Objective Through the application of recombinant human Ⅱ tumor necrosis factor-Fc function protein (rhTNFR:Fc) in the treatment of juvenile idiopathic arthritis (JIA) with randomized control study,clinical characteristic and clinical effect were summarized.Methods According to the randomized controlled principle,124 patients with JIA were divided into control group and treatment group.The basic treatment in two groups were one antirheumatic slow-acting drug,nonsteroidal drug,adrenal cortical hormone.There were no significant differences between clinical type and basic treatment in two groups (P ＞ 0.05).Sixty-two patients of JIA treated with rhTNFR:Fc by subcutaneous injection.The doses was 0.8mg /kg per week.There were 17 cases of oligoarthritis,15 cases of polyarthritis,30 cases of systemic arthritis in the treatment group and control group respectively.The basic antirheumatic drugs,nonsteroidal anti-inflamatory drugs ( NSAIDs),adrenal cortex hormone were allowed to continued.Clinical evaluation index included ACR Pedi 30,ACR Pedi 50 and ACR Pedi 70.The adverse drug reactions were recorded.Results The remission rate of ACR Pedi 30,50,70 in 2 weeks,one month,three monthes and six monthes were different in types of JIA patients in the treatment group ( P ＜ 0.05 ).The remission rate of systemic arthritis was lower than the other two groups of arthritis ( P ＜ 0.05 ).Only 44％ ACR Pedi 50 remission was achieved after three monthes medication in systemic arthritis and 41.7％ ACR Pedi 50,29.2％ ACR Pedi 70 were achieved after six monthes.The remission rate in the types of oligoarthritis and polyarthritis at different time points (2 weeks,one month,three monthes,six monthes) of ACR Pedi 30,50,70 were similar.After six monthes,more than 80％ reached ACR Pedi 50 remission,more than half of patients reached ACR Pedi 70 remission.Three cases of macrophage activation syndrome in systemic arthritis group was effective treated with rhTNFR:Fc.In the treatment group,2 cases of systemic arthritis appeared ache and discomfort after one week treatment,3 cases appeared repeated mild upper respiratory tract infection and diarrhea during treatment,including one varicella infection.The incidence of adverse reactions in the treatment group of systemic arthritis were 16.7％,Other 2 types of patients did not show adverse reaction during rhTNFR:Fc treatment.Conclusion rhTNFR:Fc has good effect on oligoarthritis and polyarthritis of JIA.The adverse reactions of six monthes were rare.The cases of systemic arthritis could reach some clinical remission,but need to guard against infection and the occurrence of adverse reactions.To whom did not respond to conventional therapy in systemic arthritis or systemic arthritis combined with macrophage activation syndrome,it could be considered with rhTNFR:Fc.

Objective To analyze the outcome of children with juvenile idiopathic arthritis (JIA) treated with recombinant human tumor necrosis factor receptor antibody fusion protein (rhTNFR:Fc) for 2 years,and to evaluate the long-term efficacy and safety as well as the related factors that affect the curative effect of rhTNFR:Fc.Methods Fifty-seven JIA patients treated with rhTNFR:Fc were followed up for 2 years.Clinical data were registered including age of onset,disease duration before rhTNFR:Fc treatment,disease activity assessment,medication before treatment,dosage regimen of infection or adverse reactions.Pearson Chi-Square statistical test and logistic regression model of binomial classification were used for statistical analysis.Results ①Twenty-two JIA cases completed 2-year therapy.Some were in the process of dosage tapering.Eight cases reached ACR Pedi 50,14 cases reached ACR Pedi 70.All of them were included in the clinical effectiveness analysis.Thirty-five cases withdrawal in 2 years because of disease remission or treatment failure or side effects or infection.Seven who withdrew and then maintained with DMARDs under the supervision of doctors were evaluated by assessment of ACR Pedi 70.They were stable at the end of 2 years,and were included for the clinical effectiveness analysis group.Nineteen cases were withdrew by the doctor because they failed to reach ACR Pedi 30 within 3 months.They were included in the treatment failure analysis.Seven cases were lost during the follow up.② The remission rate (ACR Pedi 50,70) of SO-J1A in 2 years was 33％.Oligoarthritis rate was 60％,while that of polyarthritis rate was 79％.The statistical analysis showed that different categories and RF level were significantly different in effectiveness and treatment failure (x2=31.6,P＜0.05; x2=5.488,P＜0.05).There was no significant difference in age,sex,duration before treatment,AKA,CCP,ANA between the two groups (P＞0.05).③ Logistic regression analysis showed that different categories of JIA and RF levels were correlated with therapy,P＜0.05.The relative risk (OR) value of different categories of JIA was 2.983 (P＜0.05).Negative RF might be the predictive factor for treatment response (OR =0.029,P＜0.05).④ Eight cases (2％) had recurrence and other adverse reations during treatment,the majority (7 cases) was SO-JIA.Conclusions ① The long-term therapeutic effect and safety for oligoarthritis and Polyarthritis by rhTNFR:Fc are better than SO-JIA.② Negative RF maybe the factor associated with favourable rhTNFR:Fc efficacy.③ The long-term safety of rhTNFR:Fc for SO-JIA is good.Physicians should be cautious to infection and other adverse reactions.

