ObjectiveTo explore the clinical features,etiological factors,diagnosis and treatments in children with hemophagocytic syndromes (HPS).MethodsA retrospective study was carried out to analyze etiological factors,clinical symptoms and sighs,laboratory findings and outcomes of 32 children with HPS in our hospital.Results( 1 ) Etiological factors:virus infection cases accounted for 75.0％ (24/32),in which Epstein-Barr virus infection cases were predominant,making up to 68.8％ (22/32) ; (2) Main clinical features:included persistent fever,hepatosplenomegaly,lymphadenopathy; ( 3 ) Characteristic laboratory findings:included decrease of three lines or two lines of peripheral blood cells,hyperferrttinemia,abnormal liver function and coagulation function,hypertriglycerdema and decreased fibrinogen,hemophagocytosis in bone marrow,high soluble interleukin-2 receptor level,decreased natural killer cell activity.(4) Treatments and outcomes:twenty-four(24/32) virus infection cases received antivirus and high dose of immunoglobulin treatment.Twenty-two cases were treated with glucocorticoid and cyclophosphamide,resulting in 11 cases improvement,5 cases with complete remission,2 cases giving up and 4 death.The other 10 cases were treated under the guide of HLH-2004 protocol treatmemt with 4 cases improved and 6 cases with complete remission.Conclusion HPS is mainly triggered by infections,especially Epstein-Barr virus infection.Serum ferrifin,interleukin-2 receptor level and natural killer cell activity are biomarkers of the disease,so that they should be monitored during the progress of chemotherapy.HLH-2004 protocol is a good guide for the treatment of this disease.

Objective To study the effect of the magnetic field-treated water on metabolism of monoaminergic nerve transmitters in mice. Methods Twenty healthy aged Kunming mice were divided into a control group and an experimental group randomly. The mice in the control group were given tap-water and those in the experimental group magnetic field-treated water for 30 days. The content of monoaminergic nerve transmitters in brain of mice in the two groups were determined. Results A significant difference was found in the content of monoaminergic nerve transmitters between the control and the experimental groups. The contents of NE, DA, 5-HT and 5-HIAA increased significantly(P

Objective To determine the correlation between serum asymmetric dimethylarginine (ADMA) and non-spoon-shaped blood pressure of non-dialysis chronic kidney disease (CKD) patients,also to observe the impact of the serum ADMA level on the structure and function of left ventricle.Methods One hundred and twenty cases of non-dialysis CKD patients underwent 24-hour ambulatory blood pressure monitoring were divided into three groups:CKD1-2,CKD3,CKD 4-5.Serum ADMA concentration was measured using liquid chromatograph and other clnical data such as uric acid (UA),left ventricular mass index (LVMI),24 h urine protein,and high-sensitivity C-reactive protein (hs-CRP) were collected for further statistical analysis.Results (1) With the decline of renal function,ADMA concentration was increased,from CKD 1-2 (1.70±0.48) μmol/L rose to CKD 4-5(4.46±1.56) μmol/L (P ＜ 0.05).(2)There were 42 cases of CKD patients with hypertension and 78 cases of CKD patients with normal blood pressure.The serum ADMA levels in hypertension group was significantly higher than those in non-hypertensive group [(3.53±1.70) μmol/L vs (2.01±0.65) μmol/L,P ＜ 0.05].(3)There were 50 cases of non-spoon-shaped normotensive CKD patients and 28 cases of spoon-shaped normotensive CKD patients.Serum ADMA level and LVMI in non-spoon-shaped group were significantly higher than that in spoon-shaped group when kidney functions appeared to be equal (P ＜ 0.05).(4)Serum ADMA level was positively correlated with UA(r=0.352,P ＜ 0.01),LVMI (r =0.345,P＜ 0.05),24 h urine protein(r =0.200,P ＜ 0.05),and high-sensitivity C-reactive protein (r =0.309,P ＜ 0.01),but negatively correlated with the left ventricular ejection fraction (LVEF)(r =-0.329,P ＜ 0.01) and estimated glomerular filtration rate (eGFR)(r =-0.011,P ＜ 0.01).Multiple regression results showed that eGFR,UA,LVMI,hs-CRP,24 h urine protein were associated with ADMA level.The regression equation was Y =1.991-0.011 × [eGFR] + 0.002 × [UA] + 0.008 × [LVMI] + 0.036 ×[hs-CRP]-0.084× [24 h urinary protein].Conclusions Serum ADMA level begins to increase in early stage CKD and it progressively increases with the decline of renal function,also the non-spoonshaped blood pressure ratio and the left ventricular damage increase.Kidney function,urine protein and microinflammatory state may impact on the serum ADMA level.

