| Objective: By analyzing the clinical information of kawasaki disease complicated with macrophage activation syndrome(KD-MAS)to study its clinical characteristics.Methods: The clinical data of 14 cases of KD-MAS admitted to Shengjing Hospital of China Medical University from September 2007 to September 2019 were retrospectively analyzed and compared with the patients from published literature.According to the discharge outcome and compliance with the diagnostic criteria of hemophagocytic syndrome(HLH-2004),they were respectively divided into the poor prognosis group(death or abandonment of treatment)and improvement group,HLH-2004 group and non-HLH-2004 group.Results: 1.14 KD-MAS children in our hospital were included in this study,including 8 males and 6 females.The median age was 18.5 months.The mean onset time of MAS was 15.8 days.All the children had fever and rash.The incidence rate of cervical lymph node,liver and spleen enlargement was 85.7%(12/14),64.2%(9/14),42.8%(6/14),differentiatly.Coronary artery dilatation was found in 42.8%(6/14)of the patients.Laboratory index: white blood cell count(WBC):9 cases <4 109/L;Hemoglobin(Hb):12 cases <100 g/L;Platelet count(PLT):7 cases <100 109/L;ESR :5 cases <20mm/h;Triglyceride(TG):3 cases >3.0mmol/L;fibrinogen(Fib):8 cases <1.5g/L;Serum ferritin(SF): 13 cases >500ng/ml;ALT and AST were increased in all patients.The detection rate of hemophagocytic phenomenon was 91.6%(11/12).14 children all conformed to the diagnostic criteria of 2005 systemic juvenile idiopathic arthritis complicated with MAS [s JIA-MAS(2005)],11 children conformed to the diagnostic criteria of s JIAMAS(2016),7 children conformed to the diagnostic criteria of HLH-2004.All children were treated with IVIG,13 with glucocorticoids and 4 with etoposide(vp-16).Twelve patients improved,and two patients were discharged because of critical condition.The longest follow-up time of improved cases was 2 years,and no recurrence of MAS was observed.2.61 KD-MAS children were included in the comparative study,47 cases from literature retrieval and 14 cases from our hospital.There were 50 cases in the improvement group(12 cases in our hospital),11 cases in the poor prognosis group(2 cases in our hospital),Hb、 Fib in the poor prognosis group were lower than that in the improvement group,and the coincidence rate of HLH-2004 in the poor prognosis group was higher than that in the improvement group,with statistically significant differences(P<0.05).There were 44 cases in HLH-2004 diagnostic criteria group(7 cases in our hospital)and 17 cases in non-HLH-2004 diagnostic criteria group(7 cases in our hospital).The WBC、Hb、PLT、Fib in HLH-2004 group were lower than that in non-HLH-2004 group,TG、LDH higher than that in non-HLH-2004 group,and the incidence rate of coronary artery dilatation,hepatomegaly,splenomegaly and poor prognosis was higher than that in non-HLH-2004 group,with statistically significant differences(P<0.05).Conclusion: 1.MAS-related tests should be perfected and dynamically monitored when KD children are with IVIG unresponsiveness,hepatosplenomegaly.s JIA-MAS(2005)diagnostic criteria should be applied for early diagnosis,and individual treatment plans should be formulated according to the disease stage and severity.2.KD-MAS patients who meet the HLH-2004 diagnostic criteria are more worse and have a poorer prognosis.Hb and Fib levels are correlated with the prognosis of KD-MAS. |
