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Juvenile idiopathic arthritis (JIA) is a genetically heterogeneous group of connective tissue diseases that are commonly characterized by chronic joint synovial inflammation with unknown etiology in childhood.It is cu-rrently incurable and the main therapeutic goal is to achieve clinical remission.The drugs currently used to treat JIA mainly include non-steroid anti-inflammatory drugs, glucocorticoid, disease modifying antirheumatic drugs, and biological agents.In this article, recent advances in the understanding of JIA treatment and related clinical research were reviewed, in an attempt to provide prospects for the future direction of drug development and treatment concepts.
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Objective:To investigate the efficacy and side effects of combination of methylprednisolone pulse (MDP)and mycophenolate mofetil(MMF)in the treatment of systemic lupus erythematosus(SLE)in the children.Methods:A total of 16 cases of children with SLE,lupus nephritis(LN)and type Ⅳ diffuse glomerular mesangial proliferative glomerulonephritis diagnosed by pathology were selected.Among them 7 cases were given MDP combined with MMF,and received intermittently oral small dose of corticosteroids(GC),and they were used as pulse therapy group;9 cases were given oral GC and transitional reduction,and they were used as traditional therapy group.SLEDAI was used for the evaluation of the curative effect,and body mass index(BMI),blood pressure(BP),intraocular pressure(IOP),triglycerides(TG),fasting blood glucose(FBG)and serum calcium (Ca)were analyzed during the treatment of 1 year and then the efficacies and side effects of the children in two groups were compared.Results:The SLEDAI scores,levels of complements C3 and C4,24 h urinary protein outcome of the children in pulse therapy group were better than those in traditional therapy group;the differences in SLEDAI scores were statistically significant after treating for 3 and 6 months between two groups(P<0.05);ESR and 24 h urinary protein outcome had significant differences after 6 months of treatment between two groups(P<0.05);the complement C3 difference was statistically significant between two groups(P<0.05)after 12 months of treatment.Compared with traditional therapy group,the BMI,IOP,TG,FBG of the children in pulse therapy group after treatment were decreased(P<0.05);the BMI,IOP and TG had significant differences after treating for 12 months between two groups(P<0.05);the differences in FBG were statistically significant after treating for 6 and 12 months between two groups(P<0.05).The Ca of the patients in pulse therapy group was higher than that in traditional therapy group,but there was no statistically significant difference(P>0.05).The SPB and DBP of the patients in pulse therapy group were higher than those in traditional therapy group,but the differences were not statistically significant(P>0.05).At the same time,gastrointestinal ulcers,bleeding,perforation, pancreatitis,cardiovascular events (such as cardiac arrhythmias)didn't occur in two groups. Conclusion:Compared with traditional therapy,the combined treatment of MDP and MMF can control the symptoms of SLE early and rapidly,and reduce the viscera damage.To choose 1 year after treatment as observation point,its disease activity is lower than the traditional therapy,and the curative effect is better than oral GC transitional reduction with immunosuppressant therapy.The GC-related side effects are lower than traditional therapy.
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IgA vasculitis (IgAV) is a common systemic small-sized vessel vasculitis.It's characterized by non-thrombocytopenic palpable purpura,arthralgia/arthritis,bowel angina,and nephritis,but some details of etiology and pathogenesis is not very clear.The disease course is usually benign and self-limited,however,it is necessary to discuss the therapy of severe or chronic cases,especially there is not enough evidence-based basis for using key drugs to IgAV.Excellent clinical trails is expected to establish scientific system of prediction and evaluation of disease course,which will be helpful to individualize treatment of IgAV.
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Objective To investigate the coronary arterial lesions (CAL) in Kawasaki disease (KD) in Jilin Province and Inner Mongolia Autonomous Region from 1999 through 2012 and evaluate the influence of glucocorticoids (GCs) on prognosis.Methods We performed a retrospective study among patients of KD in Jilin Province and Inner Mongolia Autonomous Region from 1999 to 2012,in order to observe CAL in KD,the incidence of sequelae and the influence of CGs on disease outcome.Comparison between groups was carried out by Chi-square test.Results Eight hundred and ten KD cases were reported from 32 hospitals and 787 patients accepted an Echo examination,as the main objective of this study.Fivehundred and forty were boys and 247 were girls,with the male to female ratio of 2.19:1,of which 498 cases (63.3％) had CAL.Most CAL occurred in patients at 58 day to 14 year old (2.7±2.4).The most common abnormality was CAL including dilatation (49.5％) and stenosis (13.4％).Compared incomplete to complete KD,the result showed that the occurrence of CAL was similar between these two groups,no significant differences could be found between different gender.Nineteen (2.4％) of them had cardiac sequelae and the male/female ratio was 3.67:1.The younger the age,the higher the incidence of cardiac sequelae.The most frequent cardiac sequelae was coronary artery dilatation.GCs therapy could increase the occurrence of CAL [80.2％(97/121) vs 62.6％ (419/666),x2=8.71,P＜0.05].Conclusion CAL is not different between patients with complete or incomplete KD and the occurrence of CAL is not associated with gender,but with age.The younger the age,the higher the incidence of CAL and the higher the incidence of cardiac sequelae.It is possible that patients treated with GCs are prone to CAL.
