Objective To investigate the clinical characteristics of Kennedy disease with secondary mitochondrial dysfunction and glutaric aciduria typeⅡ.Methods The clinical data of 1 case Kennedy disease with secondary mitochondrial dysfunction and glutaric aciduria typeⅡwas retrospectively analyzed.Results The patient presented muscle weakness in proximal limbs, fasciculation, bulbar palsy, amyotrophy, postural tremor, sensory disturbance, gynaecomastia, impotency.The level of serum creatine kinase and urinary glutaric acid were elevated. Electromyogram and nerve and muscle biopsy supported mixed damage in peripheral nerves, as well as neurogenic and secondary myogenic pathologic changes in muscles.Gene sequencing indicated trinucleotide CAG repeated amplification for 47 times in chromosome X.Diabetes was diagnosed at 7 years after onset.Conclusions The pathogenesis of Kennedy disease is very complex, which may involve myogenic factor.The treatment to the secondary mitochondrial and lipid metabolic disturbance in muscle can improve the muscle weakness to a certain extent.

Objective To compare laparoscopic appendectomy (LA) with open appendectomy (OA) in the treatment of appendicitis complicating gangrene,perforation and abscess in children.Methods This study included 614 consecutively admitted patients with acute complicated appendicitis undergoing laparoscopic (267 cases) or open (347 cases) appendectomy.Clinical outcomes were compared between the 2 groups in relation to operative time,length of hospital stay,postoperative complications and in hospital cost. Results There was no mortality.Conversion to open surgery was necessary in 7 patients in LA group.Laparoscopic appendectomy was associated with a shorter hospital stay (4.6 d vs.8.1 d,P =0.00),lower incidence of wound infection(5.3％ vs.12.8％,P =0.03),less bowel obstruction(5.0％vs.10.0％,P =0.04) and nosocomial infection (9.7％ vs.18.3％,P =0.04).Intra-abdominal abscess formation was significantly higher after laparoscopic surgery (4.1％ vs.1.1％,P =0.04 ).The cost of treatment and operative time were higher in the laparoscopic group than in open appendectomy,however,there was no statistically significant difference. Conclusions Laparoscopic technique is a safe and clinically beneficial operative procedure for complicated appendicitis in children with short hospital stay,early recovery,good cosmetic appearance,and low complication rate,except for a higher incidence of intraabdominal abscess.

Animals ; Gene Silencing ; physiology ; Gene Targeting ; Genetic Therapy ; methods ; Genomics ; Humans ; RNA Interference ; RNA, Small Interfering ; genetics ; therapeutic use ; RNA-Induced Silencing Complex

Objective To evaluate the diagnostic value of combining real-time three-dimensional ultrasonography with three-dimensional power Doppler ultrasound for the patients with small ovarian masses.Methods One hundred and seven patients with small ovarian masses were examined with real-time three-dimensional ultrasonography and three-dimensional power Doppler ultrasound.The images and the relationship between benign and malignant small ovarian masses were analyzed.Results Benign and malignant small ovarian tumors had statistical differences in these three parameters:the envelope,inner wall and whether there being central vessels.The sensitivity and specificity of combining real-time threedimensional ultrasonography with three-dimensional power Doppler ultrasound to diagnose malignant small ovarian tumors were 92.9% and 97.3% respectively.Conclusions Combining real-time three-dimensional ultrasonography with three-dimensional color Doppler energy can be used to diagnose the small ovarian masses and provide valuable information to identify malignant ovarian tumors.A small ovarian tumor with incomplete envelope,irregular inner wall and there being central vessels indicated the possibility of being malignant.

Objective To understand the clinical features,differential diagnosis,treatment and prognosis of postpartum hemolytic uremic syndrome (PHUS). Methods Three PHUS cases and relevant literature were reviewed. Results Three patients were admitted because of microangiopathie hemolytic anemia,thrombocytopenia and acute renal failure.which occurred within 3 days after cesarean section.All of them received plasmapheresis and hematodialysis.Now,one of the patients recovered,and the other lives on hematodialysis. Conelusiom Early diagnosis and proper treatment of PHUS ensures a better outcome.

Objective To investigate the effects of cobalt chloride(COCl2)induced rat alveolar epithelial cell apoptosis and the significance of related apoptosis protein hypoxia inducible factor-lalpha(HIF-1 a)expression. Methods Alveolar epithelial cells were cultured in vitro and divided into four groups with 6 samples in each.Hypoxia 4 h group:added 500μmol/L COCl2 for 4 hours inducing alveolar epithelial cell hypoxia;hypoxia 24 h group:co-cultured with COCl2 for 24 hours;hypoxia 48 h group:co-cultured with CoCl2 for 48 hours;control group:no CoCl2 added.The apoptosis rates were detected by fluorescent microscope and flow cytometry.The apoptosis morphological changes of the cell were observed by transmission electron microscopy.The protein expression of HIF-la was detected bv Western Blot. Results (1)Fluorescent microscope results showed that apoptosis rate in hypoxia 4 h、24 h、48 h group[(5.83±0.76)%,(15.00±3.28)%and(51.50±3.00)%],were higher than control group[(1.50±0.50)%](P＜0.05).(2)Flow cytometry analysis reached the same results as with fluorescent microscope.(3)Typical morphological changes of apoptosis were observed in hypoxia 24 h group.(4)HIF-1α expression in hypoxia 4 h、24 h and 48 h groups[0.69±0.035,1.02±0.044 and 0.71±0.046],were higher than control group(0.21±0.026)(P＜0.01).(5)Positive correlations were found between relative amount of HIF-1αprotein and apoptosis rate by fluorescent microscope and flow cytometry analysis(r=0.484,P=0.016;r=0.713,P=0.009).Conclusions Hypoxia could induce apoptosis of alveolar epithelial cells,which may participate in the pathogenesis of hypoxia-induced lung injury.

