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??Abstract?? Systemic vasculitis is a group of vasculitis with common inflammation of the blood vessel and involves multiple organs. It can involve all kinds of vessels of the organs??showing different clinical manifestations and lacking specificity. Through summarizing the clinical manifestation and diagnosis of some common systemic vasculitis in children??we aim to improve pediatricians’s awareness of these diseases??in order to early discover??diagnose and improve the prognosis.     

Be careful with triage in emergency departments: interobserver agreement on 1,578 patients in France

Background

For several decades, emergency departments (EDs) utilization has increased, inducing ED overcrowding in many countries. This phenomenon is related partly to an excessive number of nonurgent patients. To resolve ED overcrowding and to decrease nonurgent visits, the most common solution has been to triage the ED patients to identify potentially nonurgent patients, i.e. which could have been dealt with by general practitioner. The objective of this study was to measure agreement among ED health professionals on the urgency of an ED visit, and to determine if the level of agreement is consistent among different sub-groups based on following explicit criteria: age, medical status, type of referral to the ED, investigations performed in the ED, and the discharge from the ED.

Methods

We conducted a multicentric cross-sectional study to compare agreement between nurses and physicians on categorization of ED visits into urgent or nonurgent. Subgroups stratified by criteria characterizing the ED visit were analyzed in relation to the outcome of the visit.

Results

Of 1,928 ED patients, 350 were excluded because data were lacking. The overall nurse-physician agreement on categorization was moderate (kappa = 0.43). The levels of agreement within all subgroups were variable and low. The highest agreement concerned three subgroups of complaints: cranial injury (kappa = 0.61), gynaecological (kappa = 0.66) and toxicology complaints (kappa = 1.00). The lowest agreement concerned two subgroups: urinary-nephrology (kappa = 0.09) and hospitalization (kappa = 0.20). When categorization of ED visits into urgent or nonurgent cases was compared to hospitalization, ED physicians had higher sensitivity and specificity than nurses (respectively 94.9% versus 89.5%, and 43.1% versus 30.9%).

Conclusions

The lack of physician-nurse agreement and the inability to predict hospitalization have important implications for patient safety. When urgency screening is used to determine treatment priority, disagreement might not matter because all patients in the ED are seen and treated. But using assessments as the basis for refusal of care to potential nonurgent patients raises legal, ethical, and safety issues. Managed care organizations should be cautious when applying such criteria to restrict access to EDs.     

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??OBJECTIVE To discuss the effect and mechanism of cinepazide maleate (CM) on myocardial injured rats induced by isoproterenol (Iso). METHODS Fifty male and female rats were divided into normal group, model group, the low and the high dose group of the CM, and captopril (Cap)positive control group. The rat model of myocardial injury was established by subcutaneous injection of Iso (5 mg??kg^-1??d^-1)for 7 d, rats in treatment group were given intraperitoneal injection of CM on the second day (1.5,3 mg??kg^-1??d^-1), rats in positive control group were given intragastric administration of Cap(10 mg??kg^-1??d^-1), and rats in normal group and model group were given intraperitoneal injection of normal saline in the same volume, for 14 d. The rats in each group were tested before and after treatment by three times of running endurance test. After stopping drug all rats were anesthetized for measuring the electrocardiogram (ECG), then taken blood for measuring the activities of serum SOD, LDH and CK, and taken hearts for measuring heart weight index (HWI), left ventricular mass index (LHWI), the contents of myocardial hydroxyproline (Hyp)and MDA, and observing the morphological changes of myocardial tissue by HE staining. RESULTS Compared with rats in normal group, rats in model group showed endurance value decrease, ECG abnormalities (arrhythmia and myocardial ischemia in waveform), increased HWI, LHWI (P<0.01), myocardial Hyp content, myocardial MDA level, and the level of serum LDH and CK, and reduced the serum SOD activity (P<0.05 or P<0.01). Compared with model group, CM could significantly increase endurance value, improve abnormal phenomenon of ECG, lower the HWI, LHWI, myocardial Hyp content, myocardial MDA level, and serum LDH and CK levels were lower, and serum SOD activity increased, especially in high dose group of CM (P<0.01). HE staining showed in the model group rat ventricular remodeling, myocardial rupture, a large number of collagen fibers, in the treatment group, ventricular remodeling and myocardial fibrosis were significantly improved. CONCLUSION Cinepazide maleate has protective effect on myocardial injury of rats induced by Iso.     

