Cholangitis, Sclerosing ; complications ; Histiocytosis, Langerhans-Cell ; etiology ; Humans

Objective To investigate clinical phenotype and the characteristics of gene mutation of patients with spinocercbellar ataxia type 2 and type 3.Methods The trinucleotide repeat mutations were detected by polymerase chain reaction (PCR),fluorescence-PCR and capillary electrophoresis in 9 patients and 43 members from 4 spinocerebellar ataxia families,1 sporadic patients,and 60 normal controls without family history.Results Six patients from 3 families and one sporadic patient had SCA3/MJD (CAG) n expansion mutation(n=68-75) ;Three patients from 1 family had SCA2 allele expansion for 37-41 times. Some of clinical menifestations were same among patients with type 2 or 3,while they showed significant difference in age of onset ,disease devetopment and nervous system injury.Conclusion The difference of clinical feature helps to distinguish SCA3/MJD and SCA2,however genotype analysis is the only method of definite diagnosis.

Objective To preliminarily proved that the cerebellar hypothalamic pathways regulate inflammatory cytokines involved in post?stroke depression(post?stroke depression,PSD). Methods 160 male(Sprague?Dawley,SD)rats were randomly divided into sham operation group,the stroke group,PSD group,kainic acid(kainic acid,KA)damage of cerebellar fastigial nucleus group,electrical damaged decussation of superior cer?ebellar peduncle(superior cerebellar peduncle,xSCP)group. The behavioral observation were observed and expression of IL?1βand TNF?αin hip?pocampus by immunohistochemistry. Results Compared with the sham operation group and stroke group,PSD group,TNF?α,IL?1βcontent signif?icantly increased;and the factor of rats with KA group and xSCP group expression consistent with PSD group,there was no significant difference among the three groups. Conclusion The cerebellar fastigial nuclear may through the cerebellar hypothalamic pathways regulate inflammatory cyto?kines and participate in post stroke depression.

Crohn Disease ; pathology ; Humans ; Infant ; Male

Objective To determine the dietary nutritional status of rural residents in Midwestern Shandong Province,in order to improve the dietary pattern and health status of them.Method 11 987 residents,from 10 273 households,were included in this study by multi-stage randomized cluster sampling in 8 counties located in Midwestern Shandong Province.Questionnaire of Food Frequency (QFF) was applied to collect the information about the amount and frequency of food consumed by the subjects in the past year.Results The average intake of cereal,vegetable,fruit,meat,egg,milk,bean,oil and salt per reference man per day was 553.9 g,310.6 g,58.2 g, 36.3 g,50.1 g,16.7 g,34.4 g,44.8 g and 12.3 g,respectively.The average intake of energy,carbohydrate,protein,fat and dietary fiber per reference man per day was 12 095.6 kJ,451.8 g,85.6 g,78.8 g and 18.7 g as well Cereal food provides 66.1% of total energy; however,the meat provides only 6.6% of that.Carbohydrate,protein and fat amount to around 63.2%,12.0% and 24.8% of total energy, respectively.24.0% of fat intake was from animal food,while 76% of that was from vegetable food.Conclusions The intake of energy, protein,fat was sufficient for these subjects;however,the intake of vegetable,fruit,bean and meat was lower than the dietary reference intake.Moreover,oil and salt intake in these subjects was much higher than Chinese Recommended Nutrient Intakes.Thus,the amelioration of dietary pattern in rural residents will be an important task for nutrition workers in rural area of Midwestern part of Shandong Province in the future.

Adult ; Humans ; Male ; Patellar Dislocation ; congenital ; diagnosis ; therapy

Objective To evaluate the application of percutaneous nephroscopy in the treatment of upper urinary tract transitional cell carcinoma, particularly renal pelvic carcinoma. Methods From June 2006 to June 2010, eight cases (with 10 sides) of renal pelvic carcinoma received percutaneous nephroscopy tumor resection. There were six males (with 7 sides) and two females (with 3 sides) in the study group. There were six cases with solitary kidney and two cases with bilateral renal pelvic tumors. There were four cases with high-grade tumors and six cases with low-grade tumors. The age of patients ranged from 52 to 72 yrs (average 61.2 yrs). Tumor sizes ranged from 0.5 to 3.5 cm (average 2.6 cm). Patients were treated with laser or electrocautery through percutaneous nephroscopy. A ureteral stent was placed in the patients after the procedure. Chemotherapy was administered postoperatively through the nephrostomy tube. Results All the operations were successfully completed uneventfully. The operative time was 45-95 min (average 73 min), estimated blood loss was 20-300 ml (average 50 ml). No remarkable differences were found in serum creatinine levels before and after operation. After 10 to 36 mon. follow-up by CT, MRI, and ureteroscopy, one patient died of tumor metastasis and two patients had local tumor recurrence. The remaining patients had no local recurrence. Conclusions Percutaneous nephroscopy in treating renal pelvic tumor is safe and feasible. This is a better choice for the renal pelvic carcinoma patients who are unsuitable for ureteronephrectomy.

