Hemophilia is a common pediatric hereditary bleeding disorder,including hemophilia A (FⅧ deficiency) and hemophilia B (FⅨ deficiency).Children with hemophilia tend to spontaneous bleeding or bleeding after minor injuries or surgery.In this review,we briefly discussed the preoperative diagnosis and classification of hemophilia,dynamic monitoring of FⅧ or FⅨ,dose of clotting factor and duration of treatment and some other perioperative issues when surgery is needed,to guide hemophilia children's surgery and postoperative rehabilitation.

Objective To evaluate the clinical results of limited Smith-Petersen approach in the treatment of posterior dislocation of hip with femoral head fracture (Pipkin fracture of types Ⅰ and Ⅱ).Methods From February 2008 to July 2013,21 patients with Pipkin fracture of types Ⅰ and Ⅱ were treated at our department.They were 16 men and 5 women,with an average age of 39.2 years (range,from 19 to 56 years).The left hip joint was affected in 8 cases and the right in the other 13.The interval between injury and surgery varied from 2 to 7 days (mean,4 days).According to the Pipkin classification,there were 11 cases in type Ⅰ and 10 in type Ⅱ.All the fractures were fixated via the limited Smith-Petersen approach.Results The patients were followed up for 11 to 29 months (mean,17.4 months).All the fractures healed after an average of 15 weeks (from 12 to 19 weeks).According to their Thompson-Epstein scores of clinical and radiological outcomes at the final follow-ups,7 cases were excellent,9 good and 5 fair,giving a rate of excellent to good of 76.2％.Avascular necrosis of the femoral head occurred in 3 cases and osteoarthritis in 2.No infection or limb deep vein thrombosis was observed.Conclusion For Pipkin fractures of types Ⅰ and Ⅱof,limited Smith-Petersen approach can provide satisfactory exposure which leads to reduced soft tissue injury,better reduction and fixation,and fewer complications as well.

Objective: The study was to exlpore the nutritional, antioxidative functions and toxicology of ascorbic acid in different levels in vitro. [WT5FZ]Methods: Hela (human transformed epithelial) cells incubated with three levels of ascorbic acid, i.e. 0.1 mmol/L, 0.25 mmol/L and 0.5 mmol/L, were calculated and a single cell gel electrophoresis (SCGE) was used for measuring DNA oxidative damage. [WT5FZ]Results: The results showed that there were no differences in spontaneous DNA damage of Hela cells incubated with three levels of ascorbic acid. However, there was a less DNA oxidative damage induced by H 2O 2 in 0.1 mmol/L and 0.25 mmol/L of ascorbic acid supplemented groups respectively than in control group. In contrast, more serious DNA damage was found in 0.5 mmol/L ascorbic acid supplemented group. [WT5FZ]Conclusion: It is suggested that the higher levels of ascorbic acid might not directly damage DNA; the moderate supplementation of ascorbic acid may increase antioxidative ability of cells; excess ascorbic acid is harmful to DNA and enhances the susceptibility to H 2O 2 potentially.

Objective To study the inhibitory effect of radicamine A on ?-glucosidase and the glucose's absorption by small intestine.Methods Inhibitory effect of radicamine A was studied both in ?-glucosidase inhibition experiments and by a valgus cyst model of small in testine in vitro.Results Radicamine A inhibited ?-glucosidase and glucose's absorption in a dose-dependent manner(P

Objective To investigate the clinical efficacy of paraspinal muscle gap approach in the short segment internal fixation of thoracolumbar fractures.Methods Forty-five patients with thoraco-lumbar fractures but without spinal decompression underwent posterior short segment internal fixation,in-cluding 20 cases via paraspinal muscle gap approach(group A)and 25 cases via traditional posterior mid-line approach.Operative time,intraoperative blood loss,postoperative drainage,visual analogue scale (VAS)score,correction rate of Cobb angle and correction rate of vertebral collapse were recorded and the surgical results were compared between the groups.Results All patients got bony union without internal fixation loosening,broken nails or broken rods.There were significant differences in operative time [(88 ± 17)min vs(105 ±14)min],blood loss [(121 ±24)ml vs(230 ±31 )ml]and postoperative drainage [(66 ±28)ml vs(250 ±45)ml]between group A and B respectively(P<0.05).The differences in cor-rection rate of the Cobb angle [(82.3 ±1.58)% vs(83.5 ±3.71)%],correction rate of the vertebral collapse [(88.22 ±3.18)%vs(87.19 ±2.16)%]and postoperative VAS score were not significant be-tween group A and B respectively(P>0.05 ).Conclusion Internal fixation via paraspinal muscle gap approach has the advantages of less trauma,simple approach,short operative time,less blood loss,quick recovery after surgery.It is consistent with the modern concept of minimal invasion and worthy of sprea-ding.

