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 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 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.

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

Objective To study the clinical efficacy of unstable angina patients with the treatment of Yixin Kangtai capsule combine with diltiazem .Methods A total of 98 patients with unstable angina were randomly divided into control group and treat-ment group ,49 in each group .The control group was given conventional anti-anginal drug therapy ;while combination group was given Yixin Kangtai capsule combine with diltiazem on the basis of conventional anti-anginal medication .The course was 30 days . After the treatment ,the symptoms of angina ,ECG ST-T ,blood viscosity and markers of endothelial function NO and ET levels were observed .Results After treatment ,the total effective rate of the combination therapy group was higher than that of the con-trol group (P< 0 .05) ;the angina attacks and its duration in combination therapy group were lower than that of control group(P<0 .05) ;the total efficiency of electrocardiogram in combination therapy group was better than that of the control group (P< 0 .05) ;blood viscosity values and ET levels of combination therapy group were reduced compared with before treatment (P < 0 .05) ,but NO levels were increased(P< 0 .05) .Conclusion Yixin Kangtai capsule combine with diltiazem have significant efficacy for unsta-ble angina pectoris ,and have protective effect on endothelial function in patients with unstable angina .

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.

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