Autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED)is a clinical rare autosomal recessive disorder.The major clinical manifestations are chronic mucocutaneous candadiasis,hypoparathyroidism and Addison disease.The three manifestations usually occur sequentially.The disease is caused by loss of function mutations in the AIRE gene.The onset,diagnosis,development,therapy and follow-up of this disease usually is the pediatric process.If we can early identify,timely diagnose and treat,regularly follow-up,it will improve children prgnosis and the quality of life,and avoid emergency and severe cases.

Orthostatic hypertension (OHT)in children was first proposed by Chinese scholars in 2012.The diagnostic criterion was revised in 2015.OHT in children was not rare in clinical practice.Children and adolescents are susceptible to OHT when they suddenly change posture or standing for a long time.The mechanisms of OHT were not clear.Dizziness,headache,nausea and vomiting were the main manifestations of OHT.Severe symptom in childhood OHT includes syncope.The prognosis of OHT was good,but the abnormal blood pressure will last to adults.Further study in OHT will help to predict the risk of cardiovascular and cerebrovascular diseases and help to evaluate target or-gan injury.The treatment of OHT has been explored,and non -medical treatment is the main option.Study in medical treatment for OHT has not been conducted.Now,the diagnostic criteria in children,epidemiologic status,clinical fea-tures and treatment of OHT were focused to deepen the understanding of OHT in children and improve the clinical standard of the disease.

Objective: To observe the effect of auricular needling on abilities of learning and memory as well as expression of NMDAR1 in rats with vascular dementia(VD).Methods:VD rat model was established by blocking 4-vessel.The immunohistochemistry method was used to detect the changes of NMDAR1 expression,the Y-type maze test was used to measure the abilities of learning and memory.Results:There were slight expression of NMDAR1cells in the brain tissue of normal group.Compared with the normal group,the expression of NMDAR1 positive cells in model group were significantly enhanced,but the average optical density is obvious reduced(P

Objective To observe the effect on IL-2,TNF,TGF in uterus and serum,and IL-2RmRNA in spleen of menopausal rats with preventive accupuncture on Guanyuan point,and analysis the mechanism.Methods 112 ten month-aged SD female rats examined by vaginal smear were chosen and divided into seven groups.And another 16 three point five month-aged SD female rats were another group.Radio-immunity and hybridiztion in situ methods were applied to observe the effect on IL-2,TNF,TGF in uterus and serum and IL-2R mRNA in spleen of 12,14 and 16 month-aged menopausal rats with preventive accupuncture on Guanyuan point(twice a week,eight weeks continued) in 10th month.Results Compared with the normal group,IL-2,TGF in uterus and serum and TNF in uterus of menopausal rats reduced,TNF in serum came down in the 12th month and went up in 14th and 16th month,IL-2R mRNA in spleen decreased.Compared with the same-aged model group,IL-2,TGF of serum and uterus and TNF in uterus increased.TGF in serum of 14 and 16 month-aged group,IL-2 in uterus of 12 and 16 month-aged group,TNF in uterus of 12 month-aged group and TGF in uterus of 12 and 16 month-aged group rised significantly(P

Objective To study the short-term effects of delayed cord clamping (DCC) in preterm infants.Method A thorough search was conducted on medical databases including Cochrane Library,PubMed,Ovid,Medline,VIP citation databases,Wanfang database and CNKI.Randomized control trials (RCTs) of DCC in preterm infants were retrieved from medical literature published during January 1,2000 to January 1,2016.DCC group had cord clamping 30 ～60 s after birth,and immediate cord clamping (ICC) group had cord clamping within 30 s after birth.Methodological quality was evaluated using Cochrane Handbook for systematic reviews and RevMan 5.1 software.Meta-analysis was performed using RevMan 5.1 software.Result Seventeen RCTs were included.Meta-analysis showed that:the blood pressure within 4 hours after birth (WMD =2.49,95％ CI 0.74 ～ 4.24),the hemoglobin concentration (WMD =15.92,95 ％ CI 6.37 ～ 25.47) and the hematocrit (WMD =4.84,95 ％ CI 3.47 ～ 6.22) within 24 hours after birth in the DCC group were higher than the ICC group,P ＜0.05;the risk of anemia (RR =0.62,95％ CI 0.47 ～ 0.81),intraventricular hemorrhage (RR =0.64,95 ％ CI 0.45 ～ 0.91) and mortality (RR =0.42,95％ CI 0.20 ～0.86) in the DCC group were lower than the ICC group,P ＜0.05;there were no statistically significant differences between the two groups in peak of serum bilirubin,phototherapy duration,rate of phototherapy treatment and blood transfusion,the incidence of hyperbilirubinemia and polycythemia (P ＞ 0.05).Conclusion DCC is safe and feasible for premature infants,and can improve the outcome of premature infants.

Objective To construct an eukaryotic co-expression plasmid pBM9 carrying human granulysin active peptide and murine IL-12 and determine its expression.Methods The primer pairs including granulysin leader peptide DNA sequence were designed to amplify granulysin from the plasmid pZM03 carrying granulysin gene by polymerase chain reaction(PCR).PCR product was directly cloned into an eukaryotic co-expression plasmid pBudCE4.1 to construct plasmid pBudCE4.1-S9K.pBudCE4.1-S9K plasmid was identified by DNA sequencing.Murine IL-12 gene was subcloned into pBudCE4.1-S9K to construct eukaryotic co-expression plasmid pBudCE4.1-S9K/mIL-12.Mycobacteria replicon Orim from plasmid pZM03 was subcloned into NotⅠsite of pBudCE4.1-S9K/mIL-12 to construct eukaryotic co-expression shuttle plasmid pBM9.pBM9 was tansfected into RAW264.7 cells.RT-PCR was used to detect the mRNA expressions of granulysin and IL-12.The protein expression of them were observed by immunocytochemical method and ELISA respectively.Results RT-PCR identified the expressions of granulysin and mIL-12 in transfected cells and culture supernatant.Immunocytochemical method and ELISA verified the protein expressions.Conclusion The recombinant shuttle plasmid pBM9 is successfully constructed and expressed in vitro,which laid a foundation of gene therapy for tumors with granulysin and mIL-12.

