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Wysłany: Wto 4:04, 15 Mar 2011
Temat postu: mbt scarpe yfu nil lnj etj
Juvenile idiopathic arthritis, systemic activation of macrophages merger syndrome
Children are still hot, the highest temperature 38.8 ℃. Day 2 increased jaundice, liver and spleen were increased, the liver under the right costal 3cm, Xiphoid 5cm, quality tough side of blunt splenic ribs 2cm, positive to the continuous blood purification (CBP) treatment of 3d,
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, electrolytes returned to normal and to plasma exchange (PE) 1 times. Since then, significant improvement in children with mental reactions, temperature is normal, jaundice reduced aminotransferase decreased, platelets returned to normal, the condition has stabilized. After 6 weeks serum enzyme spectrum and hematological examination was normal, but increase the number of swollen joints, low-dose prednisone maintenance therapy for 2 weeks, joint symptoms disappeared. 2 discussion soJIA common rheumatic diseases of children, and adult rheumatoid arthritis is different, the disease is heterogeneous. MAS is a special children with chronic rheumatic disease and serious complications, can be found in juvenile idiopathic arthritis (JIA),
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, systemic lupus erythematosus (SLE),
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, Kawasaki disease, juvenile dermatomyositis and other rheumatic diseases, especially common in soJIA children. MAS program will now hemophagocytic lymphohistiocytosis in the histiocytosis (HLH) A 2004] classification, but retained the name associated with autoimmune diseases, MAS (AID-MAS). The patients met the diagnostic criteria for HLH a 2004. The pathogenesis of MAS is not clear, the body may be related to NK cell killing function disorders, low levels of perforin defects and related to pathological immune macrophages of control over activation; when the virus infection, especially f) is , the body can quickly produce very similar with the HLH excessive immune response. The patients had a number of pathogenic or serum immunological tests positive pathogens, including CMV and HPVB, often in a latent state, when the host immune status showed lower replication. Experimental method to remove the specificity of the factors and HAV, HSV hepatotropic of differences between the two can lead to MAS. There is no uniform standard for treatment of MAS, but many advocates should be individualized and constantly adjust the phase of treatment. The particularity of the AID-MAS, that is the basis of autoimmune diseases occur HLH, therefore,
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, treatment should take into account the primary disease and HLH. When the body is in a serious life-threatening multiple organ injury time,
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, CBP can improve the immune suppression status and condition, clear effects of cytokines and regulate immune function iv, to improve the coagulopathy of severe liver damage and useful. This example is the rapid progress of the disease, medication for the best platform and opportunity, the implementation of a 2004 program of HLH United CBP + PE, making treatment a success.
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