Hemophilia B is an X chromosome linked hereditary hemorrhagic disease, which is caused by the lose function mutation of factor IX (FIX), and significantly affects the patients' lifespan and life quality. The severity of hemophilia B depends on the FIX level in the plasma. By referring to the relevant literatures, we reviewed and summarized hemophilia B replacement therapies. Specifically, we focus on recombinant factor IX products on the market and those in the pipeline, especially on the long-acting factor IX drugs, to provide the basis for researches of new hemophilia B drugs.
Factor IX ; genetics ; therapeutic use ; Hemophilia B ; drug therapy ; Humans ; Mutation ; Recombinant Proteins ; therapeutic use

Autophagy ; Humans ; Myocardial Reperfusion Injury

Objective:To determine whether there were alteration of miRNA expression in peripheral Treg cells,through the contrast between normal people and patients with ITP and to explore the pathogenesis of ITP.Methods:Peripheral blood was obtained from ITP patients and normal people ,and Treg cells were isolated by flow cytometry.RNA was extracted from Tregs in each group,then for miRNA arrays analysis through Solexa sequencing.Compare ITP group and normal control group microarray to identify differentially expressed miRNA.Differentially expressed miRNA were analyzed to find the abnormal signaling pathways.Results: We screened multiple miRNA abnormal expression in Treg cell of ITP patients,which has significant differences were miR-1976,miR-548ae-5p,miR-5096-p3,miR-548am-5p,PC-3p-63471,and PC-3p-96627.By enrichment analysis,we found two abnormal signaling pathways:ErbB signaling pathway and TGF-βsignaling pathway.Conclusion:Differentially expressed miRNA were found in peripheral Treg cell of ITP patients,which may be affected the normal functioning immune regulation of Treg cells,and involved in the pathogenesis of ITP.

OBJECTIVE:To improve pharmacists’ consciousness, abilities and skills on pharmaceutical services and to ensure safe and effective use of drugs in patients. METHODS: With the trainning of basic theroy, basic knowledge and basic skills for the priciple to develop technique trainning in view of pharmaceutical technicians in outpatient pharmacy, it were trained or examined by means of traditional exam and operation practice with the results analyzed. RESULTS:In the examinations of drug laws and regulations and pharmacal basic knowledge, 72.8% and 36.4% of pharmacists got excellent level respectively. In the review of prescriptions, and the use of some special drugs, pharmacists masted in sufficiency. CONCLUSIONS: The new open pharmaceutical service mode in the outpatient pharmacy calls for a higher level of expertise and skills for pharmacists, thus the training in fundamental theory, basic knowledge and essential skills are significant in our hospital.

Objective To observe the effect of nursing intervention on voiding effect in patients after percutaneous renal biopsy. Methods 80 cases of patients after percutaneous renal biopsy were randomly divided into the control group and the observation group with 40 patients in each group. The control group only received general health education, while the observation group was given specialist care measures pre, during and post operation. The complication after operation was compared between the two groups. Results Postoperative complication of the observation group was lower than that of the control group. Conclusions The whole process nursing intervention can alleviate voiding effect, reduce postoperative complications, and is worthy of clinical application.

Biopsy ; Child, Preschool ; Clitoris ; pathology ; Disorders of Sex Development ; diagnosis ; pathology ; Female ; Genitalia, Female ; abnormalities ; Humans ; Hypertrophy ; Magnetic Resonance Imaging ; Neurofibromatoses ; diagnosis ; pathology ; Vulvar Neoplasms ; diagnosis ; pathology

Objective To review computed tomographic angiography (CTA) signs and its potential use in the diagnose of blunt thoracic aortic injury Methods We reviewed 37 cases with blunt thoracic aortic injury (TAI) confirmed by surgery or aortography in Chinese PLA General Hospital emergency department from June 2004 to June 2014.CTA were performed on these patients,and CTA images were analyzed retrospectively to obtain characteristic CTA signs of blunt thoracic aortic injury.Results Of the 37 cases,the blunt injury in aortic isthmus were 83.8％ (31/37),aortic arch 5.4％ (2/37),descending aorta 8.1％ (3/37),and ascending aorta 2.7％ (1/37),respectively.CTA signs of thoracic aortic injury manifested as intimal flap in 34 patients (91.8％),abnormal aortic contour or cavity in 37 cases (100％),arterial contraction in 17 cases (45.9％),aortic dissection in 10 cases (27.0％),pseudoaneurysm in 21 cases (56.7％),thrombosis in 1 case (2.7％).Conclusions CTA signs could be used to facilitate the diagnosis of blunt thoracic aortic injury with a qualitative diagnostic value.