Objective To study the clinical outcomes and safety of immunoadsorption therapy for refractory autoimmune disease in children.Methods Three boys who suffered of severe autoimmune disease-one boy suffered of severe dermatomyositis and pulmonary infection; one suffered of severe anaphylactoid purpura with alimentary tract hemorrhage and entero ablation for intestinal perforation ; other one suffered of systemic juvenile idiopathic arthritis and severe prosopo-cellular tissue infection,macrophage active syndrome,were treated with blood immunoadsorption by resin immunoadsorbent of HA280.Then evaluated the clinical outcome of 3 cases,including symptom improvement,change of serum immune globulin,complement,enzyme of liver and heart,autoantibody.Results After the treatment of immunoadsorption,the symptom of 3 cases improved obviously; the sensitivity of the corticosteroids increased; autoantibody of antinuclear antibody (ANA) and anti-cyclic citrullinated peptide antibody (CCP) changed to negative; C-reactive protein (CRP) dropped (P ＜ 0.05) ; descending scale of IgM,IgA,C3,C4 increased (P ＜ 0.05) ; the normal scale of immunoglobulin didn't changed (P ＞ 0.05) ; besides aspartate aminotransferase (AST) dropped in the case of dermatomyositis,the other enzyme of liver and heart didn't changed.Conclusions The body could be restored quickly by the treatment of immunoadsorption together with the drug; CRP in the blood could be removed by immunoadsorbent of resin; 1 or 2 times blood immunoadsorption could not change the level of enzyme,but it need to do more on severe cases,especially those with poor organ function; for the safe of the treatment of immunoadsorption for the young age,low weigh and severe cases,the operative procedure should be critical care.

39℃)developed at the progressive stage of this disease.Physical examination showed variously sized,round or oval,atrophic and variola-like scars along with scattered erythematous patches,papules, necrosis and crusts on the face and extremities.The face was edematous,and there were some edematous and erythematous plaques with a necrotic center on the legs and arms.Histological examination revealed a massive infiltration with atypical CD8~+lymphocytes around the vessels and appendages in dermis.A diagnosis of CD8~+cutaneous T-cell lymphoma(CTCL)was made.Glucocorticoid and immunosuppressants were effective in controlling the condition.Up to the time of the writing,there has not been any definite evidence of systemic involvement.