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??Abstracts?? Objective To study the T2* magnetic resonance imaging for evaluation of iron deposition of liver?? pancreas and heart in β- thalassemia major ??β-TM?? patients and the relationship of the serum ferritin with iron overload. Methods Measurement of hepatic?? pancreatic and cardiac MRI T2* was performed in 153 patients with β-TM from May 1?? 2010 to December 31?? 2010??The demographics and serum ferritin ??SF?? were collected. The rate of the iron overload in liver?? pancreas and heart was analyzed. The rates of combined pancreatic and cardiac iron overload in different hepatic iron overload groups were compared. Results According to MRI T2*?? there was serious hepatic iron overload in 85 cases?? moderate in 39?? mild in 21?? no iron overload in 8. Similarly?? there was pancreatic iron overload in 134?? no iron overload in 19. In terms of cardiac iron overload?? there was serious cardiac iron overload in 37 cases?? mild in 21?? no iron overload in 95. No correlation was found between hepatic?? pancreatic and cardiac MRI T2* and age or SF?? but hepatic MRI T2* correlated with pancreatic and cardiac MRI T2*??P = 0.000?? r = 0.529?? r = 0.369???? and pancreatic MRI T2* correlated with cardiac MRI T2* well ??P = 0.000?? r = 0.715??. As grade of hepatic iron overload increases?? the patients demonstrated higher rate of combination of pancreatic and cardiac iron overload simultaneously ??χ2 = 20.78??P = 0.000??.     

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??Abstract??Objective To explore the role of miR-125b in pediatric classical APL?? in order to seek new therapeutic strategies for drug resistant APL. Methods The target genes of miR-125b were predicted online?? validated by Dual-luciferase assay and western blot assay. MiR-125b expression levels were measured in 33 matched-pair APL samples??treated in the First Affiliated Hospital of Sun Yat-sen University and other members of South China Children APL Cooperative Group from March 2007 to September2012??at initial diagnosis and complete remission ??CR?? and in 5 relapsed patients by qRT-PCR. Proliferation and apoptosis were analyzed respectively using the RNA transfection?? MTT assay and flow cytometry. Results The expression of miR-125b was up-regulated in pediatric APL at diagnosis and relapse bone marrow samples?? but returned to normal after complete remission?? miR-125b could promote leukemic cell proliferation and inhibit cell apoptosis by regulating the expression of tumor suppressor Bak1. Remarkably??it was also found to be up-regulated in leukemic drug-resistant cells??NB4-R1??NB4-R2??HL-60/DOX???? and overexpression of exogenous miR-125b could increase their resistance to therapeutic drugs. Conclusion MiR-125b can regulate pediatric classical APL cells proliferation?? apoptosis and drug resistance by repressing BAK1 protein expression.     

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??Abstract?? Objective To study balloon dilatation with cryotherapy under bronchoscope for subglottic stenosis in infants and young children??and to explore the efficacy of this new technology??the timing of treatment and safety. MethodFrom July 2009 to May 2014 in 28 SGS cases aged from 1 month to 3 years?? bronchoscopic balloon dilatation treatment with CO2 cryotherapy was performed?? and different types of SGS treated by minimal invasi were assessed regarding efficacy, complications and outcome. Results The infant SGS were divided into ??°~??° by severity?? and soft membrane SGS and scarring SGS by the causes of stenosis. ??° SGS included 16 cases, in which 8 cases soft membrane SGS were treated by balloon dilatation effectively?? 9 cases after combined treatment; in scarring SGS balloon dilatation was effective in 1 case?? 4 cases after combined therapy; ??° SGS included 8 cases?? soft membrane SGS were treated effectively in 2 cases?? 4 cases after combined treatment; in scarring SGS balloon dilatation was effective in 1 case?? 2 cases after combined therapy; ?? °SGS included 4 cases?? 1 case of soft membrane SGS and 1 case of scarring SGS responded well to integrated treatment .Conclusion MSCT and bronchoscopy assessment is used in grading the extent and causes of stratification in infants with subglottic stenosis?? and then we can select the appropriate treatment programs to improve the prognosis of the disease and improve life quality of SGS children.     