Objective To investigate the correlations of expression of interferon inducible protein 10 ( IP-10) mRNA in peripheral blood mononuclear cells ( PBMCs) with serum levels of HBsAg and HBV DNA in patients with HBV-related acute on chronic liver failure ( HBV-ACLF ) , and to assess its value in predicting disease prognosis .Methods Eighty patients with HBV-ACLF, 60 patients with chronic hepatitis B (CHB), and 25 healthy subjects were enrolled during October 2013 and February 2015.IP-10 mRNA in PBMCs was measured by real time quantitative polymerase chain reaction ( RT-qPCR) , and the difference in IP-10 mRNA expression among three groups was compared by ANOVA .Serum levels of HBsAg and HBV DNA were also measured in patients with HBV-ACLF, and Pearson correlation test was performed to analyze the correlations of IP-10 mRNA with HBsAg and HBV DNA levels .t test was used to analyze the differences in IP-10 mRNA, HBsAg and HBV DNA levels between fatal and surviving cases after 3-month entecavir therapy in HBV-ACLF group.Receiver operating characteristic curves ( ROC) was used to evaluate the prognostic value of IP-10 mRNA, HBsAg and HBV DNA in HBV-ACLF patients .Results IP-10 mRNA level in HBV-ACLF group was 0.998 ±0.186, which was higher than those in CHB patients and healthy controls (0.641 ±0.083 and 0.412 ±0.062, t=3.841 and 16.661, P<0.01).Pearson correlation analysis showed that IP-10 mRNA level was negatively correlated with HBsAg level in HBV-ACLF patients (r=-0.576, P<0.01), but positively correlated with HBV DNA level (r=0.547, P<0.01).After 3-month entecavir therapy , IP-10 mRNA level in surviving cases of HBV-ACLF group was 0.894 ±0.164, which was lower than that in fatal cases ( 1.103 ±0.177, t =-4.328, P <0.01 ); HBsAg level in surviving cases was higher than that in fatal cases (3.303 ±0.565 vs.2.605 ±0.844, t =3.251, P<0.01).The area under ROC of IP-10 mRNA in evaluating prognosis of HBV-ACLF was 0.820, which was higher than those of HBsAg (0.663) and HBV DNA (0.570).Conclusions IP-10 mRNA in patients with HBV-ACLF is over-expressed and is correlated with HBsAg and HBV DNA levels .It may be used for predicting the prognosis of patients with HBV-ACLF.

Objective To investigate the expression and clinical significances of miR-215 and runtrelated protein1 (RUNX1) in retinoblastoma (RB),and to study the regulation effect of miR-215 on RUNX1 in the retinoblastoma cell line PMC-RB.Methods The expressions of miR-215 and RUNX1 in the tumor tissue,non tumor tissues adjacent to cancer,human RB cell line FMC-RB and human normal retinal vascular endothelial cell line ATCC of RB patients were detected by quantitative real-time polymerase chain reaction (qRT-PCR).miR-215 mimics (miR-215-mimic),miR-NC,si-RUNX1 and si-NC were transfected into FMC-RB cell line respectively.Cell proliferation,migration and invasion ability were measured respectively,thus detecting the regulation effect of miR-215 on RUNX1.Results The expression of miR-215 in RB tissues was significantly lower than that in non tumor tissues adjacent to cancer,while the mRNA expression of RUNX1 was higher than that in non tumor tissues adjacent to cancer (P ＜ 0.05).The expression of miR-215 in PMC-RB cells was lower than that in ATCC,while the mRNA expression of RUNX1 was higher than that in ATCC (P ＜0.05).The expression of miR-215 and RUNX1 in RB tumor tissues were closely related to the clinicopathological features of optic nerve infiltration,tumor tissue differentiation and lymph node metastasis (P ＜ 0.05).Cell proliferation,migration and invasion in miR-215-mimic group were significantly lower than those in miR-NC group (P ＜ 0.05).In transfected 3' untranslated region (3 'UTR)-Wt cells,the luciferase activity in miR-215-mimic group was lower than that in miR-NC group (P ＜ 0.05);the expression level of RUNX1 protein in transfected miR-215-mimic cells was lower than that in transfected miR-NC cells (P ＜ 0.05).Cell proliferation,migration and invasion in si-RUNX1 group were all lower than those in si-NC group (P ＜ 0.05).There was a negative correlation between mRNA expression level of miR-215 and RUNX1 in RB tumor tissues.Conclusions During the occurrence and development of RB,the down-regulation of miR-215 expression can promote malignant progression of tumor by targeting RUNX1.miR-215 can be used as a biological markers and therapeutic target for RB diagnosis.