The chemiluminescence reaction of amidopyrine-potassium permanganate with formaldehyde as an enhancer was investigated by flow injection system. A method for the determination of amidopyrine on the basis of this technique was proposed. The detection limit is 3.0×10－8 g/mL, the relative standard deviation is 1.3% (4.0×10－6 g/mL amidopyrine,n=11).The linear range is 1.0×10－7～8.0×10－5g/mL amidopyrine.　The method has been applied to the determination of amidopyrine in the antondin injection.

Objective To investigate the effect of one day method in endoscopic sphincterotomy (EST) combined with laparoscopic cholecystectomy (LC) in treatment of cholecysto lithiasis and combined.Methods A retrospective analysis was carried out for fivety-seven cases of cholecystolithiasis combined with choledocholithiasis were peformed EST combined with LC as one day method (Conducted EST firstly and then took LC within 24 hours) from September 2007 to September 2012.Results Fifty-six cases completed LC,the surgical success rate was 98.24％,transit operation rate was 1.76％.Two cases performed abdominal pain and serum amylase rised the other 1 case was transited operation because of the inflammation of triangular parts of the gallbladder and discharged after 9 days.All patients total bilirubin fell to normal level within 72 hours.Conclusion The one day method in EST combined with LC treatment of cholecystolithiasis and choledocholithiasis can effectively reduce patients trauma,achieve good effect with fewer complications and shorter recovery time.

Objective To assess the changes of right atrial size and mechanical function after radiofrequency catheter ablation in patients with paroxysmal atrial fibrillation using real-time threedimensional echocardiography(RT-3DE), and to study the correlation between the changes of left atrial(LA)and right atrial(RA) volume and function. Methods Thirty-five patients with paroxysmal atrial fibrillation were undergone radiofrequency catheter ablation (RFCA) successfully. Transthoracic echocardiography (TTE),tissue Doppler imaging(TDI) and RT-3DE were performed before, 1 month and 3 months after procedure respectively. Late systolic volume and area of RA and LA,ejection fraction(EF) of RA and LA,late diastolic peak velocity of mitral valve inflow, tricuspid valve inflow and late diastolic peak velocity of mitral annulus and tricuspid annulus were recorded. Results The 3DE images of all patients were satisfied.LA max area and 3DE LA max volume were significantly reduced at 1 months and 3 months after procedure compared with basic stage [ ( 18.8 ± 6.3) cm2 vs (21.5 ± 6.2) cm2 , (38.8 ± 17.0) ml vs (46.1 ± 20.0) ml,P ＜ 0.05]. 3DE LA EF also declined markedly at 1 month after RFCA, and restored at 3 months later compared with baseline [(41.1 ± 13.7) % vs (51.7 ± 15.9) %, (41.1 ± 13.7) % vs (45.6 ± 18.3) %, P ＜0.05]. The size and mechanical function of the right atrial after procedure were no obvious changes. There were no evidently correlation between the changes of LA and RA volume and function. Conclusions RT3DE can provide a precise method to quantify the value of atrial volume and function. The LA size and volume are significantly reduced after RFCA in patients with paroxysmal atrial fibrillation, however, the RA size and function are no obvious changes.

ObjectiveTo explore the clinical and pathological features of Hainan patients with thyroid carcinoma treated in Hainan Provincial People' s Hospital.Methods A total of 239 clinical cases of Hainan patients with thyroid carcinoma treated in Hainan Provincial People' s Hospital from 2006 to 2010 were retrospectively analyzed.Results The number of thyroid carcinoma cases between 2006 to 2008 was stable.But the cases in 2009 were 72.97％ higher than that in 2008.Female' s peak of onset age was 25 to 55 years and male was 20 to 55 years.The cases of Hant were 231 (96.65％) and the national minority were 8(3.35％ ).So ethnic composition ratio between cases and local population has a very significant difference (x2 =21.376,P ＜0.01 ).The eastern and western regions had 175 cases and 64 cases respectively,138 (78.86％) and 34(46.88％ ) cases from city respectively.Urban and rural ratio between eastern and western areas had a significant difference (x2 =4.420,P ＜ 0.05 ).The 239 cases were composed of 228 cases (95.4％) of papilarry thyroid carcinoma,7 cases (2.92％) of medullary thyroid carcinoma,both 2 cases (0.84％) of follicular thyroid carcinoma and anaplastic thyroid carcinoma.Conclusions The incidence of Hainan nationality patients with thyroid carcinoma treated in Hainan Provincial People' s Hospital has increased from 2006 to 2010,with younger trend and regional difference.Thyroid carcinoma has a difference in race and region.The rank of the rate of pathological type is papillary thyroid carcinoma,medullary thyroid carcinoma,follicular thyroid carcinoma,undifferentated thyroid carcinoma in order.