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.

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

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

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.

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.