Objective To study the quality of life (QOL) of patients with ductal carcinoma in situ (DCIS) and to analyze the relevant factors affecting their QOL.Methods A total of 84 patients with DCIS and 125 patients with invasive breast cancer were surveyed.Researchers used SF-36 to assess the QOL of participants at one year after operation.The relationships between some information of patients and SF-36 score were analyzed,such as age,the type of surgery,endocrine therapy,education,marital status,working status and health insurance.Results Compared to normal women,patients with DCIS had lower QOL in physical function (t =2.468,P =0.029),bodily pain (t =2.076,P =0.039),general health (t =2.153,P =0.033) and mental health (t =3.396,P =0.003).Patients with invasive breast cancer also had poorer QOL in physical function (t =5.638,P =0.002),bodily pain (t =5.417,P =0.002),vitality (t =4.438,P =0.002),general health (t =3.960,P =0.002) and mental health (t =6.020,P =0.001).QOL of DCIS patients was similar to that of invasive breast cancer patients,except that scores of physical function (t =2.714,P =0.032) and vitality (t =2.134,P =0.040) were better in DCIS patients.Endocrine therapy significantly affected the score of QOL of DCIS patients.DCIS patients with endocrine therapy had poorer score in physical function (t =2.082,P ＜ 0.05),bodily pain (t =2.003,P ＜ 0.05),general health (t =2.751,P ＜0.05),vitality (t =2.048,P ＜ 0.05) and mental health (t =4.162,P ＜ 0.05).Conclusion Patients with DCIS have poor QOL at one year after operation.Endocrine therapy significantly reduces their QOL.

Objective To analyze the clinical features, diagnosis and treatment of X-Linked Agarnmaglobulinemia (XLA). Methods Clinical features, cellular and humoral immune functions, treatment and prognosis from 3 patients with XLA were retrospectively reviewed. Results The age of onset were from 11 months to 6 years in these 3 cases, however, the median age of diagnosis was 12 years. All patients showed multiple recurrent bacterial infections, arthritis involved large joints such as knee, ankle, elbow and hip. Laboratory examination revealed the decrease of serum gammmaglohulin and absence of B lymphocytes in the peripheral blood. All 3 patients were identiifed BTK mutations, which were frameshift mutation and nonsense mutation in exon 3, frameshift mutation in exon 10, missense mutation in exon 18. After XLA was diagnosed, the patients were managed by intravenous gammagloulin (IVIG) replacement. The non-steroidal anti-inflammatory drugs (NSAIDs) were administrated in patients combined arthritis. The small dose of hormones had been applied. All patients had a significantly improvement. Conclusions The clinical features of XLA have greater variability, with recurrent bacterial infections. Markedly decreased and absent tosils and lymph nodes, serum immunoglobulin may be one of the warning signs for early diagnosis of XLA. IVIG and NSAIDs can be jointly treatment of XLA with arthritis. The steroid and immunosuppressant agents should be used with caution.

