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 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.

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 The aim of this study was to evaluate magnetic resonance cholangiopancreatography(MRCP)using half-Fourier acquisition single-shot fast spin-echo(HASTE)sequence in the diagnosis of biliary obstructions.Methods Forty-five patients with pancreaticobiliary duct diseases underwent MRCP on 1.5 T scanner,a heavily T 2-weighted HASTE was used during a breath-hold.The source images were three-dimensional reconstructed postprocessed on workstation.The findings of MRCP images were analyzed and compared with US,CT,ERCP or PTC.Results The diagnostic accuracy of MRCP was 94%,the same as ERCP(92%),but it was superior to US and CT(?

Objective To study the diagnosis and treatment of infected pseudoaneurysms(PA) resulting from drug injection. MethodsThe clinical data of 11 cases of i nfected PA resulting from drug injection were reviewed retrospectively.1 pat ient was treated by non-operation,10 cases were treated by artificial blood ve ssel transplantation after removal of PA and local debridement. Results One case dead. All the 10 cases treated by operation had satisfactory results and none had ischemic symptom of the limbs.Eight grafts were patent d emonstrated by color Dopler ultrasonography 3 months postoperatively. Conclusions The diagnosis of PA induced by drug injection can be m ade by the history of drug injection, pulsate mass of injection site. The eff ective treatment should include control of infection,local debridement,PA res ection and blood vessel implantation .

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

Adult ; Aged ; Carcinoma, Non-Small-Cell Lung ; metabolism ; pathology ; Female ; Humans ; Lung Neoplasms ; metabolism ; pathology ; Lymph Nodes ; metabolism ; pathology ; Lymphatic Metastasis ; Male ; Middle Aged ; Neoplasm Staging ; RNA, Messenger ; metabolism ; Vascular Endothelial Growth Factor C ; blood ; genetics ; metabolism

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.