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Background: Juvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The anti-NXP2 antibody is one of the most common antibodies and muscle ischaemia associated with NXP2 autoantibodies was a severe subtype of JDM. Further information is needed regarding clinical characteristics and factors associated with poor prognosis. But there are no reports about clinical characteristics and high risk factor of poor prognosis. For the first time, we introduced the clinical characteristics and poor predictors of anti-NXP2 antibody-associated juvenile dermatomyositis in Chinese children. Methods: Twenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM Chinese patients were diagnosed from January 2016 to November 2019. Logistic regression was used to analyze the risk factors for refractory cases and mortality. Results: The ratio of male to female was 1:1.9. The median age of onset was 4.5 (1–13) years. Twenty-four cases (92.3%) had rash and muscle weakness. Treatments included glucocorticoids, immunosuppressive agents, biological agents (7 cases), plasma exchange, Janus kinase inhibitor (7 cases) and autologous stem cell transplant (1 case). Refractory JDM patients (11/26, 42.3%) were associated with edema, skin ulcer, muscle strength<=grade 3, CD4/CD8 ratio < 1.4 and ferritin > 200μg/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after autologous stem cell transplant (ASCT). The risk factors for gastrointestinal involvement and mortality were edema, skin ulcer, severe muscle weakness (dysphagia/ hoarseness/ soft voice), BMI < 15 and ANA positive. Conclusions: Edema, skin ulcer and severe muscle weakness predicted refractory disease, GI involvement, and mortality in anti-NXP2 antibody-positive JDM of Chinese children. Decreased CD4/CD8 ratio and high ferritin related with refractory cases, and very low BMI and ANA (+) are probably, associated with gastrointestinal involvement and mortality. Trial registration: http://www.chictr.org.cn/showproj.aspx?proj=49846. [ABSTRACT FROM AUTHOR]

The purpose of this study is to summarize the manifestations, diagnosis, differential diagnosis, and treatment of childhood brucellosis in non-epidemic areas of China. A retrospective review of 16 admitted children patients with brucella's disease who were diagnosed of brucellosis during the period from 2011 to 2016 was performed. Diagnostic criteria, clinical presentations, and outcomes were recorded. The most common symptom was fever. Osteoarticular involvement was found in 50% of the patients. They were infected by contacting with infected animals or consuming of unpasteurized milk or meat of sheep or goats, also. Standard agglutination test was positive in all patients and blood culture in 10 (62.5%) patients as well as medulloculture in 3 (18.8%) patients were positive. A combination of antibiotic treatment with rifampin plus cotrimoxazole showed good response and all clinical manifestations improved. Brucellosis is misdiagnosed frequently and should be considered in the differential diagnosis when patients do not respond to standard treatment. Blood culture, together with brucella serology test, is important and helpful in the diagnosis. MRI is a good method in differentiating those with symptoms of arthritis. [ABSTRACT FROM AUTHOR]