A target molecule affinity-ultrafiltration liquid chromatography-electrospray ionization tandem mass spectrometric (LC-ESI-MSn) method was established for rapid screening acetylcholinesterase (AChE) inhibitors from total alkaloids of fibraurea recisa Pierre.A total of 12 potential inhibitors were screened from Fibraurea recisa Pierre.and 6 compounds were identified including palmatine,berberine,jatrorrhizine,palmatrubine,7,8-dihydro-8-hydroxyberberine and groenlandicine.The AChE inhibitory activity of these 6 compounds was validated in vitro.Palmatine showed the strongest inhibitory activity for AChE,which was stronger than that of donepezil hydrochloride,demonstrating the potential of palmatine as anti-Alzheimer's drug.This method is simple,rapid,and accurate for directly screening active ingredients which can inhibit AChE from complex extract of traditional Chinese medicines.

BACKGROUND: In the field of organ transplantation, patients often take immunosuppressants after organ transplantation, such as CsA, FK506, DEX and MPA. However, their mechanisms of immunosuppression are different. The effect of immunosuppressive drugs on monocyte chemoattractant protein-1 (MCP-1) remains poorly understood. OBJECTIVE: To investigate the effects of different immunosuppressants on the secretions of MCP-1 in whole blood. METHODS: The whole blood of healthy volunteers was mixed with different immunosuppressants for 6 hours, such as CsA, FK506, DEX and MPA, which included low, middle and high concentrations, followed by PMA and IONO stimulation for 6 hours. MCP-1 levels in whole blood samples were compared. The whole blood cultured alone served as control. RESULTS AND CONCLUSION: MCP-1 secretion was inhibited by DEX (1, 10 mg/L) and CsA (0.25,1.25 mg/L)- However, FK and MPA exhibited no such effect. Therefore, DEX and CsA may inhibit the function of monocytes and macrophages in immune system by diminishing the secretion of MCP-1. The combination of FK (5 μg/L), MPA (10 mg/L) and DEX (1 mg/L) or CsA (0.25 mg/L), MPA (10 mg/L) and DEX (1 mg/L) can inhibit the secretion of MCP-1, but only DEX among all the immunosuppressants mentioned above exhibited significant effect on inhibiting the secretion of MCP-1 when using alone.

For investigating the epidemiology and genetic characteristics of enterovirus 71 (EV71) in Fujian from 2010 to 2015,we analyzed the surveillance data of EV71 and sequenced VP1 genes of 72 EV71 strains randomly picked from the past 6 years.The overall infection rate was gradually down and one incidence peak (from May to July) was observed each year.Major infectious population were focused on Xiamen,Fuzhou,Nanping and Quanzhou,the ages ranged from one to three years old.Scattered children were the most infected ones.The proportion of EV71 in the severe case was higher than in the HMFD(χ2 =732.064 5,P＜0.000 1).EV71 circulated from 2010 to 2015 in Fujian Province was belonged to subgenotype C4a in consistent with vaccine strain (H07).Compared with the VP1 of vaccine strains,the divergence of complete VP1 nucleotide sequence was gradually expanding as time distance increased,but the sequence of amino acid was not found obvious difference.Variations in 4 key immune epitopes of amino acid had not appeared a regular pattern in year and not consistent with the trend of proportion of EV71 in HMFD.As a result,we considered the epidemiology characteristics of EV71 in Fujian was obvious,72 strains still belonged to C4a subgenotype and had no outstanding antigenic drift or mutation.Extensive epidemiology surveillance and genetic characteristic are needed for the application of EV71 vaccine.

Objective To evaluate the diagnostic value of endoscopic ultrasonography (EUS), and explore the efficacy of endoscopic treatment in patients with esophageal submucosal tumor. Methods Sixty-eight patients with esophageal submucosal tumor were selected, and the tumor was derived from the muscularis mucosa and submucosa according to the common endoscope and endoscopic ultrasonography detection. Endoscopic mucosal resection (EMR) was applied to remove submucosal tumor with diameter less than 1.0 cm, endoscopic piecemeal mucosal resection (EPMR) or endoscopic submucosal dissection (ESD) was applied to remove submucosal tumor with diameter 1.1 - 1.5 cm, and ESD was applied to remove submucosal tumor bigger than 1.5 cm. Samples were examined by pathology after treatment. Results Tumors in all the patients were completely removed, and the tumor diameter was 0.6-2.3 cm. Forty-one cases were treated with EMR, 9 cases were treated with EPMR and 18 cases were treated with ESD. Four patients had intra-operative bleeding that was stopped by electrocoagulation hemostasis. No perforation occurred in all cases. Postoperative pathology revealed 43 cases had leiomyoma, 23 cases had interstitialoma, and 2 cases had lipoma. Patients were reviewed by gastroscope 3 months after operation. The white scars formed in all patients, and there was no residue or recurrence. Conclusions Different origin layers and property of esophageal submucosal tumor can be diagnosed accurately by EUS, and endoscopic therapy (EMR, EPMR and ESD) is an effective treatment for submucosal tumor from muscularis mucosa and submucosa. Endoscopic therapy is safe and effective. It provides sufficient pathological information.