Post-marketing vaccine safety surveillance, including both passive and active surveillances, aims to detect and alert to signals of adverse events following immunization (AEFI), and to further ensure public safety and public confidence in vaccination. Active surveillance could proactively seek information of AEFI and timely investigate the potential safety signals, therefore, it has become the main development trend of post-marketing surveillance worldwide. Nowadays, there is an ongoing interest in developing active surveillance systems that can incorporate and use existing electronic data such as administrative claims and electronic health records. Researchers have also began exploring ways of accruing data closer to "real-time" in order to speed the recognition of potential safety problems.This near real-time vaccine safety surveillance is gradually emerging worldwide. This study reviews the development and methodology of near real-time surveillance and aims to accelerate the foundation of the active surveillance system for vaccine safety in China.

To summarize the development and application of tree-based scan statistic (TreeScan), explain the methodology and provide a reference for future use of this method by reviewing the original pharmacoepidemiological and vaccine studies using the TreeScan. Medline, Embase and Web of Science databases were used for the retrieval of eligible studies using keywords related to TreeScan. A total of 15 eligible studies were included, in which 9 studies explored the adverse events of drugs and 6 studies focused on the safety of vaccines. Three types of models (Poisson probability model, Bernoulli probability model and tree-temporal scan statistic model) of TreeScan were used. The major differences among the three models were 1) whether predefined control was used according to research question, 2) whether the time from exposure to onset of adverse events was considered. Several studies explored its ability by comparing with other methods for adverse event detection or by using known adverse events. This review shows that TreeScan is an effective method for the safety signal detection of drugs or vaccines, which develops rapidly and globally. It is very necessary to promote its use in drug safety monitoring and other related fields in China.

OBJECTIVE To explore the clinical features of patients carrying deletions of the rod domain of the dystrophin gene. METHODS Clinical data of 12 Chinese patients with Becker muscular dystrophy (BMD) and such deletions was reviewed. RESULTS Most patients complained of muscle weakness of lower limbs. Two patients had muscle cramps, one had increased creatine kinase (CK) level, and one had dilated cardiomyopathy. CONCLUSION Compared with DMD, the clinical features of BMD are much more variable, particularly for those carrying deletions of the rod domain of the dystrophin gene. Muscular weakness may not be the sole complaint of BMD. The diagnosis of BMD cannot be excluded by moderately elevated CK. For male patients with dilated cardiomyopathy, the possibility of BMD should be considered.

Objective To explore the correlation between serum lipoprotein-associated phospholipase A2 (Lp-PLA2) level with the occurrence of ischemic stroke and prognosis in the patients with hypertension . Methods Eighty-eight patients with hypertension in this hospital from January 2013 to January 2016 were se-lected ,including 34 cases of simple hypertension and 54 cases of hypertension complicating ischemic stroke . The Lp-PLA2 level was detected .The severity of ischemic stroke was evaluated on 1 ,14 d after admission by adopting the NIHSS .The patients with hypertension complicating ischemic stroke were divided into the good prognosis group and poor prognosis group according to the NIHSS change situation .Moreover the independent predictive factors of prognosis were evaluated by adopting the Logistic multivariate regression .Results The Lp-PLA2 level in the patients with hypertension complicating ischemic stroke was significantly higher than that in the patients with simple hypertension .The Lp-PLA2 level (OR=1 .523 ,95％ CI=1 .323-1 .657) and NIHSS score (OR=3 .275 ,95％ CI=1 .402 -6 .208) at admission were the independent predictive factors of poor prognosis .Conclusion Serum Lp-PLA2 level is closely correlated with the occurrence and prognosis of ische-mic stroke in the patients with hypertension ,which can serve an evaluation index for the occurrence and prognosis of ischemic stroke in the patients with hypertension and provides an objective basis for the clinical therapy .