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Studies have indicated approximate 50% cases of coronary heart disease are closely associat-ed with genetic factors and it. It is well evidenced that lipid abnormality is the important risk factors of coronary heart disease. Many single nucleotide polymorphisms that influence levels of blood lipids were found and con-firmed in recent years. The article summarized the single nucleotide polymorphisms associated with the level of lipid.
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Two sets of monozygotic [MZ] twins with Kawasaki disease [KD] from two different families are reported. Twin set 1, previously healthy 71-day-old MZ twin girls were diagnosed with incomplete KD and pneumonia. The symptoms occurred at the same time in both girls. Both girls had ectasia of right coronary arteries. In twin set 2, the younger of 18-month-old MZ twin boys was diagnosed with KD and bronchitis. After 53 days, his elder brother was diagnosed with the same disease. The symptoms occurred at different time, but were almost identical. Neither boy displayed coronary artery changes. These findings support the hypothesis that genes susceptible to KD and coronary-artery lesions may exist in families. The different clinical characteristics among MZ twins from different families also suggest diverse and complex nature of KD
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Humans , Male , Female , Twins, Monozygotic , Environment , Pneumonia , Bronchitis , Genetic Predisposition to DiseaseABSTRACT
Background: Hereditary ataxia is a group of hereditary diseases that are characterized by chronic progressive uncoordinated gait and are frequently associated with cerebellar atrophy.Objectives: To investigate evidence-based diagnosis of hereditary ataxia by retrospective analysis of the diagnostic process in one Chinese family.Methods: Clinical records of 15 ataxia patients from one Chinese family with 46 family members were retrospectively reviewed and a tentative diagnosis was made based on clinical manifestations, signs and symptoms, mode of inheritance, and progression. Since hereditary ataxia is a group of heterogeneous diseases having various subtypes and overlapping symptoms, we adopted a stepwise evaluation to achieve a tentative diagnosis. To confirm the diagnosis, we performed polymerase chain reaction (PCR) specific for the suspected causative gene of spinocerebellar ataxia (SCA) subtype 3 (SCA3).Results: Through analysis of hereditary and clinical characteristics of family histories of the patients, we suspected that the family might suffer from SCA, especially, SCA3. The PCR assay for SCA3 showed that, five of the ten samples analyzed had a CAG trinucleotide expansion of the SCA3 gene, and four of the five members developed ataxia. The remaining one, a seven-year-old girl, showed no symptoms or signs except for uvula deviation. No clinical symptoms were found in five other members with negative PCR results. Thus, based on both clinical findings and laboratory results, we further confirmed that the family suffered from SCA3.Conclusion: Hereditary ataxias are disorders sharing overlapping symptoms. Comprehensive analysis of medical and family records together with genetic diagnosis improves diagnostic efficiency of hereditary ataxia and aides in family counseling.Keywords: Cerebellar ataxia, diagnosis, heredity, PCR
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Objective To understand the cause of chest pain (precordium indisposition) in children and explore the thread of thought in its analysis.Methods Clinical data of 314 children with chest pain who visited the cardiovascular department for children of our hospital were collected and analyzed to study its causes.Results The cause could be attributed to organic cardiac diseases in 43 of 314 children (13.7%) with chest pain,to non-organic cardiac diseases in 108 (34.4%),to psychiatric-psychological factors in 60 (19.1%) and to unknown cause in 41 (13.1%).Average age of children with organic cardiac diseases was younger than that of children with other causes (P<0.05).Symptoms accompanied simultaneously in children with organic cardiac diseases were much more frequent significantly than those in them with non-organic cardiac chest pain and unknown causes (P<0.05).Conclusions Chest pain in children could be caused by a few causes,which was non-specific symptom for organic cardiac diseases,so, more should be taken into account in analyzing the cause of chest pain in children.