Objective To investigate the association with death for serum parameters at the time of diagnosis and its value in predicting the death in infants with hemophagocytic syndrome (HPS).Methods A retrospective case-control study was conducted on 108 children with HPS who were admitted to our center between July 2005 and July 2012.For each patient,demographic,laboratory data and outcome information were collected.The patients were divided into death and surviving groups based on the follow-up results.The relation between serum markers and death was examined using the COX proportional hazards model and decision tree.Results Of 108 infants with HPS,33 died corresponding to a fatality rate of 30.6％ and 90.3％ of deaths occurred within 8 weeks after diagnosis.Following features were significantly associated with death:white blood cells (WBC) ＜5 x 109/L (HR =9.08,95％ CI 3.07 ～ 26.87),hemoglobin ＜80 g/L (HR =6.15,95％ CI 1.68 ～ 22.49),albumin ＜ 28 g/L (HR =4.63,95％ CI 1.12 ～ 7.39),serum ferritin ＞ 1 100 μg/L (HR =3.05,95％ CI 1.28 ～ 16.75),trigeminal ganglion ≥4 mmol/L (HR =2.88,95％ CI 1.51 ～ 8.60),and prothromin time ≥ 16 s (HR =3.60,95 ％ CI 1.28 ～ 7.24),and fever for more than 2 weeks (HR =5.39,95％ CI 1.97 ～ 14.66).Decision tree demonstrated that the probability of death was as high as 100％ for infants with WBC ＜5 x 109/L and hemoglobin ＜ 80 g/L.The odds of dying was still 66.7％ for infants who had WBC≥5 × 109/L but reported trigeminal ganglion ≥4 mmol/L after having fever for more than 2 weeks.Conclusion The first 8 weeks after the onset of HPS is the critical period of treatment.There are several easily available serum predictors of early mortality in HPS infants,particularly the WBC and hemoglobin level,which may help guide treatment decisions.

Objective To explore the heredity susceptibility of children to Kawasaki disease (KD) through studying expression and genomic density polymorphism of peripheral erythrocyte complement receptor-1 (ECRI). Methods Thirty cases of KD patients and 28 cases of healthy children were included in this study. The rates of red blood cell (RBC)-C3bRR and RBC-ICR were detected by method described elsewhere. The ECR1 activity and genomic density polymorphism were detected by Hind Ⅲ restriction enzyme digestion polymerase chain reaction-restriction fragment length polymorphism. Results Rates of RBCoC3bRR of KD patients during the acute phase was significantly lower than that of the control group (P < 0.01), and remained lower than the control group during the recovering phase (P < 0.05). The rates of RBC-ICR were significantly higher in KD patients than that of the control group (P < 0.05). Frequencies of HL and LL genotypes of KD patients were more than those of the control group (P < 0.01). A significant difference was found in the frequency distribution of ECR1 genotype between the two groups (P < 0.01). L allele frequency in the patient group was higher than that in the control group. Conclusions Depressed RBC immune function in KD patients may be linked to the high frequency of L allele, which implies the genomic density polymorphism of ECR1 play an important role in determining susceptibility to Kawasaki disease. (J Clin Pediatr,2010,28(2):160-163)

Objective:To observe the expressions of Th1/Th2/Th1 7 cytokines and antimyocardial antibodies in mouse dilated cardiomyopathy (DCM),and explore their roles and significance in DCM pathogenesis.Methods:BALB/c mouse model of DCM was built with coxsackievirus(CVB3).Mice in control group were treated with phosphate-buffered saline (PBS)i.p. Mice were killed at the 24th week,and the fibrosis degree of myocardial tissue was observed by myocardial Masson’s staining. Expressions of Th1/Th2/Th1 7 cytokines in plasma were detected by using protein microarray.The mouse plasma levels of an-timyocardial antibodies such as adenine nucleotide translocator(ANT)antibody,β-myosin heavy chain(β-MHC)antibody,car-diac L-type calcium channel(CACH2),and anti-β1-adrenergic receptor(β1 AR)were measured by enzyme-linked immunosorbent assay (ELISA).Results:The result of myocardial pathological examination in DCM group fit the features of DCM 24 weeks af-ter virus infection.Compared with those in control group,expression levels of interleukin(IL)-2,IL-10,IL-13,IL-28 and TNF-αin DCM group decreased(P <0.05),however,the expression levels of IL-6,IL-1 7,IL-21 ,IL-22,IL-23 and TGF-βin-creased significantly(P <0.05).Meanwhile,compared with those in control group,the plasma levels of ANT andβ-MHC anti-bodies in DCM group increased significantly (P <0.05).Furthermore,positive correlation was found between level of IL-22 and level of anti-ANT according to correlative analysis(P <0.05 ).Conclusions:Immunologic derangement induced by imbal-ance of antimyocardial antibodies such as ANT and β-MHC,and Th1/Th2/Th1 7 cytokines such as IL-2、IL-10、IL-13、IL-28、TNF-α、IL-6、IL-1 7、IL-21 、IL-22、IL-23 and TGF-βmay play important roles in the pathogenesis of DCM.Furthermore,IL-22 may boost the production of ANT antibody.