Objective:To investigate the therapeutic efficacy of Golimumab in the treatment of children with refractory juvenile dermatomyositis(JDM).Methods:The clinical data of a child diagnosed with JDM in the Department of Allergy, Immunology and Rheumatology of Guangzhou Women and Children′s Medical Center in February 2019 were collected.The treatment effect was studied and literature review was conducted.Results:The patient was a 7-year-old boy with subacute onset of the disease.The illness protracted, and main manifestations included skin rashes, limb weakness, and swallowing dysfunction.Physical examination showed heliotropic rashes, Gottron papules, positive Gower, proximal limb muscle strength grade Ⅲ-Ⅳ, distal limb muscle strength grade Ⅳ, and a choking cough when swallowing fluid food.Laboratory tests revealed alanine aminotransferase (ALT) of 36 U/L, aspartate aminotransferase (AST) of 115 U/L, alkaline phosphatase of 69 U/L, lactate dehydrogenase of 941 U/L, creatine kinase of 974 U/L, hypersensitive C-reactive protein of 26 mg/L and an erythrocyte sedimentation rate (ESR) of 52 mm/1 h. Antinuclear antibody spectra were negative.Electromyography suggested myogenic damage.Thigh magnetic resonance imaging indicated diffuse abnormal signal shadows in the subcutaneous fat, muscles and muscle spaces of both hips, thighs and knee joints.The child was diagnosed with JDM, and given standardized treatment of Methylprednisolone, intravenous immunoglobulin, Methotrexate and Hydroxychloroquine sulfate.However, after the treatment, the facial rashes were still red, proximal limb muscle strength and swallowing dysfunction did not improve, the choking cough symptom still existed, and a Cushing face appeared.Recheck results showed ALT of 24 U/L, AST of 32 U/L, alkaline phosphatase of 56 U/L, lactate dehydrogenase of 216 U/L, creatine kinase of 527 U/L, hypersensitive C-reactive protein of 8 mg/L and an ESR of 15 mm/1 h. Refractory JDM was considered.After negotiating with the patient′s family members, they agreed to treat the patient with Golimumab 50 mg by subcutaneous injection once a month.Then tapered prednisone gradually, stopped Hydroxychloroquine sulfate tablets and continued to give the patient oral Methotrexate.After two doses of Golimumab 50 mg, proximal limb muscle strength and swallowing function improved markedly.After the third subcutaneous injection of Golimumab, proximal limb muscle strength improved to grade Ⅳ-Ⅴ, and he was able to go up and down stairs, squat and stand up after squatting.Besides, dysphagia and the choking cough disappeared, and skin rashes improved.Recheck results suggested a normal ESR and creatine kinase levels.Magnetic resonance imaging of thighs indicated no muscle inflammation.Conclusions:Golimumab works well in the treatment of refractory JDM and can effectively improve muscle strength.Therefore, it can be used as a treatment option for refractory JDM.

Objectives To search for the pathogenesis of vaseular endothelial injury and its clinical significance.Methods 36 patients with acute lower respiratory trect infection(ALRI)and 30 healthy controls were envolved. Circulating endothelial cells (CEC)and nitric oxide (NO) levels were tested.Results Circulating nitrite/nitra levels,the stable metabolic products of NO,were found to be significantly higher in the patients with ALRI (44.6?22.6umol/L) than that in the controls (24.5?14.1umoI /L, P

Objective To explore the prevalence of the nuclear dense fine speckled ( DFS ) immunofluorescence pattern in routine antinuclear antibodies(ANA) testing and its significance in patients with autoimmune diseases( AID) .Methods The ANA in 13 728 specimens were measured by indirect immunofluorescence( IIF) using HEp-2 cell slides from department of clinical laboratory, wenling hospital from 2011 to 2014.The frequencies, clinical manifestations and laboratory features of DFS positivity were restrospectively analyzed in patients with AID,usingχ2 test.Results ANA was positive in 20.56%(2 822/13 728) of the total patients, and the frequency of DFS pattern was observed in 1.81%(248/13 728) of the total patients and in 8.79%(248/2 822) of the patients with ANA positivity.In different age groups (≤20 years old, 21-49 years old and≥50 years old) , there were statistical significance of DFS pattern positive rate (male:χ2 =18.17,P<0.01; female: χ2 =1 500.00,P<0.01).And the highest frequency of ANA positivity was observed in patients from department of rheumatology(30.07%).The frequency of DFS pattern was higher in the departments of infection ( 32.58%) , dermatology ( 21.76%) , neurology ( 18.58%) and nephrology(6.73%) among the patients with ANA positivity(χ2 =123.00,P<0.01).Amony the 248 cases with DFS pattern positivity.41 cases were AID ( 16.53%) and 207 cases were non-autoimmune diseases ( 83.47%) . In AID group 13 cases were autoimmune thyroiditis ( 31.71%) , 12 cases were rheumatoid arthritis ( 29.27%) , 4 cases were autoimmune liver disease ( 9.76%) , 4 cases were undifferentiated connective tissue disease (9.76%), 3 cases were ankylosing spondylitis(7.32%), 2 cases were Sj?gren′s disease ( 4.88%) , 2 cases were inflammatory bowel disease ( 4.88%) and 1 case was systemic lupus erythematosus(2.44%).The titers of DFS in patients with AID were predominantly above 1∶320 and less than 1∶100 in non-AID.AID patients with DFS pattern positivity have different clinical manifestations and laboratory features.Howerer, antinuclear antibodies ( ANAs ) in 15 specific auto-antibodies were all negative.Conclusions The DFS pattern is a common pattern in ANA positivity patients and it mainly exists in non-AID patients.Further more, it is suggested that patients with DFS pattern identified by IIF should then be tested for anti-DFS70 antibodies with a specific immunoassay.

