Clinical Analysis For 9 Children Of Macrophage Activation Syndrome Complicating Kawasaki Disease

Objective:To summarize and analyze the clinical data of macrophage activation syndrome complicating kawasaki disease(KD-MAS),to provide possible clues for the diagnosis and treatment of the disease.Methods:The data of 9 children with KD-MAS in our hospital from Jan1993 to Dec 2017 were collected and analyzed.The clinical manifestations,diagnosis,treatment and prognosis of children were summarized and analyzed.Results:1.Among the 9 children,there were 6 males(66.7%)and 3females(33.3%).There were 8 cases(89%)at the age of less than 5 years old and 1 case at the age of over 5 years old.2.The duration of fever was 6~60days,with an average of 32.33±22.60 days,which was significantly higher than that of general KD(7~14 days).The IVIG treatment for the first time was 5~22 days,with an average of 11.83±6.05 days,which was longer than that of the general KD.All patients showed IVIG resistance.3.Most of the patients didn’t show cervical lymphadenopathy,and were not consistent with the diagnostic criteria of PLT≥450×10~9/L after 7 days at the course of the disease.All patients’Ferritin>1500 ng/ml.Bone marrow examination:positive:5/8 cases(62.5%),negative:3/8 cases(37.5%).Complete KD:3cases;incomplete KD:6 cases.Coronary artery dilatation:positive 7/9cases(77.8%),negative 2/9 cases(22.2%).4.Among the 6 patients who had knowable prognosis,4 cases(67.7%)survived,and 2 cases(33.3%)died.Conclusion:The occurrence of KD-MAS is very rare,but the mortality is high.The number of diagnostic examples of KD-MAS has been rising in recent years.Compared with patients over 5 years old,the number of patients less than 5 years old is more.Patients with KD who are beyond the usual duration of fever and show IVIG resistance should be especially alert to the occurrence of MAS.No cervical lymphadenopathy,PLT<450×10~9/L after 7 days at the course of the disease and ferritin>1500 ng/ml may be characteristic for KD-MAS.A considerable proportion of KD-MAS patients in clinical not have been identified correctly.