Mast cells regulate inflammation and immunity.Experimentally induced abdominal aortic aneurysm in mast cells-deficient animals and animals treated with mast cells inhibitors demonstrate that mast cells are involved in the pathogenesis of abdominal aortic aneurysm via several different mechanisms.Mast cells-dependent activation of metalloproteinases and the renin-angiotensin system,contribution to smooth muscle cell apoptosis,and release of proteolytic enzymes are some key examples.Activated mast cells also contribute to neovascularization,inflammation,and atherosclerosis,all hallmarks of abdominal aortic aneurysm.Thus,we may envision that mast cells stabilizing agents,as well as leukotriene receptor antagonists and histamine receptor blockers already in clinical use for treatment of other diseases,could also be tested for their efficacy in treatment of abdominal aortic aneurysm.

Objective To evaluate the short-term effectiveness of Amplatzer device closure for congenital heart diseases.Methods Under X-ray fluoroscopy or/and transthoracic echocardiography(TTE),percutaneous puncture of the femoral artery or vein was conducted and the Amplatzer occluder was passed through the catheter.Effectiveness evaluation by transthoracic echocardiography,electrocardiography(ECG),and X-ray examination was applied at 24 hours,1 month,6 months,and every 1 year after the procedure.Results In 1 case of perimembranous ventricular septal defect(VSD),the detachment of the Amplatzer occluder to the arch of aorta was due to an inadequate small size.This patient was immediately operated with successful removal of the device through the femoral artery and later underwent a re-deployment of a larger-sized Amplatzer occluder closure successfully.The remaining 29 patients with atrial septal defect(ASD),patent ductus arteriosus(PDA),or perimembranous ventricular septal defect underwent a successful deployment of the Amplatzer occluder on one session without complications.The operation time was 20~90 min (38?16 min),the X-ray exposure time was 5~45 min(18?10 min),and the length of hospital stay was 3~7 days(4?2 days).Conclusions Amplatzer occluder transcatheter closure of congenital heart diseases has advantages of simplicity of deployment,good safety,and high success rate.This procedure is suitable for patients with secundum ASD,membranous VSD,and PDA.

Appendiceal mucinous adenocarcinoma is a rare disease.The preoperative diagnosis is almostly impossible due to the lack of typical symptoms and inexperienced surgeons.One patient with appendiceal mucinous adenocarcinoma was diagnosed successfully at the 210th Hospital of Chinese PLA,who was misdiagnosed as with periappendiceal abscess by other hospitals.The result of intraoperative frozen pathological section confirmed appendiceal mucinous adenocarcinoma.And then the patient received extended resection and effective recovery.

Objective To study the regional cerebral blood flow (rCBF) and correlated factors in patients with hepatolenticular degeneration (HLD).Methods The rCBF of lentiform nucleus,thalamus and other sites in 14 patients with HLD of cerebral type (cerebral type group) and 10 patients with HLD of non-cerebral type (non-cerebral type group) were determined by magnetic resonance-perfusion imaging technology,meanwhile 13 healthy volunteers were selected as control group,and calculated the relative regional cerebral blood flow (rrCBF) for avoiding perfusion time lag.The correlation between the clinical symptom scores,the content of urinary copper,duration and rrCBF in HLD patients were evaluated.Results The rrCBF of cerebral type group in the left and right frontal lobe,temporal lobe,lentiform nucleus,caudate nucleus,thalamus,midbrain,pons and the left hippocampus,cerebellar cortex,dentate nucleus were lower than those of control group (1.91 ±0.35 vs.2.44 ±0.64,1.80 ±0.30 vs.2.37 ±0.65,1.37 ±0.35 vs.2.14 ±0.91,1.58 ±0.52 vs.2.39 ±0.99,1.61 ±0.38 vs.2.59 ±0.74,1.52 ±0.64 vs.2.63 ±0.73,1.88 ±0.32 vs.2.61 ±0.67,1.70 ±0.40 vs.2.35 ±0.50,1.48 ±0.13 vs.2.01 ±0.59,1.46 ±0.38 vs.2.38 ±0.99,1.47 ±0.55 vs.2.02 ±0.72,1.27 ±0.48 vs.1.91 ±0.51,1.24 ±0.38 vs.1.47 ±0.29,1.25 ±0.39 vs.1.53 ±0.37,1.74 ±0.27 vs.2.40 ±0.89,1.79 ±0.50 vs.2.22 ±0.66,2.15 ±0.41 vs.2.64 ± 0.61),and there were significant differences (P ＜ 0.05 or ＜ 0.01).There were no significant differences in the rrCBF of the parietal and occipital lobe,etc between cerebral type group and control group (P ＞ 0.05).The rrCBF of cerebral type group in the left and right lentiform nucleus were lower than those of non-cerebral type group (1.61 ± 0.38 vs.1.94 ± 0.58,1.52 ± 0.64 vs.1.99 ± 0.59),and there were significant differences (P ＜ 0.05).The clinical symptom scores were positively correlated with the rrCBF of the left and right lentiform nucleus in 24 patients with HLD (r =-0.792 and-0.764,P ＜ 0.01),the content of urinary copper and duration had no correlation with the rrCBF of the left and right lentiform nucleus(P ＞ 0.05).Conclusions The rCBF of cerebral type and non-cerebral type HLD is significantly reduced,cerebral type patients have lower rCBF than non-cerebral type patients.The rCBF is correlated with the clinical symptom scores.

