminotransferase(AST), liver caspase-3 activity, and apoptotic hepatocytes were examined in the three groups. RESULTS: After 6 h of reperfusion, the liver caspase-3 activity was markedly elevated and reached its peak, which was statistically higher than that of before I/R [(18.1?1.8 ) ?mol?h -1 ?g -1 (tissue) vs (6.6?2.0) ?mol?h -1 ?g -1 (tissue), P

Objective To summerize the experience in diagnosis and surgical treatment for hilar cholangiocarcinoma in recent years.Methods 86 consecutive cases of hilar cholangiocarcinoma were surgically treated in this hospital from Jan. 1992 to Oct. 1998. Results The tumor resection rate was 37%(32/86) in this series. The postoperative morbidity and mortality rate were 47% and 7% respectively. The median survival time in palliative resection group was 16 months and the 1-year, 3- year, 5-year survival rate was 63%,21%,15% respectively. For curative resection,the median survival time was 19 months and the 1-year, 3- year, 5-year survival rate was 80%,35%,25% respectively( P =0 038). For drainage group, the median survival time was 5 months and the 1-year, 3- year, 5-year survival rate was 28%,8 4%,5% respectively.Conclusion Early diagnosis, improvement of patients′ general preoperative condition and the surgical expertise may help in increasing the curative resection rate and reducing postoperative morbidity and mortality rate, which are keys to prolong patients′ survival.