Objective:To develop a biotin-streptavidin system (BAS)-mediated folate receptor (FR)-targeted quantum dot (QD) fluorescent probe and preliminarily validate the targeting ability and signal amplification effect of the probe. Methods: Streptavidin (SA) was covalently coupled with QD through the active ester method;the physical characteristics of the prepared QD-SA were veri-fied. Biotinylated folate was synthesized through the carrier bovine serum albumin using the same method and then reacted with QD-SA to form the special probe. The probe was used to identify SKOV3 cells and FR-negative A549 cells to verify its targeting speci-ficity. QD-SA was used as the contrast. SKOV3 cells were imaged using the BAS-mediated FR-targeted QD probe with a biotinylated folate incubation time of 1 or 4 h. Various reaction times were also tested between the probe and the QD-FA that was formed without BAS mediation. Results:The BAS-mediated FR-targeted QD probe specifically recognized FR-positive SKOV3 cells. The probe ob-tained higher fluorescent intensity after 4 h than after 1 h of biotinylated folate incubation. The BAS-mediated FR-targeted QD probe al-so had a stronger fluorescent signal than the QD-FA probe. Conclusion:The proposed probe presents a great potential in the early diag-nosis of ovarian cancer because of its high specificity and sensitivity.

Objective To observe the muscles and skeleton involvement surrounding sacroiliac joint (SIJ) of axial seronegative spondyloarthropathy (SPA) patients with magnetic resonance imaging (MRI),and to analyze the relationship between them.Methods A prospective study of 38 patients who meet the 2009 axial SpA diagnostic criteria was conducted.We carried out MRI of the SIJ for these patients to evaluate the muscles and skeleton involvement.Those cases were divided into muscles differences between the two groups of image scores,including Spondyloarthritis Research Consortium of Canada (SPARCC) scores and Spondyloarthritis Research Consortium of Canada MRI Sacroiliac Joint Structural Score (SPARCC SSS).The extent of muscles edema in patients with sacroiliac joints was ranked into twelve grades from 0-12,and we did Spearman rank correlation test of muscles edema scope and two indexes.Results We found that 28 cases (73.68％) of the 38 patients had significant muscle involvement by analyzing the STIR sequence,and found erector spinae in 22 cases (57.89 ％)gluteal muscles in 13 cases (34.21 ％),iliacus muscle in 11 cases (28.95 ％),obturator muscle in 5 cases (13.16％),piriform muscle in 5 cases (13.16％) and other 4 cases (10.53％).SPARCC (t =2.28,P =0.03) and SPARCC SSS (t =3.37,P =0.00) were statistically different between the two groups.SPARCC (P =0.00,r =0.67) and SPARCC SSS (P-0.01,r =0.47) were positively correlated with the extent of muscles edema.Conclusions The muscles edema around sacroiliac joint is an important sign of axial SpA magnetic resonance imaging.Patients who had muscles edema tended to have more serious skeleton changes.

Objective To investigate the safety and effectiveness of using covered stent in endovascular isolation treatment of spontaneous peripheral artery dissection.Methods A total of 9 patients with peripheral artery dissection were collected.The dissectionwas located at iliac artery in 6 patients and at subclavian artery in 3 patients.Preoperative CT angiography was performed in all patients to determine the site and degree of the dissection,based on which the corresponding covered stents were prepared.After puncturing the femoral artery with Seldinger technique,angiography was performed to confirm the lesions in all patients,then,according to the angiographic findings the implantation of covered stent was carried out.Results Among the 9 patients,the dissections were located at the right common iliac artery in 3,at the right external iliac artery in one,at the left common iliac artery in 2 and at the left subclavian artery in 3.The length of the dissection was 0.9-3.3 cm with a mean of 1.7 cm.The distance between the initial part of arterial dissection and the target vessel opening was 0.5-5.6 cm with an average of 2.3 cm.The covered stent was successfully implanted in all patients.In one patient,the implanted stent covered the non-dominant left vertebral artery.During the procedure no obvious complications occurred.The patients were followed up for 3-32 months (mean of 18 months).No procedure-related complications were observed in all patients.Color Doppler ultrasound examination showed that all the implanted stents were unobstructed.Conclusion For peripheral artery dissection,endovascular isolation therapy by using covered stent is safe and effective.