AIM: In vitro isolation and culture of esophageal epithelial cells are basic component of tissue-engineered esophagus. This study explored the method to culture esophageal epithelial cells for research on tissue-engineered esophagus. METHODS: The experiment was performed at experimental center of Lanzhou University from May to November 2007. Normal esophagus tissues, 2.0-3.0 cm, were harvested from patient with esophagus cancer by surgery. The informed consent was obtained from the patient. Esophageal epithelial cells for tissue engineering were obtained and passaged. The cells cultured by DMEM+F12 (1∶1) after 20 minutes, 1-4 days were observed by immunohistochemistry staining and inverted phase contrast microscope. The growth curve of cells was drawn by MTT method. RESULTS: The immunohistochemistry staining results showed that 90% of the cells were positive, which indicated the cultured cells were esophageal epithelial cells. Normal cells were big and globular, floating in the culture-medium. Cells began to adhere after 20 minutes, and most cells were polygon-like or irregular globular and adherent after 1 day; the cells began to cluster after 2 days; the cells grew at peak after about 3-4 days with abundant endochylema and large and spherical nuclear. Cells growth reached the peak after about 3 days of culture and its absorbance was significantly different compared with that on the 1st, 2nd, 5th, and 6th days (P

Objective To compare the clinical effectiveness of transcatheter domestic occluder with those of surgical closure of atrial septal defect (ASD) , and to evaluate the feasibility of transcatheter ASD closure using domestic occluder. Methods From January 2002 to December 2007,69 patients underwent transcatheter ASD closure using domestic occluder, and 123 patients underwent surgical closure were observed. The technical success rate,residual shunt rate,total complication rate,operative time,blood transfusion volume,length of hospital stay and clinical long-term results were compared. Results The technical success rate was 98. 6% in domestic occluder group,and the one unsuccessful patients underwent surgical closure in the other day. Complication included cardiac tamponade in 1 patient (1. 4%) , residual shunt in 1 patient (1.4%). All patients in surgical group were treated successfully, residual shunt in 2 patient (1. 6%), total complications were progressive hemothorax in 1, cerebral embolism in 1,pulmonitis in 2 patient,pulmonary atelectasis in 1 patient,hydrothorax in 2,and incisional infection in 5, the total complication rate were 9.8% . Long-term follow up shows that both groups had good clinical results. Conclusions Transcatheter closure of ASD using domestic occluder is an ideal procedure owing to its reliability and safety, with less complication than and same long-term results with the surgical group. It is an alternative to surgery within acceptable limits.

Objective To establish a fast and effective gene diagnosis method for Wilson's disease(WD) patients by double PCR-SSCP technology.Methods We amplificated exon8 and exon12 of ATP7B gene by double PCR from genomic DNA of 140 unrelated WD patients and 30 normal controls,then used SSCP technology to screen them.At last we identificated these patients' mutation features by direct sequencing.Results No abnormality shift was found in 30 controls.In 140 patients,we found 7 types of abnormal mobility shifts in 66 cases(47.14%).In subsequent direct sequencing,mutation rate of Arg778Leu/Gln was 37.14%(52/140),and Thr935Met was 12.86%(18/140).Conclusion Double PCR-SSCP diagnosis technology is a effective method which can improve diagnosis rate for Wilson disease.

BACKGROUND:Angiotensin II receptor antagonists have been found to exerct a stronger protective effect on bone than angiotensin converting enzyme inhibitors. OBJECTIVE:To investigate the effect of different concentrations of irbesartan (angiotensin II receptor antagonist) on the differentiation and mineralization of mouse preosteoblasts. METHODS:Mouse preosteoblast cel lines MC3T3-E1 in logarithmic phase were selected and cultured in the osteogenic induction medium containing 0 (control group), 0.001, 0.01, 0.1 mmol/L irbesartan, respectively. Ten days later, the cel differentiation was observed by alkaline phosphatase staining. The mineralization was observed by alizarin red staining after 21 days of culture. mRNA expressions of osteocalcin, alkaline phosphatase and Runt-associated transcription factor 2 in osteoblasts were detected by real-time PCR at 1, 4, 7, 14 and 21 days of culture. RESULTS AND CONCLUSION:The activity of alkaline phosphatase in al the irbesartan groups (0, 0.001, 0.01, 0.1) was higher than that in the control group (P<0.05), which was the most obvious in 0.01 mmol/L. The number and area of calcium nodules in each irbesartan group were significantly higher than those in the control group (P<0.05), especial y in 0.01 mmol/L. Compared with the control group, 0.01 mmol/L irbesartan significantly upregulated the mRNA expressions of osteocalcin, alkaline phosphatase and Runt-associated transcription factor 2 (P<0.05). These results suggest that 0.01 mmol/L irbesartan significantly promotes the differentiation and mineralization of osteoblasts.

