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Background <p>Kawasaki disease complicated with macrophage activation syndrome (KD-MAS) has the characteristics of low incidence, high rate of sequelae, and high mortality. We aims to explore early warning indicators that can be used for the diagnosis of KD-MAS, providing a reference basis for early clinical intervention.</p> Methods <p>The clinical data of 15 children with KD-MAS and 140 children with Kawasaki disease (KD) admitted to Jinan Children’s Hospital from January 2020 to March 2025 were retrospectively collected, and statistical analysis was conducted on the above data of the KD-MAS group and the KD group.</p> Results <p>There were no statistically significant differences between the two groups in terms of gender ratio, the proportion of incomplete Kawasaki disease, the incidence of coronary artery lesion, peripheral blood white blood cell(WBC) count, neutrophil ratio(N%), C-reactive protein(CRP), serum total bilirubin(STB) and serum potassium༈K<sup>+</sup>༉ level (<i>P</i> &gt; 0.05). The age of onset in the KD-MAS group was significantly younger than that in the KD group, and the hospitalization time was longer (<i>P</i> &lt; 0.05). Among the 9 KD-MAS children who completed bone marrow smear examination, 6 showed hemolysis phenomenon.The incidence of hepatosplenomegaly, organ damage, and insensitivity to intravenous immunoglobulin (IVIG) was higher in the KD-MAS group than in the KD group (<i>P</i> &lt; 0.05).The levels of serum ferritin(SF), alanine aminotransferase(ALT), aspartate aminotransferase(AST), lactate dehydrogenase(LDH) and triglyceride(TG) in the KD-MAS group were higher than those in the KD group, while the levels of hemoglobin(HB), platelet(PLT) count, serum albumin(ALB), erythrocyte sedimentation rate(ESR), fibrinogen(FIB) and serum natrium(Na<sup>+</sup>) level were lower than those in the KD group (<i>P</i> &lt; 0.05).The ROC curve analysis showed that the AUC values for indicators such as hepatomegaly, no response to IVIG treatment, SF, AST, FIB, PLT count, ALT, LDH, TG, and HB were all relatively high.</p> Conclusion <p>When children with KD show significant elevation of SF, hepatomegaly or no response to IVIG treatment, the possibility of concurrent MAS should be vigilant. Moreover, the simultaneous increase of AST, ALT, LDH and TG, along with the decrease of HB, PLT and FIB, has certain clinical significance in suggesting the occurrence of MAS.</p>

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Retrospective study: clinical analysis of Kawasaki disease complicated with macrophage activation syndrome

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  • 发表日期：

    2026年3月21日

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  • Wenhui Bie,
  • Yuqiang Guan,
  • Qingxia Du,
  • Lei Li,
  • Peiru Yu

摘要

Background

Kawasaki disease complicated with macrophage activation syndrome (KD-MAS) has the characteristics of low incidence, high rate of sequelae, and high mortality. We aims to explore early warning indicators that can be used for the diagnosis of KD-MAS, providing a reference basis for early clinical intervention.

Methods

The clinical data of 15 children with KD-MAS and 140 children with Kawasaki disease (KD) admitted to Jinan Children’s Hospital from January 2020 to March 2025 were retrospectively collected, and statistical analysis was conducted on the above data of the KD-MAS group and the KD group.

Results

There were no statistically significant differences between the two groups in terms of gender ratio, the proportion of incomplete Kawasaki disease, the incidence of coronary artery lesion, peripheral blood white blood cell(WBC) count, neutrophil ratio(N%), C-reactive protein(CRP), serum total bilirubin(STB) and serum potassium༈K+༉ level (P > 0.05). The age of onset in the KD-MAS group was significantly younger than that in the KD group, and the hospitalization time was longer (P < 0.05). Among the 9 KD-MAS children who completed bone marrow smear examination, 6 showed hemolysis phenomenon.The incidence of hepatosplenomegaly, organ damage, and insensitivity to intravenous immunoglobulin (IVIG) was higher in the KD-MAS group than in the KD group (P < 0.05).The levels of serum ferritin(SF), alanine aminotransferase(ALT), aspartate aminotransferase(AST), lactate dehydrogenase(LDH) and triglyceride(TG) in the KD-MAS group were higher than those in the KD group, while the levels of hemoglobin(HB), platelet(PLT) count, serum albumin(ALB), erythrocyte sedimentation rate(ESR), fibrinogen(FIB) and serum natrium(Na+) level were lower than those in the KD group (P < 0.05).The ROC curve analysis showed that the AUC values for indicators such as hepatomegaly, no response to IVIG treatment, SF, AST, FIB, PLT count, ALT, LDH, TG, and HB were all relatively high.

Conclusion

When children with KD show significant elevation of SF, hepatomegaly or no response to IVIG treatment, the possibility of concurrent MAS should be vigilant. Moreover, the simultaneous increase of AST, ALT, LDH and TG, along with the decrease of HB, PLT and FIB, has certain clinical significance in suggesting the occurrence of MAS.