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.

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.

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.

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 evaluate the effect and safety of conservative treatment for treating cervical spondylotic radiculopathy by cochrane evaluation systems.DATA SOURCE:CBM(from January 1978 to October 2008),CNKI(from January 1978 to October 2008)and VIP(from January 1989 to October 2008)databases were retrieved by computer and partial documents were searched by hands.DATA SELECTION:Randomized controlled trial was adopted in course of acupuncture and moxibustion treatment for cervicel spondylotic radiculopathy and its quailty was assessed.Dafa source was randomized and semi-randomized controlled.Some evaluations such as random method-selection bias,allocation concealment-selection bias,blind method-performance bias and measurement bias,exclusion.lose to follow-up,withdraw-attrition bias were chosen.MAIN OUTCOME MEASURES:①Abdominal acupuncture needles were superior to the conventional method's efficiency.②Electro-acupuncture plus cupping with barbed floc were superior to the conventional method's efficiency.③Abdominal acupuncture were superior to traction's efficiency.④Cervical traction,electro-acupuncture combined with point injection and massage were superior to traction's efficiency.⑤Traction,electro-acupuncture,massage,TDP-guided were superior to traction,physiotherapy,TDP combined with exercise therepy's efficiency.⑥Vinegar iontophoresis and electro-acupuncture were superior to simple electro-acupuncture's efficiency.⑦Long's approach acupuncture was superior to simple acupuncture's efficiency.⑧Adverse reaction.RESULTS:A total of 1416 subjects were selected from 7 randomized controlled trials.All the studies have selection bias,measurement bias,loss of the high probability of bias,the quality and grade of"C"grade.And only one study proved that the efficiency of the experimental group was better than that of the control group.Study 1 showed that there was insufficient evidence suggested that acupuncture treatment of abdominal cervical spondylotic radiculopathy was efficient than conventional acupuncture.Study 2 showed that there was insufficient evidence suggested that electro-acupuncture plus cupping therapy with barbed floc was efficient than conventional acupuncture to cure cervical spondylotic radiculopathy.Study 3 showed that there was insufficient evidence suggested that acupuncture treatment of abdominal cervical spondylotic rediculopathy was efficient than traction.Study 4 showed that the cervical traction,electro-acupuncture combined with acupoint-injection treatment of cervical spondylotic radiculopathy was efficient than traction combined massage therapy.Study 5 showed that there was insufficient evidence suggested that traction,electro-acupuncture,massage,TDP-guided therapy was efficient than traction,physical therapy,TDP combined with exercise therapy.Study 6 showed that there was insufficient evidence suggested thatelectro-acupuncture combined with vinegar iontophoresis treatment of cervical spondylotic radiculopathy was better than the simple electro-acupuncture therapy.Study 7 showed that there was insufficient evidence suggested that the acupuncture therapy using Long's approach was better than simple acupuncture.No adveme reactions appeared to terminate the test in 7 studies.CONCLUSlON:The above seven studies mainly present with low quality,absence of a unified curative method,small samples,difference of follow-up time,iudgment standard and measurement standard.So more samples,multicenter and more regular randomized controlled trial should be performed.