Serious market failure, as a result of insufficient incentive, exists in the orphan drugs market. Through legislation, the United States designed and established its owns systematic and linked orphan drugs incentive mechanism, which direct sat research, definition, clinical trials and approval of orphan drugs, so as to change the supply and demand status of orphan drugs. Such incentive mechanism erables the United States possess the most ap-proved orphan drugs in the world, and effectively relieves the problem of market failure of the orphan drugs. The ex-perience of the United States provides beneficial reference for the establishment and positive development of orphan drugs incentive mechanism in China.

Thanks to the policies of resources to strengthen grassroots ,and innovative resource placement in Zhejiang province and the help of Zhejiang University School of Medicine ,Shaoxing regional medical center has achieved significant improvement in its medical capabilities .The cooperation features the following :active promotion of new medical ideas and key technologies ;overall enhancement of scientific and technological innovation ;talent training to advance disciplinary development ;and joint efforts to train postgraduates .The momentum ,however ,can only be maintained ,unless such problems as the means of resources placement ,disciplines alignment ,and medical workers development can be well addressed .

Objective To investigate the olfactory function in patients with systemic lupus erythematosus (SLE) and to explore factors that may influence it.Methods The Connecticut Chemosensory Clinical Research Center (CCCRC) test was carried out in SLE patients and healthy controls for olfactory function testing.ELISA method was used to detect anti-ribosomal P protein antibody in the serum.The statistical methods used in this study including t test,ANOVA,LSD-t test,Pearson correlation analysis,multiple linear regression analysis,andx2 test.Results ① The CCCRC scores of the active,inactive group and healthy controls were compared,the difference was statistical significant (F=26.52,P=0.01).CCCRC score in active SLE group (62±16) was lower than that of the inactive group (80±13) the and the normal control group (83±12) (P＜0.01),while there was no statistical significant difference between the inactive group and the normal controls (P=0.226).② CCCRC score was lower in 16 ARPA positive SLE patients (61±17) than 49 negative patients (74±16) (t=2.681,P=0.009).③ In addition,CCCRC score showed a negative correlation with ARPA serum concentration (r=-0.327,P=0.008).There was no significant correlation between CCCRC score and disease course (r=0.141,P＞0.05).④ Multiple linear regression analysis indicated that CCCRC score was associated with age and ARPA.Conclusion SLE patients have olfactory dysfunction and the dys-function is associated with age and ARPA.

Objective:To investigate the ethical cognition of human sperm among the volunteers who intend to donor sperm,and to analyze the voluntteers' thoughts for donor behavior and its countermeasures.Methods:A questionnaire survey was conducted to investigate the volunteers who were the first time consulted sperm donation in the human sperm bank of the affiliated reproductive hospital of Shandong university.The cognition on the purpose of sperm,the causes of sperm shortage,privacy protection of the offspring,and the future of sperm banks in China were collected.Results:Donors were not well known the uses of sperm and privacy protection of the offspring.They thought traditional values and ethical worry caused sperm shortage and worried about the future of human sperm banks in China.Conclusion:The overall cognition on the sperm bank of the volunteers still exist certain one-sidedness and blind,which has seriously affected the enthusiasm of volunteers to donate sperm.The relevant departments should pay more attention to this,focus on promoting the propaganda and supervision work of ethical system of human sperm bank,construct objective,justice,and dedicative atmosphere for sperm donation,in order to dispel the volunteers' psychological concerns,and promote the healthy,fast and sustainable development of human sperm bank.

Objective To explore the safety and long-term results of preoperative imatinib mesylate administration (IM) in patients with locally advanced gastrointestinal stromal tumors (GIST).Methods From Sep 2009 to Nov 2016,locally advanced GIST patients treated in Fujian Medical University Cancer Hospital were analysed retrospectively.Result 34 patients were included.Preoperative median IM treatment was 27 weeks(range 12-71 weeks).65％ patients had a partial response to IM,35％ showed stable disease.All patients underwent surgical R0 resection.The complication rate was 9％ and no death occurred within 30 days post operation.The median follow-up time was 62.2 months (range of 13-89 months).20 patients continued to take imatinib orally,14 patients did not.The 3 year survival rate of patients undergoing surgery was 67％.Univariate analysis showed that tumor location,preoperative imatinib effect,pathology,targeted therapy after surgery were factors affecting prognosis.Multivariate analysis show that the independent risk factors affecting prognosis were tumor location,pathology,targeted therapy after surgery.Conclusion In locally advanced GISTs,preoperative IM is useful and safe that can effectively decrease tumor size,facilitating resection.

This paper introduced the development of medical specialists alliance in Zhejiang University. With Jinhua Central Hospital as an example,merits and setbacks of the 3-level trusteeship model in practice were discussed,with improvement recommendations raised for progress of the hierarchical medical system.

OBJECTIVE: To explore the cause of inconsistent genotypes for an α-thalassemia carrier by using two commercial genotyping kits. METHODS: GAP-PCR and PCR-reverse dot blotting (PCR-RDB) were employed to determine the genotype of the carrier, while Sanger sequencing was used to verify the results. RESULTS: Sequencing analysis demonstrated that the subject has carried a α1 globin gene with a 3.7 kb heterozygous deletion. In addition, two novel mutations, IVS-II-55(T>G) and IVS-II-119(G>TCGGCCC), were found in intron 2 of α2 globin gene. CONCLUSION The two mutations located in the binding regions of PCR primers have caused failure of PCR amplification and misreading of the genotype. Combination of clinical and hematological phenotypes is indispensible to infer the genotype of carriers for accurate diagnosis.
Genotype ; Heterozygote ; Humans ; Mutation ; alpha-Thalassemia ; genetics