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: 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 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 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 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 evaluate the correlation between the endolymphatic hydrops in vestibule and symp‐toms ,audiovestibular functions in patients with Meniere's disease .Methods Twenty -four hours after bilateral intra-tympanic 8 times diluted gadolinium administration , three - dimensional fluid - attenuated inversion recovery MRI ,using a three-Tesla unit ,was performed in 34 patients .The extent of endolymphatic hydrops in the MRI images was assessed and compared the hydrops with age ,duration ,stage of the disease ,frequency of attacks ,pure tone audiometry ,caloric test ,electrocochleography and vestibular evoked myogenic potentials .Results Thirty -four patients showed different levels of enhancement of perilymth in the inner ears .In these patients ,obvious signs of endolymphatic hydrops were visualized ,including 8 slight hydrops ,11 mild hydrops and 15 significant hydrops . In all 34 patients ,the results of PTA were 9 dB HL to 90 dB HL ,with average of 43 .56 dB HL and there were 21 abnormal and 13 normal patients in caloric test with a diagnostic rate 61 .76% ;17 normal ,7 abnormal and 10 not elicited patients in VEMP with a diagnostic rate 50% ;22 abnormal and 12 normal patients in electrocochleography with a diagnostic rate 64 .71% .There were almost no complications in all 34 patients after bilateral intra-tympanic injec‐tion except short vertigo among 5 of them .A statistically significant correlation was found between degrees of en‐dolymphatic hydrops in vestibule and age ,stage of the disease and VEMP (P<0 .05) ,whereas no significant corre‐lation was found between the extent of endolymphatic hydrops and duration ,frequency of attacks ,pure tone audi‐ometry ,caloric test ,electrocochleography(P> 0 .05) and the correlation coefficient were 0 .494 ,0 .568 ,0 .590 , 0 .495 ,respectively .Conclusion The degree of endolymphatic hydrops in Ménière's disease has significant correlation with age ,VEMP ,stage of the disease and pure tone audiometry .No significant correlation was found between hy‐drops and duration ,frequency of attacks ,functional level scale ,caloric test and electrocochleography .

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.

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 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 observe and analyze therapeutic effects of internal fixation reconstruction with titanium plate for intra articular calcaneus fractures. Methods 48 patients (56 feet) with calcaneal fracture were treated with reconstruction titanium plate, under the monitoring of C shape X ray. The therapeutic effects were evaluated according to the Maryland foot score system. Results 48 patients were followed up for an average of 18 months. 36 feet achieved excellent results, 14 good, and 6 fair. The good and excellent rate was 89.3%. The length, height, width and Bhler angle of the calcaneus were well improved. Conclusion Open reduction and internal fixation with reconstruction titanium plate is a reliable measure to treat intra articular calcaneus fractures, as long as the surgeon has a good command of the anatomic structure of calcaneus, indication and right timing for the operation.