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Association between high mobility group box 1 protein and juvenile idiopathic arthritis: a prospective longitudinal study

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

Objective To analyze the levels of high mobility group box 1 (HMGB1) protein on different courses of juvenile idiopathic arthritis (JIA). Methods In o......

Adolescent-onset anti-MDA5 antibody-positive juvenile dermatomyositis with rapidly progressive interstitial lung disease and spontaneous pneumomediastinum: a case report and literature review

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundDermatomyositis with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody has a distinct phenotype associated with ......

Streptococcal infection in childhood Henoch-Schonlein purpura: a 5-year retrospective study from a single tertiary medical center in China, 2015-2019

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundThe present study focuses on the associations of streptococcal infection with the clinical phenotypes, relapse/recurrence and renal involvem......

Ruxolitinib treatment permits lower cumulative glucocorticoid dosing in children with secondary hemophagocytic lymphohistiocytosis

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundThis study aimed to analyze the effects of ruxolitinib on children with secondary hemophagocytic lymphohistiocytosis (HLH).MethodsEleven ped......

Factors affecting the duration of coronary artery lesions in patients with the Kawasaki disease: a retrospective cohort study

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

Background Coronary artery lesions (CALs) are the most severe complication of Kawasaki disease (KD). Approximately 9-20% of the patients with KD devel......

Predictive model based on gene and laboratory data for intravenous immunoglobulin resistance in Kawasaki disease in a Chinese population

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

Background Here, we investigated the predictive efficiency of a newly developed model based on single nucleotide polymorphisms (SNPs) and laboratory d......

Clinical characteristics and genetic analysis of A20 haploinsufficiency

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

Purpose To evaluate the clinical and genetic characteristics of 3 children with Haploinsufficiency of A20 (HA20). Methods:The clinical and genetic tes......

Predictive value of C-reactive protein to albumin ratio as a biomarker for initial and repeated intravenous immunoglobulin resistance in a large cohort of Kawasaki disease patients: a prospective cohort study

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundIntravenous immunoglobulin (IVIG) resistance prediction is one pivotal topic of interests in Kawasaki disease (KD). This study aimed to pros......

Integrin alpha 2 gene polymorphism is a risk factor of coronary artery lesions in Chinese children with Kawasaki disease

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

Background Kawasaki disease (KD) is a systemic vasculitis, and the formation of coronary artery lesions(CAL) is its most common sequela. Both genetic ......

Type I interferonopathies with novel compound heterozygous TREX1 mutations in two siblings with different symptoms responded to tofacitinib

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundType I interferonopathies are a group of rare autoimmune diseases characterised by excessive activation of type I interferon that leads to d......

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundTo summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile d......

Clinical characteristics and poor predictors of anti-NXP2 antibody-associated Chinese JDM children

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundJuvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The anti-NXP2 antibody is one of the most common antibodie......

Systemic inflammation and chronic kidney disease in a patient due to the RNASEH2B defect

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

IntroductionAicardi-Goutieres (AGS) is a rare immune dysregulated disease due to mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR1, or I......

Diagnosis and management of adenosine deaminase 2 deficiency children: the experience from China

期刊: PEDIATRIC RHEUMATOLOGY, 2021; 19 (1)

BackgroundDeficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease caused by mutations in the ADA2 gene. Few Chinese cases have ......

The association of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis

期刊: PEDIATRIC RHEUMATOLOGY, 2020; 18 (1)

Background Systemic juvenile idiopathic arthritis (sJIA) has many clinical features overlapping with familial Mediterranean fever (FMF), which is caus......

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