The Report of macrophage activation syndrome in 9 children
- VernacularTitle:巨噬细胞活化综合征9例分析
- Author:
Xiao-Yan XIONG
;
Hua-Song ZENG
;
Hong-Wei WANG
;
Xiao-Pin LUO
;
- Publication Type:Journal Article
- Keywords:
Macrophage activation;
Juvenile idiopathic arthritis, systemic-onset, hemophagocytic lymphohistiocytosissis
- From:
Chinese Journal of Rheumatology
2000;0(06):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical characteristics of macrophage activation syndrome (MAS). Methods Clinical data was analyzed in 9 children who had been diagnosed as MAS in our hospital from Jan, 2003 to Aug, 2006. Results Seven children were boys, 2 children were girls, aged 5 months to 12 years. Clinical manifestations included long-term fever, hemophagocytic cell in bone marrow, anemia, arthritis, enlargement of lymph nodes, enlargement of liver and spleen, liver dysfunction, abnormal fat metabolism. Two cases had acute respiratory, distress syndrome(ARDS), 2 cases were complicated with multi- pie organ failure(MOF), two patients died. Glucocorticoid combined with immunosuppressive therapy were effective, HP(Plasma Exchange)was applied in one severe case and was shown to he effective. Conclusion MAS is a serious complication of JIA, especially in systemic-onset juvenile idiopathic arthritis. It is very im- portant to recognize and treat MAS earlier.