SUMMARY When placenta previa complicated with placenta percreta, the exposure of operative field is difficult and the routine methods are difficult to effectively control the bleeding, even causing life-threatening results. A 31-year-old woman, who had been diagnosed with a complete type of placenta pre-via and placenta percreta with bladder invasion at 34 weeks gestation. Her ultrasound results showed a complete type of placenta previa and there was a loss of the decidual interface between the placenta and the myometrium on the lower part of the uterus, suggestive of placenta increta. For further evaluation of the placenta, pelvis magnetic resonance imaging was performed, which revealed findings suspicious of a placenta percreta. She underwent elective cecarean section at 36 weeks of gestation. Firstly, two ureteral stents were placed into the bilateral ureter through the cystoscope. After the infrarenal abdominal aorta catheter was inserted via the femoral artery ( 9 F sheath ) , subarachnoid anesthesia had been estab-lished. A healthy 2 510 g infant was delivered, with Apgar scores of 10 at 1 min and 10 at 5 min. Imme-diately after the baby was delivered, following which there was massive haemorrhage and general anaes-thesia was induced. The balloon catheter was immediately inflated until the wave of dorsal artery disap-peared. With the placenta retained within the uterus, a total hysterectomy was performed. The occluding time was 30 min. The intraoperative blood loss was 2 500 mL. The occluding balloon was deflated at the end of the operation. The patient had stable vital signs and normal laboratory findings during the recovery period and the hemoglobin was 116 g/L. She was discharged six days after delivery without intervention-related complications. This case illustrates that temporary occlusion of the infrarenal abdominal aorta using balloon might be a safe and effective treatment option for patients with placenta previa complicated with placenta percreta, who were at high risk for peripartum hemorrhage. Early removal of the endovascu-lar catheter and close postoperative surveillance of the vascular system are required with this procedure to minimize the risk of vascular complications. However, further studies are needed to determine whether the potential benefits of temporary occlusion of the infrarenal abdominal aorta using balloon outweigh the potential risks.

In this study, we explored the establishment of the database of Chinese medicine and minimally invasive surgery (MIS) for tumor based on the internet, thus to promote the application of Chinese medicine in the comprehensive treatment for tumor. The database was composed of general information database, Chinese medicine database, MIS database, which was characterized by the internet-based information system structure. Data exchange between net server and remote terminal can be achieved through the firewall. The results of testing performance showed that the established database can realize the real-time share of case report, and is convenient in basic retrieve and information analysis function, indicating the feasibility and stability of the database.

BACKGROUND:Skin grafting is crucial for patients with deep burns, but limited source of autologous skin grafts is an existing difficulty.
OBJECTIVE:To investigate the effect of xenogeneic (porcine) acelular dermal matrix in the treatment of deep burn wounds and the feasibility of its application.
METHODS:Forty-one patients with deep burn were divided into two groups according to the intention of the patients, 21 cases in control group and 20 cases in observational group, folowed by autologous split-thickness skin grafting alone or combined with different (porcine) acelular dermal matrix, respectively. After 12 months of folow-up, the graft success rate at postoperative 1, 2, 3, 4 weeks and skin graft contraction rate and wound repair at postoperative 3, 6, 9, 12 months were observed and compared between two groups. Moreover, levels of inflammatory factors were detected and compared between two groups at postoperative 1, 2, 3 months.
RESULTS AND CONCLUSION: The skin graft success rates showed no difference between two groups at postoperative 1, 2, 3, 4 weeks (P > 0.05). The skin graft contraction rates also showed no difference between two groups at postoperative 3, 6, 9, 12 months (P > 0.05). After 12 months of folow-up, no serious scar hyperplasia, but soft texture appeared in the control group. In the observational group, three cases presented with local pigmentation at the early stage, but it gradualy subsided with time; no obvious scar, but only smal, point-like scar, was visible, and the repaired wound exhibited soft touch. No adverse events and death occurred in both two groups. Experimental results show that the treatment of deep burns with autologous split-thickness skin grafting combined with xenogeneic (porcine) acelular dermal matrix is safe and effective, which can improve the quality of wound healing.