Serine/arginine-rich splicing factors (SRSFs) refer to twelve RNA-binding proteins which regulate splice site recognition and spliceosome assembly during precursor messenger RNA splicing. SRSFs also participate in other RNA metabolic events, such as transcription, translation and nonsense-mediated decay, during their shuttling between nucleus and cytoplasm, making them indispensable for genome diversity and cellular activity. Of note, aberrant SRSF expression and/or mutations elicit fallacies in gene splicing, leading to the generation of pathogenic gene and protein isoforms, which highlights the therapeutic potential of targeting SRSF to treat diseases. In this review, we updated current understanding of SRSF structures and functions in RNA metabolism. Next, we analyzed SRSF-induced aberrant gene expression and their pathogenic outcomes in cancers and non-tumor diseases. The development of some well-characterized SRSF inhibitors was discussed in detail. We hope this review will contribute to future studies of SRSF functions and drug development targeting SRSFs.

Objective Interleukin-27 (IL-27) has been reported to reduce the levels of interleukin17(IL-17) and alleviate the severity of experimental autoimmune myocarditis.IL-17,an important tissueprotective cytokine in viral myocarditis (VMC),has been reported to increase synovial expression of IL-27 in rheumatoid arthritis.However,the influence of IL-17 on IL-27 expression in murine model of VMC remains unknown.Methods Wild-type (WT) and IL-17A-deficient (IL-17A-/-) mice on the BALB/c background were intraperitoneally (i.p) injected with coxsackievirus B3 (CVB3) for establishing VMC models.Cardiac tissue was obtained on day 7 after CVB3 injection.Myocardial histopathologic changes were observed by hematoxylin-eosin (HE) stained myocardial sections.Expression of IL-27 in heart and serum was measured by real-time reverse transcription-polymerase chain reaction (RT-PCR) and enzyme linked immunosorbent assay (ELISA),respectively.Furthermore,splenic lymphocytes and peritoneal macrophages were purified 1 week after injection from WT mice.Isolated lymphocytes were cultured in the presence of different concentrations (0 and 25 ng/ml) of recombinant IL-17 (rIL-17) for 24 h.Macrophages were cultured with different concentrations of rIL-17 (0 and 10 ng/ml) for 48 h.IL-27 mRNA expression of cultured cells was assayed by RT-PCR,and their protein level in the culture supernatant was measured by ELISA.Results Compared with WT mice,significantly less cardiac inflammation was evidenced in the heart of IL-17A-/-mice (0.9 ± 0.3 vs.1.9 ± 0.5),relative cardiac IL-27 p28 mRNA expressions (1.11 ± 0.24 vs.3.1 ± 0.8) and serum IL-27 protein [(72 ± 18) pg/ml vs.(95 ± 25) pg/ml] were also significantly lower in IL-17A-/-mice (all P ＜ 0.05).In the culture lymphocytes,the relative mRNA (1.02 ± 0.13 vs.1.32 ± 0.21) and protein [(49 ± 9) pg/ml vs.(52 ± 11) pg/ml] expressions of IL-27 p28 and were similar post treatment with 0 and 25 ng/ml rIL-17 (all P ＞0.05).Compared with 0 ng/ml rIL-17 culture with macrophages,higher relative mRNA (8.5 ±3.1 vs.2.2 ±0.7) and protein [(368 ±95) pg/ml vs.(150 ± 38) pg/ml] expressions of IL-27 p28 were detected in 10 ng/ml rIL-17 group (all P ＜0.05).Conclusion Our data indicates that cytokine IL-17 may contribute to the secretion of IL-27 in VMC mice.Furthermore,macrophages but not lymphocytes may be the important IL-27-producing immune cells and major target cells for IL-17.Thus,IL-27 and IL-17 might be actively involved in the pathogenesis of VMC.

Objective To establish and evaluate a talent training system for hospital information pharmacists based on a competency model,thereby enhancing refined management of pharmaceutical affairs in hospitals and promoting high-quality development of pharmacy services.Methods The current development status of hospital information pharmacists at home and abroad was examined.A competency model for hospital information pharmacist positions was established using methods such as behavioral event interviews.In conjunction with this model,training course outlines addressing knowledge and capability requirements were developed.National skill training classes for hospital information pharmacists were conducted using innovative teaching methods like scenario simulation and case discussion.Training effectiveness was evaluated by tracking participants'work performance and scientific research achievements in the field of pharmaceutical information through questionnaire surveys before and after the training.Results The hospital information pharmacist positions competency model was constructed in four parts:knowledge,experience,skills,and personal traits.Based on this model,a training system for hospital information pharmacists was established,which included setting job responsibilities and performance indicators,establishing a pharmacy information department system,designing a series of training courses and publishing textbooks,founding national training classes,creating a specialized question bank,and developing a digital pharmacy network platform.The project team tracked the work performance and scientific research achievements of participants before and after training,using the national hospital information pharmacist training class hosted by the Guangdong Pharmaceutical Association as an example.From 2018 to 2024,a total of 465 information pharmacists from 298 hospitals across 28 provinces and cities were trained over seven sessions.Questionnaires were sent to participants who had completed at least one year since graduation,and 236 valid responses were received.Of these,169(71.6% )participants reported playing a major role in pharmacy informatization projects after the training,and 65(27.5% )participants published papers,applied for projects,or filed for patents after the training,with the majority related to pharmacy automation and informatization.Conclusion A competency model for hospital information pharmacist positions was constructed and applied nationwide,achieving favorable results.