Objective To investigate the complications of deep brain stimulation (DBS) internal pulse generator (IPG) replacement procedures and discuss the reasons,preventive measures and treatments.Methods From 2012 to 2016,285 procedures (according to the number of replacement IPG) were performed for 211 patients in our hospital.Among them,178 patients were with Parkinson's disease,29 patients were with dystonia,3 patients were with tic disorder,and one with essential tremor.Thirty-two patients previously used Medtronic replaced with local DBS with brand of PINC and Sceneray,and the other 179 patients remained the use of Medtronic brand.Furthermore,36 patients got extension cable reimplantation along with IPG replacements.Results Replacement surgeries were divided into 3 types:IPG replacement in situ;bilateral side single-channel IPG was replaced by double-channel IPG or double-channel IPG was replaced by bilateral single-channel IPG;extended cables and IPG replacement were carried out simultaneously.The follow up period was from one to 6 years for these 211 patients,and 15 got surgical-and hardware-related complications:6 with sack hemorrhage,2 with skin erosion,one with IPG rejection,3 with adaptor fracture,and 3 with impedance abnormality were recorded;no infection was noted.Twenty-six patients got significant improvement after new IPG replacement.Conclusions DBS IPG replacement operations is a regular surgery with certain safety.Personalized surgical procedures,rigorous intraoperative operation and correct postoperative management can effectively reduce and prevent the complications of IPG replacement surgery.

OBJECTIVETo explore the significance of SMN1 gene mutations among patients with spinal muscular atrophy (SMA) and the value of multiplex ligation dependent probe amplification (MLPA) for its diagnosis.

METHODSPotential mutations of the SMN1 gene were detected among 78 SMA patients with a MLPA assay.

RESULTSHomozygous deletion of SMN1 exons 7 and 8 was detected in 70 (89.7%) of all patients. Homozygous deletion of exons 7 and heterozygous deletion of exon 8 was detected in 3 patients (3.8%). Homozygous deletion of SMN1 exons 7 alone was detected in 3 patients (3.8%). Heterozygous deletion of SMN1 exons 7 and 8 was detected in 2 patients (2.6%). For 77 of the patients, both parents were found to carry heterozygous deletion of the SMN1 gene, which was consistent with the recessive inheritance of SMA. One patient with SMA type I was found to be rather rare. The patient was found to carry homozygous deletion of SMN1 exons 7 and 8, for which her mother was heterozygous, while no mutation was found in her father.

CONCLUSIONHomozygous deletion of the SMN1 gene have been detected in more than 95% of SMA patients. No homozygous deletion of exon 8 has been found. Homozygous deletion of exon 7 is more significant in the pathogenesis of SMA.

Exons ; Female ; Gene Deletion ; Humans ; Male ; Multiplex Polymerase Chain Reaction ; Muscular Atrophy, Spinal ; genetics ; Mutation ; Survival of Motor Neuron 1 Protein ; genetics

Objective To study the clinical effect of bilateral anterior internal capsulotomy on patients with refractory anxiety disorders.Methods Twenty-one patients with anxiety disorders,failed to pharmacological and cognitive behavior therapies for 5 years and admitted to our hospital from November 2009 to January 2012,were chosen; they received MRI-guided stereotactic bilateral anterior capsulotomy.The coordinates of the lesion target were determined under high resolution MRI directly and confirmed by intra-operative impedance test and high frequency stimulation.The lesions were received radiofrequency at 80 ℃ for 60 seconds.Pre-and post-operative mini-mental state examination (MMSE),self-rating anxiety scale (SAS),Hamilton depression rating scale (HAMD) and Hamilton anxiety rating scale (HAMA) were performed by the same psychiatrists.Results In the 21 patients,8 had anxiety-free,10 had significant improvement,and 3 had no significant improvement.No serious surgery-related complications were noted.Nine patients had mild cognitive deficit and transient dysmnesia only at 1-2 weeks after operation.Personality changes,such as lack of motivation,apathy and indolence,appeared in 4 patients.Psychiatric evaluations demonstrated significant decrease of SAS,HAMD and HAMA scores after operation as compared with those before operation (P＜0.05).However,the difference of MMSE scores between pre-and post-operation was not statistically significant (P＞0.05).Conclusion MRI guided stereotactic bilateral capsulotomy is precise,safe and much an effective treatment for refractory anxiety disorders,whcih is promising to alleviate the symptoms of anxiety and depression,as well as improving the quality of life.

