Hereditary Autoinflammatory Diseases ; Humans

Familial mediterranean fever (FMF) is a hereditary inflammatory disorder,which caused by mutations in MEFV gene located on short arm of Chromosome 16 p13.3.The majority of patients onset before the age of 10 years,which is characterized clinically by the episodes of inflammatory reaction and serositis,including fever,peritonitis,synovitis and pleurisy.AA-amyloidosis with kidney failure is the most important complication.The children patients were diagnosed mainly on clinical evaluation and the criteria developed by Yal(c)inkaya.The ultimate goal of treatment in FMF is to obtain complete control of unprovoked attacks and minimise subclinical inflammation in between attacks.The only agent that decreases the frequency and severity of the episodes and the development of amyloidosis is colchicine,which should be startcd as soon as a clinical diagnosis is made,alternative biological treatments such as IL-1 receptor antagonist are indicated in patients with non-responders or resistant to colchicine.

Juvenile systemic sclerosis (SSc) is a chronic connective tissue disease characterized by symmetrical thickening and hardening of the skin,associated with fibrous changes in internal organs.In the last years,several therapies have showed promising beneficial effects in the treatment of SSc.We summarized the recent advances in the anti-inflammation and immunosuppressive therapy,treatment of SSc related vascular diseases,anti-fibrosis therapy as well as prospective treatments.

Following great progress on the pathogenesis of lupus nephritis (LN) and publication of many high quality (prospective,randomized,multicenter) clinical trials of glucocorticoids-plus-immunosuppressive interventions,the American College of Rheumatology (ACR),European League Against Rheumatism (EULAR)/European Renal Association-European Dialysis and Transplant Association (ERA-EDTA),and Kidney Disease:Improving Global Outcomes (KDIGO) published or renewed in succession in 2012 special guidelines or recommendations for screening,casedefinition,treatment and management of LN.The paper introduces the key contents to provide reference for the clinical practice.

Antirheumatic Agents ; therapeutic use ; Arthritis, Juvenile ; therapy ; Biological Factors ; therapeutic use ; Humans

Takayasu arteritis (TA) is a devastating vasculitis of the aorta and its major branches,coronary and pulmonary arteries.The clinical manifestations in children are less specific than in adnlts:the disease in children presents with fever,arthralgias,vomiting,weight loss and hypertension.Conventional angiography,which is recognized as the golden standard in evaluating vascular lesions in TA,combined with computer tomography angiography (CTA),magnetic resonance angiography (MRA),ultrasonography,could not only provide important information for early diagnosis,but also detect disease activity.New immunosuppressive agents and biological therapies,such as TNF-a blocking agents,have been verified to be effective although corticosteroids and conventional immunosuppressive agents are still basic treatment.

Consensus ; Humans ; Immunocompromised Host ; Immunologic Deficiency Syndromes ; Practice Guidelines as Topic ; Vaccination

Scientific research archives management work is a basic work in the hospital work,establish hospital scientific research management information system,implementation of scientific research archives management informationization can not only realize the scientific research resources sharing,and can greatly improve the scientific quality of service and efficiency.This paper analyzes the necessity of scientific research archives information management,and the main plate and the function of information management system of scientific research archives has carried on the detailed introduction.

Objective To explore the relationship of the plasma ox-LDL level and endothelium flow mediated vasodilation(FMD)in acute coronary syndrome patients(ACS).Methods From March to October 2004,in the Department of Cardiology,the Fourth PLA Hospital,51 patients with ACS were divided into 2 groups:AMI group(22patients)and UAP group(29 patients).20 subjects with normal coronary angiograms served as the control group.Nitrir oxide(NO)and plasma ox-LDL was measured by spectrophotometer and Sandwich ELISE method,respectively.The brachial arterial hyperemia-induced flow mediated vasodilation(FMD)and sublingual nitroglycerin(NTG)mediated vasodilation were measured by high resolution ultrasound.Results (1)The FMD in ACS group was significantly decreased compared with the control group[(7.2?1.42)、(7.6?1.12)vs(15.46?1.2),P0.05).(2)The No in ACS group was sigificantly lower than that in control group;the NO in AMI group was even lower[(48.46?12.44)、(60.42?10.30)vs(94.72?12.34)?mol/L,P1,which meant ox-LDL was a risk factor;the OR of FMD was

Objective To explore whether pioglitazone can ameliorate radiation-induced fibrosis in rat heart.Methods A total of 46 Sprague-Dawley rats were divided into six groups (control group, pioglitazone (Pio) 10 mg·kg-1 ·d-1 group, Pio 20 mg·kg-1 ·d-1 group, 18 Gy irradiation + placebo group;18 Gy irradiation + Pio 10 mg·kg-1 ·d-1 , and 18 Gy irradiation + Pio 20 mg·kg-1 ·d-1 group).Experimental animals were exposed to radiation at the chest, then administered Pio or placebo for one month.At 3 months later, the rats were killed and their heart tissues were collected for Masson staining, Western blot analysis and real-time polymerase chain reaction assay (real-time PCR).Results Masson staining revealed significant myocardial fibrosis in rats exposed to radiation, while these changes were reduced when the rats were given Pio.Western blot analysis showed that the PPAR-γ protein expression in the heart tissue of irradiated rats were higher than in the non-irradiated group (F =12.435, P ＜ 0.05).Real-time PCR assay showed that PPAR-γ mRNA expression in the irradiation + Pio 20 mg· kg-1· d-1 group was higher than that in the Pio 20 mg· kg-1 ·d-1 group (F =2.333, P ＜ 0.05).The expression of TGF-β1 protein in the irradiation + placebo group were higher than those in the other five groups (F =17.578 ,P ＜0.05), and the CDK5 protein expression had a high level in the three irradiated groups while only the irradiation + placebo group was statistically higher than controls (F =3.651, P ＜ 0.05).Conclusions Pioglitazone may ameliorate radiation fibrosis in the rat heart through its anti-fibrotic activity, perhaps via inhibiting Cdk5-mediated PPAR-γ phosphorylation.