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??Abstract?? Objective To identify the clinical features and the prognosis of anti-NMDAR encephalitis in children??so as to improve the pediatrician’s awareness about this disease. Methods The clinical features??therapeutic regimen and prognosis of 17 children with anti-NMDAR encephalitis were summarized by a retrospective and follow-up study. Results Among the 17 patients with anti-NMDAR encephalitis??12 were males and 5 were females.Their age varied from 2 to 12 years??and the average age was 5 years 8 months.The most common initial symptoms were convulsions ??7 cases?? and psychiatric symptoms ??7 cases??.The common symptoms in the course of the disease were psychiatric symptoms??17 cases????sleep disorders ??16 cases????extrapyramidal symptoms ??14 cases????and convulsion ??11 cases??.Only 4 patients had autonomic symptoms.The anti-NMDAR antibody were found in all the patients’CSF.The EEG test of 16 patients showed diffused slow background.Tumors were not found in any patient. Fourteen cases were treated with IVIg and methylprednisolone??2 cases only received IVIg therapy and 1 case gave up.Rituximab ??2 cases?? and cyclophosphamide ??1 cases?? were administrated in 3 cases with no improvement after the above treatment.The patients had been followed up for 3 to 15 months.Twelve of them recovered completely??2 cases died??2 cases had epilepsy??and 1 case had speech disorder. Conclusion Psychiatric symptoms??sleep disorders??extrapyramidal symptoms and convulsion are the common symptoms of anti-NMDAR encephalitis in children.Tumors and autonomic nervous system dysfunction rarely happen. Immunotherapy is effective in the majority of the patients.The prognosis of anti-NMDAR encephalitis in children is optimistic.     

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??Abstract?? Objective To investigate the diagnosis and management of congenital supravalvular aortic stenosis. Methods Data of 26 cases is collected and analyzed retrospectively?? among which there were mental retardation in 10 cases??growth retardation in 12 cases??positive family history in 2 cases and other heart abnormalities in 4 cases. Results Local supravalvular aortic stenosis was proved in 26 cases by thoracic echocardiography. Catheterization and angiography was performed in 2 cases??and one patient died because of respiration and circulation failure after angiography. Computed tomography angiography was done in 12 cases. Surgical operation was conducted in 13 cases??and 11 patients recovered uneventfully. Of the other two patients??one died during operation??and the other was complicated with brain problem. Conclusions Echocardiography is the basic method for diagnosis; computed tomography angiography and/or catheterization is needed necessarily in establishing diagnosis. Surgical operation is feasible in correcting supravalvular aortic stenosis with satisfied recovery in short-term follow-up??and sometimes pulmonary artery plasty performed if necessary.     

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??Abstract?? Objective Toinvestigatethe correlation among exhalednitric oxide??lung function and Asthma Control Test inasthmatic childrenand the clinicalsignificance. Methods A total of 136 cases of asthmatic children aged 5 to 15 years old were recruited from Shengjing Hospital of China Medical University.The fraction of exhaled nitric oxide?? lung function and ACTscore were measured??and the correlation among them was analyzed. Results Negative correlation was found between FeNOand ACT??r= -0.251??P<0.005??.Negative correlation was found between FeNOandFEV1/FVC of lung function??r=-0.206??P<0.05????but not FEV1or PEF.Positive correlation was found betweenACT scoreandFEV1/FVC of lung function ??r=0.242??P<0.01???? but not FEV1or PEF. Conclusion For all subjects??FeNO is negatively correlated with ACT score and FEV1/FVC.ACT is positively correlated with FEV1/FVC.When the subjects are grouped??the three don’t have stable correlations.FeNO is a good biomarker to evaluate the airway inflammation of asthmatic children.ACT score evaluates the level of asthma control in children.Lung function evaluates airway obstruction.FeNO??lung function andACTare different measurements that evaluate asthma in different ways.None of them can be used instead of another in clinical practice??and they are complementary.Combinedapplicationscan effectivelyimprove the diagnosis??treatment and asthmacontrol inasthmatic children.     

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??Abstract??Allogeneic hematopoietic stem cell transplantation ??HSCT?? is the only curative therapy for severe beta-thalassemia major. Patients with an available human leukocyte antigen ??HLA?? identical sibling donor should be offered HSCT as soon as possible before development of iron overload and transfusion associated complications?? and the cure rate was up to 80%??90%. High-resolution HLA typing matched unrelated donor transplants also achieved good results. HSCT from HLA-mismatched relatives or HLA-phenotypically-identical donor is an option to be performed in expert centers. Optimization of conditioning regimen and techniques to control transplant-related complications will further improve outcomes.