Objective:To study the medicine storage management mode in our hospital. Methods: The medicine sales and inventory data were obtained from 2010 to 2013 by the HIS of our hospital. Medicine storage turnover ratio was calculated by using Microsoft Excel. The efficiency in the inventory management was evaluated by medicine storage turnover ratio. The storage control in our hospital was analyzed by the relevant references. Results: The medicine storage turnover ratio from 2010 to 2013 in our hospital was improved to 20. 06 times, the turnover days was reduced to 18 days which met the criteria for the review of the grade A class 3 hospital. Conclusion: The medicine storage management should be scientifically adjusted on the basis of the clinical characteristics and the use of medicines in the hospital. In this way, the improvement of medicine storage turnover ratio, the optimized control of the medicine stocks and the improvement of the social and economic benefit of the hospital will be realized.

Objective To investigate the effects and mechanisms of adrenomedullin(ADM)synthesized in alveolar macrophages in patients with chronic obstructive pulmonary disease.Methods The bronchoalveolar lavagae fluids(BALF)were collected by bronchoscopy in 15 cases of COPD and 14 healthy cases.The expression of ADM in plasma,BALF and the culture supernatants of alveolar macrophages(AMs)were analysed by radioimmunoassay.Results (1)The cells,PMNs and AMs in BALF of COPD group were significantly higher than those of healthy group(P

Objective To investigate the association of anti-C1q antibody with disease activity and lupus nephritis in patients with systemic lupus erythematosus(SLE). Methods The serum Level of anti-C1q antibody was determined in 35 patients with SLE by ELISA. The correlation between anti-C1q level and other disease activity parameters, such as SLEDAI score, anti-dsDNA antibody, C3, C4 and CH50 levels, was analyzed. Results Anti-C1q antibody was positive in 51.4% of patients with SLE, the positive rates in patients with active nephritis and without one was 77.8%(14/18) and 42.3%(22/52), respectively, and that was significantly higher in the former than in the later(P

Objective To study the diagnosis, therapy and prevention of bile duct carcinomas in choledochal cysts. Methods We retrospectively analysed clinico pathologic data, the ways of operation and living time of 11 patients with bile duct carcinomas in choledochal cysts in two hospitals from 1986 to 1997. Results There were complete excision of the choledochal cysts associated with choledochojejunostomy in 4 patients, exploration of bile duct and drainage with T shape tube in 3 patients. And there were also the excision of the wall of bile duct cysts and the tumor and cholangioplasty, choledochocystoduodenostomy, biopsy, pancreaticoduodenectomy and cholangiojejunostomy at the porta hepatis, resection of original rhynchostoma of choledochojejunostomy in one patient, respectively. One patient has lived for 3.6 years after radical resection. Others lived for 13.6 months after operation. Conclusions It is the key of improving and lengthening lives to find this disease in the early stage and to resect radically as early as possible.

Aneurysmal bone cyst of mastoid bone is seldom, here one case was reported. The mastoid bone of the patient presented with a baloon-like swelling full of non-coagulated blood and serous-hemorrhagic fluid. CT scan demonstrated a large expansile destructive mass located in left mastoid bone region with the thin or absent cortical bone. The MRI demonstrated T2-weighted images and clear boudary from surrounding tissue. Pathologic reported that the mastoid bone was repalcement with lacunar divided by fibro-tissue, containing numerous hemosiderin, giant cells and inflammatory cells. A surgery was performed and the patient was cured.
Bone Cysts, Aneurysmal ; diagnosis ; pathology ; surgery ; Humans ; Magnetic Resonance Imaging ; Mastoid ; pathology ; Tomography, X-Ray Computed