Objective:To study the influence of protein transduction domain (PTD)-oligomerization domain (OD)-HA fusion proteins on apoptosis of bcr/abl-positive cell lines. Methods:bcr/abl-positive cells were treated with PTD-OD-HA protein. The apoptoses of the cells were detected by flow cytometry (FCM), DNA ladder and transmission electron microscopy (TEM), and the levels of apoptosis-related genes bax and bcl-2 were detected by RT-PCR and Western blotting. Results:FCM examination demonstrated that PTD-OD-HA protein induced the apoptosis of bcr/abl-positive cells; DNA ladder showed that the classic DNA ladders appeared in BaF3-P210 and K562 cells after 48 h treatment with PTD-OD-HA proteins; the apoptoses of BaF3-P210 cells were observed by TEM; the levels of bax in mRNA and protein increased in BaF3-P210 and K562 cells, and bcl-2 decreased. Conclusion:PTD-OD-HA proteins specifically induced the apoptosis of bcr/abl positive cells.

Objective To explore the effect of risk assessment tracking management in reducing unplanned extubation in patients with tracheal intubation.Methods From January to December 2015,120 patients with tracheal intubation in ICU were selected as the control group with routine nursing care of indwelling endotracheal intubation.From January to November 2016,120 patients with tracheal intubation were selected as the observation group,where the risk assessment and risk management were done on the basis of routine nursing as in the control group.The two groups were compared in terms of tracheal intubation and unplanned extubation related knowledge,the implementation of the risk assessment,the accuracy in risk assessment and the incidence of unplanned extubation.Result The tracheal intubation and unplanned extubation related knowledge in the observation group was lower than that of the control group (P＜0.001),The implementation rate of the risk assessment and the accuracy in risk assessment in the observation group were significantly higher than the control group,and the rate of unplanned extubation in the observation group was significantly lower than in the control group (all P＜0.01).Conclusion The implementation on risk assessment and extubation-preventing nursing quality tracing management can enhance the awareness and knowledge of preventing trachea cannula exodus,improve the quality of nursing and reduce the rate of unplanned extubation.

Objective To investigate the correlation between serum homocysteine (HCY) and chronic heart failure (CHF) hypercoagulable state in patients.Methods A total of 105 cases of patients with CHF was divided into three groups according to the New York Heart Association (NYHA) classification standard functions:heart functional grade Ⅱ group (42cases),cardiac function grade Ⅲ group (35 cases) and,NYHA class Ⅳ group (28cases).At the same time,40 healthy individuals were regard as the control group.HCY,fibrinogen (Fbg),D-dimer (DDI),HCY,N-terminal pro-brain natriuretic peptide (NT-proBNP) were detected by fasting venous blood samples which were collected within 24 hours after admission.Results Compared to the control group,the expression of Fbg,DDI,HCY and NT-proBNP increased,whereas,antithrombin Ⅲ (AT-Ⅲ) was reduced.Fbg,DDI,HCY,NT-proBNP,and AT-Ⅲ were found in all patient cases.Four groups were compared with each other,except for cardiac function Ⅱ group and the normal group had no significant difference between them (P ＞ 0.05),the difference between both other groups was significantly different (P ＜ 0.05),HCY had a positive correlation with Fbg,DDI,and NT-proBNP (r =0.268,0.295,and 0.404,P ＜ 0.05),and negative correlation with AT-Ⅲ (r =-0.240,P ＜ 0.05).Conclusions HCY might be a reliable indicator as a judge of CHF patients with hypercoagulable state,to detect HCY,FBG,DDI,and AT-Ⅲ in CHF patients.It benefits for judging thrombosis risk and determining the severity of the diseases.Anticoagulant therapy might be beneficial to reduce the long-term adverse events.