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 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 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 .

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.

Objective To analyze the correlation of visual acuity and metamorphopsia with foveal morphology before and after vitrectomy with internal limiting membrane peeling (ILMP) in idiopathic macular epiretinal membrane (IMEM) eyes.Methods This is a retrospective case series of 47 IMEM patients (49 eyes).All the patients underwent 25G pars plana vitrectomy with IMEM removal and ILMP.The best corrected visual acuity (BCVA) was measured using the international standard visual acuity chart,and the results were converted to the logarithm of the minimum angle of resolution (logMAR) visual acuity.The severity of metamorphopsia was measured using M-charts.The central macular thickness (CMT),inner nuclear layer thickness (INT),inner retinal layer thickness (IRT),outer retinal layer thickness (ORT),the status of ellipsoid zone (EZ) were assessed with spectral-domain optical coherence tomography at baseline and each month postoperatively.The differences in BCVA,CMT,INT,IRT,ORT and status of EZ before and after surgery were analyzed,so did the correlations between these indexes at the same time.Results Compared with baseline,the postoperative BCVA was significantly increased (F=6.133,P＜0.001),but the M value,CMT,INT,IRT,ORT were significantly decreased (F=12.481,10.565,15.739,6.046,10.569;P＜0.001);the integrity of EZ was improved significantly (x2=12.309,P＜0.001).Preoperative BCVA was positively related to the CMT (r=0.720) and ORT (r=0.720,0.887;P＜0.05),while negatively related to preoperative integrity of EZ (r=-0.295,P＜0.05).The postoperative BCVA was positively related to the preoperative BCVA and postoperative CMT,ORT (r=0.774,0.754,0.842;P＜0.05),while negatively related to postoperative integrity of EZ (r=-0.676,P＜0.05).The preoperative M value was positively related to the preoperative CMT,INT,IRT,and ORT (r=0.931,0.668,0.840,0.637;P＜ 0.05).The postoperative M value was positively related to the preoperative M value and postoperative CMT,INT,IRT,and ORT (r=0.723,0.722,0.767,0.825,0.387;P＜0.05).Conclusions Vitrectomy with ILMP for IMEM can improve the visual acuity,metamorphopsia and foveal morphology.Both visual acuity and metamorphopsia correlate with foveal morphology.

Objective To evaluate the safety and effectiveness of vitrectomy combined with internal limiting membrane (ILM) tamping on macular hole and retinal detachment (MHRD) in highly myopic eyes.Methods 23 patients (23 eyes) were retrospectively reviewed,who were diagnosed as MHRD through examination of the ocular ftmdus,optic coherence tomography (OCT) and B-mode ultrasonography.There were 5 males (5 eyes) and 18 females (18 eyes).The mean age was (62.35 ± 8.28) years.The mean course of disease was 1.1 months.The logarithm of the minimum angle of resolution (logMAR) best-corrected visual acuity (BCVA) was 2.31 ± 0.72.The mean axial length was (28.66 ± 1.99) mm.All patients underwent 23G microincision vitrectomy.After vitreous gel and cortex were gently resected,the ILM around the edges of the macular hole was stained with indocyanine green,and was folded and pushed to fill the macular hole gently.Then silicone oil or C3F8 gas tamponade was applied in 18 eyes and 5 eyes,respectively.The silicone oil was removed after 3 months.The follow-up was 6 months.The BCVA,macular hole closure,retinal anatomical reattachment were retrospectively observed,and were used to evaluate the safety and effectiveness of the surgery.Results At the 6 months after surgery,the logMAR BCVA was improved to 1.13 ± 0.38,the difference was significant (t=l 5.33,P=0.00).The postoperative macular hole closure rate and retinal anatomical reattachment rate were 100％.There were no ocular or systemic adverse events observed in all patients.Conclusion Vitrectomy combined with ILM tamping is an effective and safe treatment for the high myopic eyes with MHRD.