Objective To explore agreement between parent-child report of self-rating scale of systemic family dynamics (SSFD),as well as factors that may influence the agreement.Methods SSFD was used to investigate the agreement between parent and child reports on family dynamics in a sample of 639 Chinese children aged 10 to 18 years from Mainland China,and possible influence factors.Results The results showed that there was significant difference between parent-child report of SSFD.The parents had significant lower score than their children at atmosphere,individuation and disease conception and had higher score at system logic (all P＜0.01).But the two still had significant positive correlation (correlations ranging from 0.15 to 0.38),indicating a low to moderate agreement between them(r=-0.15-0.38,all P＜0.01).The age factor was related to parent-children agreement on SSFD.Parent-child agreement was highest for family atmosphere,followed by individuation,system logic and disease conception.The age factor was related to parent-child agreement on SSFD.Conclusions Both parents' and children's reports of SSFD can be used to assess family dynamics,however,attention should be payed to the affection of parent-child report's difference and age factor to the results.

Objective To investigate the clinical characteristics,treatment effect,long-term follow up results,guanosine triphosphate (GTP) cyrclohydrolase Ⅰ (GCH Ⅰ)gene and tyrosine hydroxylase(TH) gene mutations in patients with dopa-responsive dystonia (DRD).Methods The clinical features of 3 families with 4 affected members were analyzed and all of 4 patients were screened for mutations of the GCH Ⅰ gene and TH gene with DNA sequences.Results Four patients were females,average age at onset was (15.3 ± 5.6) years (range:from 9 to 20 years).The initial symptoms were a gait disorder,stiffness or tremor of the lower limbs in all patients presented with diurnal fluctuation.As the increase of disease duration,bilateral hand tremor was found in three patients,systemic torsion was found in one patient and torticollis was found in one patient.All patients' symptoms were in complete remission after administration of low dose of levodopa.Four patients were followed up for 0.5 to 10.0 years,and all were still responsive to the levodopa treatment and effective dosage was decreased as the increase of the disease duration.No longterm side effects of levodopa had occurred after long-term treatment.One patient was found to have c.607G ＞A(p,Gly203Arg) heterogenetic mutation in GCH I gene.Molecular analysis revealed a compound heterozygous mutation in the TH gene (p.Y447Ter and p.V468M) in one patient.No point mutations in both genes were found in other patients.Conclusions DRD patients have dramatic and sustained response to levodopa and no long-term side effects of levodopa after long-term treatment.The detection of GCH Ⅰ and TH gene mutations is helpful in early diagnosis but the negative results could not exclude the diagnosis of DRD.

Objective To investigate the family dynamic characteristics of adolescents with emotional disorder.Methods 74 adolescents with emotional disorder from Child Mental Health Research Center of Nanjing Brain Hospital Affiliated to Nanjing Medical University were chosen and compared with 148 gender- and agematched controls from schools in Nanjing.Self-rating scale of systemic family dynamics(SSFD) was used to assess the characteristics of family dynamics,and general demographic information inventory was conducted for all subjects.Results Adolescents with emotional disorder showed higher scores in family atmosphere and systemic logic than control group ( ( 30.54 ± 8.28 ) vs (23.45 ± 7.40),( 17.14 ± 4.26 ) vs ( 15.43 -± 3.86 ),all P ＜ 0.01 ).Logistic regression analysis showed that high individuation ( OR =0.903,95％ CI:0.834 ～ 0.977 ) and high disease thinking( OR =0.853,95％ CI:0.750 ～ 0.970 ) were protective factors for emotional disorder.High family atmosphere ( OR =1.167,95％ CI:1.101 ～ 1.236) was risk factor for emotional disorder.Conclusion Adolescents with emotional disorder demonstrate boring and hostile family atmosphere and Either/or family systemic logic on the family dynamic characteristics.Family atmosphere,disease thinking and individuation are associated with emotional disorder.