Abstract
Kawasaki disease (KD) is the most common cause of heart disease acquired in childhood. Even if treated with high-dose intravenous immunoglobulin G (IVIG) at the early stage; children are still at risk of developing coronary artery lesions. Accumulating evidence suggests that autophagy is enhanced in various heart diseases. Evaluating the pathogenic role of autophagy in KD and coronary artery lesions (CAL) may aid in identifying a potential therapeutic target for the treatment or prevention of the disease. Blood samples were obtained from 20 children with KD at the onset of disease and 21 days after IVIG therapy. Twenty children with other causes of febrile disease and 20 healthy children were included as controls. Total RNA was extracted from white blood cells; and autophagy-related gene mRNA expression levels were measured using real-time polymerase chain reaction. The patients with KD had downregulated levels of LC3B mRNA (0.50 ± 0.06 vs. 1.67 ± 0.15; p < 0.001), BECN 1 mRNA (0.70 ± 0.08 vs. 1.43 ± 0.23; p < 0.05), and ATG16L1 mRNA (0.28 ± 0.04 vs. 0.96 ± 0.16; p < 0.01) compared to the febrile control group. The values of these parameters all increased significantly 21 days after the IVIG therapy as follows: LC3B mRNA (1.77 ± 0.29 vs. 0.50 ± 0.06; p < 0.001), BECN 1 mRNA (1.67 ± 0.36 vs. 0.70 ± 0.08; p < 0.05), and ATG16L1 mRNA (2.96 ± 0.43 vs. 0.28 ± 0.04; p < 0.001), while the level of ATG16L1 mRNA persists low in KD patients with CAL. Our results showed the autophagy-related genes expressions in KD and their change after IVIG administration. This suggests that autophagy may have a protective effect on KD.
Highlights
Kawasaki disease (KD) is the most common cause of heart disease in childhood in the developed nations [1]
There was no significant difference in age and sex between the children with KD and those in both the control groups (Tables 1–5)
We observed that the downregulated mRNA levels of autophagy markers in children with
Summary
Kawasaki disease (KD) is the most common cause of heart disease in childhood in the developed nations [1] It is known as mucocutaneous lymph node syndrome [2] and typically presents with fever for more than 5 days in children. Administration of high-dose intravenous immunoglobulin (IVIG) plus aspirin early in the course of KD reduces the prevalence of coronary artery abnormalities from 18 to 4% [6]. Despite this success, children with persistent or recurrent fever are at an increased risk of developing CAL [7]. Additional therapies administered to these patients include retreatment with IVIG, use of immunomodulatory
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