Objective To examine the brain metabolic changes in WD patients receiving copper chelation by us?ing 1H-MRS. Method Thirty-nine patients with WD was randomly divided into four groups: non-brain type group (18 cases), brain type prior-treatment group and short-term treatment group (21 cases), long-term treatment group (20 cases) from short-term treatment group, and 20 healthy volunteers served as a control group. 1H-MRS and MRI were performed on patients on 1.5/MR/MRS system to detect these above-mentioned items before and after treatment. Result The mean of NAA/Cr was significantly lower in the left putamen and head of the caudate nucleus than in the left basal ganglion in the 39 patients with WD. The mean of NAA/Cr and Cho/Cr in the left putamen and basal ganglion was significantly lower in non-brain type group than in control group(P<0.01). The mean of NAA/Cr Cho/Cr and NAA/Cho in the left putamen,head of the caudate nucleus and basal ganglion were significantly lower in brain type group than in control group(P<0.01 or P<0.05). The mean of NAA/Cr in the left putamen was much lower in brain type group than in non-brain type group (P<0.01). The mean of NAA/Cr, Cho/Cr and NAA/Cho of short-term treatment group in the left putamen, head of the caudate nucleus and basal ganglion was not significantly different between brain type group and short-term treatment group(P>0.05). The mean of NAA/Cr and NAA/Cho in the left putamen and basal ganglion was much higher in long-term treatment group than in brain type group(P<0.01 or P<0.05). The mean of Cho/Cr in the left head of caudate nucleus were much higher after treatment compared with prior-treatment group(P<0.05). The mean of NAA/Cr in the left putamen, head of the left caudate nucleus and basal ganglion in all groups was negatively correlated with course of the disease. Conclusion There are significant differences in brain metabolism among different type of WD. The long-term but not short-term copper chelation significantly improves brain metabolism. NAA/Cr may be used as a non-invasive indicator to examine the efficacy of treatment.

OBJECTIVETo observe the clinical manifestation of 155 patients with hepatolenticular degeneration (HLD) complicated with epilepsy and the therapeutic effect of integrative Chinese and Western medicine treatment on them.

METHODSClinical manifestation of patients and its relationship with abnormalities in cranial CT and/or MRI were observed. Patients were treated by combined treatment of copper repellent with sodium dimercaptosulfonate 20 mg/kg per day by intravenous dripping, and modified Gandou Decoction (GDD) by oral intake and antiepileptics as well, after treatment for 8-10 courses, the clinical effect, copper levels in urine and serum were compared between groups.

RESULTSIn the 155 HLD patients, 96 were complicated with petit mal and 59 with grand mal. In the CT and/or MRI conducted in 72 patients, all showed abnormal images, besides such frequently met images as bilateral symmetrical basal ganglia focal lesion in 65 case-episode (90.3%) and brain atrophy of various degrees in 61 case-episode (84.7%), the massive lesions in cerebral white matter as principal, with the cortex involved, were also found in 54 patients (74%), which were mostly bilateral and symmetric or located in 2 adjecent lobes of brain, the sites of damage, in sequence of occurrence, were frontal lobe, parietal lobe, temporal lobe and callosal gyrus. Brain atrophy was found in all the remained patients without above-mentioned lesions. Abnormal EEG was shown in 29 patients (40.2%), which mainly manifested as theta wave of moderate to high potential and/or short paroxysmal spike-slow or sharp-slow complex wave evoked. The urinary copper level in patients after treatment was 34.5 +/- 21.6 micromol/24 hrs, significantly higher than that before treatment, 4.49 +/- 1.93 micromol/24 hrs (P < 0.01). And the serum copper level in patients also lowered significantly (P< 0.01). Epileptic seizure was controlled completely along with the gradually improving of extrapyramidal symptoms.

CONCLUSIONPartial seizure was the most common type of seizure of HLD patient complicated with epilepsy, the next is systemic seizure. Cerebral damage lesion and obvious brain atrophy could be the main etiological factors of HLD complicated with epilepsy, combined copper repellent therapy of integrative Chinese and Western medicine, and antiepileptics produced good clinical effect on the patients.

Adolescent ; Adult ; Anticonvulsants ; therapeutic use ; Chelating Agents ; therapeutic use ; Drug Therapy, Combination ; Drugs, Chinese Herbal ; therapeutic use ; Epilepsy ; complications ; drug therapy ; Female ; Hepatolenticular Degeneration ; complications ; drug therapy ; Humans ; Male ; Phytotherapy ; Unithiol ; therapeutic use