Objective To discuss the technique of laparoscopie radical cystectomy with Studer orthotopic ileal neobladder. Methods Eight men with bladder cancer who were indicated for radical surgery underwent laparoscopic cysteetomy with Studer orthotopic ileal neobladder. The ages were be-tween 51 and 69 with the mean age was 57 years. Seven cases were transitional cell carcinoma and 1 case was adenocarcinoma. A 5-port approach was employed. The first step was the bilateral pevic lymphadenectomiy including obturator lymph nodes, internal and external iliac lymph nodes. Then ventral surface of the bladder was mobilized and the bilateral endopelvic fascia was incised. Next step included the stich of dorsal vein complex and dissection of both the vas deferens and seminal vesicles. Denonvillier's fascia was incised to develop the plane between the rectum and the prostate. The urethra located in the prostatic apex was divided and transected thereby completely separating the specimen. Then the specimen was placed in a packet. Finally, a 6-8 cm lower median abdominal incision was performed, through which the specimen was extracted. Construction of the Studer pouch. A portion of terminal ileum about 45cm long was isolated approximately 20cm proximal to the ileocecal valve. The ends of the isolated ileal segment were closed to restore the bowel continuity. The distal 40 cm segment of ileum was placed in a U shape and opened along the antimesenteric border. The two medial borders were then oversewn and the bottom was folded over to form a neobladder. The ureters were anastomosed to the nonineised 5 cm portion of ileum. Finally, the neobladder was put into the abdomi-nal cavity and the anastomosis between the neck of the neobladder and the end of the remaining ure-theral was performed with interrupted suture. Results All the procedures were successful. The time of the operation was 6-8 h with the mean estimated blood loss 420(200-800)ml. Complication included 1 case of right ureteral indigitation in neobladder. The postoperative histopathology revealed 7 cases of pT2 and 1 cases of pT3. No positive margin was found. The follow-up (3 - 12 months) showed no recurrence. Conclusions The technique of laparoseopic radical cystectomy is feasible with the advantages of smaller incision and less blood loss. Studer orthotopic ileal neobladder has the ad-vantages of simple skill, antireflux, better postoperative urination.

OBJECTIVETo investigate the effects of hydrogen sulfide (H₂S) on oxidative stress and endoplasmic reticulum stress (ERS) in a rat model of diabetic cardiomyopathy (DCM).

METHODSThirty male SD rats were randomly divided into control group, diabetes group and treatment group( n = 10). Intraperitoneal injection of streptozotocin was utilized to establish a rat model of DCM. The rats with DCM in treatment group were intraperitoneally injected with NaHS solution. After treated for 12 weeks, the hearts isolated from rats were perfused on a langendorff apparatus. The ventricular hemodynamic parameters were measured. The ultrastructures of myocardium were observed using electron microscopy. The content of malondialdehyde (MDA), the activities of superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) in myocardial tissue were determined by spectrophotometry. The expressions of C/EBP homologous protein( CHOP), glucose-regulated protein 78 (GRP78) and Caspase 12 at mRNA level in myocardium were detected using RT-PCR.

RESULTSCompared with control group, the cardiac function and myocardial ultrastructure were damaged obviously in diabetic rats. In myocardial tissue, the content of MDA was increased, while the activities of SOD and GSH-Px were decreased. CHOP, GRP78 and Caspase 12 mRNA expressions were increased significantly. Compared with diabetes group, cardiac function and myocardial ultrastructure damage were improved in treatment group. The content of MDA was decreased, while the activities of SOD and GSH-Px were increased significantly. The mRNA levels of CHOP, GRP78 and Caspase 12 were increased.

CONCLUSIONH2S can protect myocardium in diabetic rats, maybe it is related to reduce oxidative stress damage and inhibition of the ERS-induced apoptosis pathway.

Animals ; Apoptosis ; Caspase 12 ; metabolism ; Diabetes Mellitus, Experimental ; drug therapy ; Diabetic Cardiomyopathies ; drug therapy ; Endoplasmic Reticulum Stress ; Glutathione Peroxidase ; metabolism ; Heat-Shock Proteins ; metabolism ; Hydrogen Sulfide ; pharmacology ; Male ; Malondialdehyde ; metabolism ; Myocardium ; ultrastructure ; Oxidative Stress ; Rats ; Streptozocin ; Superoxide Dismutase ; metabolism ; Transcription Factor CHOP ; metabolism

Objective To observe the parameters and effect of endocardial pacing by steel wire electrode cardiac puncture on heart with normal beat in living animal, and evaluate its safety.Methods After anaesthesia and thoracotomy in 6 living dogs with normal heart beat, the pericardia were excised. Steel wire electrodes with annular or hook tips were used respectively at right ventricular 4 corresponding spots to perform cardiac puncture endocardiac pacing (each dog experienced 8 times of puncture); the time from puncture to effective pacing, pacing parameter and puncture complication (time and quantity of bleeding) of each electrode at each spot were recorded. Finally, the two types of electrode completed 24 times of manipulation respectively; the data collected of the two types were compared. Results The cardiac pacing successful rates in the two groups were 100%; the time taken from the beginning of heart puncture to effective pacing in annular tip group was less than that in hook tip group, but the time difference between the two groups showed no statistical significance (s: 18.4±2.3 vs. 19.6±4.1,P > 0.05). The parameters of pacing in the annular tip group, such as operation time (s: 18.4±2.3 vs. 19.6±4.1), the threshold value of pacing (V: 2.1±0.2 vs. 2.2±0.8), the amplitude of R wave sensed (mV: 11.3±3.2 vs. 12.6±4.1) and the impedance of electrode (Ω: 674.2±89.7 vs. 668.5±101.3) were not significantly different compared with those in the hook tip group (allP > 0.05). Either after puncture or after the electrodes were taken out, the time of bleeding [after puncture (minutes): 4.4±2.3 vs. 4.5±3.1, after the electrodes taken out (minutes): 4.1±2.2 vs. 4.8±2.5] and the volume of bleeding [after puncture (mL): 2.8±2.4 vs. 3.2±3.5, after the electrodes taken out (mL): 3.3±1.7 vs. 3.5±2.6] were not significantly different between the two groups (allP > 0.05).Conclusions In living dogs with normal heart beat, the manipulation and function of endocardiac pacing by cardiac puncture with either steel wire annular or hook tip electrode are well and effective, and the performance is simple and safe without any serious myocardial injury and complication. Thus, it is helpful to quickly establish efficient endocardiac pacing in emergency cases.