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Abstract
Kawasaki disease (KD) is a form of systemic vasculitis. Regarding its pathogenesis, HAMP gene encoding hepcidin, which is significant for iron metabolism, has a vital function. In this study, we recruited a total of 381 KD patients for genotyping. Data from 997 subjects (500 subjects from cohort 1; 497 subjects from cohort 2) were used for analysis. Using TaqMan allelic discrimination, we determined five tag SNPs (rs916145, rs10421768, rs3817623, rs7251432, and rs2293689). Treatment outcome data related to such clinical phenotypes as coronary artery lesions (CAL), coronary artery aneurysms (CAA), and intravenous immunoglobulin (IVIG) effects were also collected. Furthermore, we measured plasma hepcidin levels with an enzyme-linked immunosorbent assay. We found that HAMP gene polymorphism (rs7251432, and rs2293689) was significantly correlated with KD risk and that plasma hepcidin levels both before and after IVIG treatment had a significantly positive correlation with length of hospital stays (R = 0.217, p = 0.046 and R = 0.381, p < 0.0001, respectively). In contrast, plasma hepcidin levels has a negative correlation with KD patients’ albumin levels (R = −0.27, p < 0.001) prior to IVIG treatment. This study's findings indicate that HAMP might have a role in the disease susceptibility, as well as its expressions correlated length of hospital stays, and albumin levels in Taiwanese children with KD.
Highlights
A form of acute vasculitis, KD (Kawasaki disease) affects various systems, most often in children under the age of five years old [1]
We found that HAMP gene polymorphism was significantly correlated with KD risk and that plasma hepcidin levels both before and after intravenous immunoglobulin (IVIG) treatment had a significantly positive correlation with length of hospital stays (R = 0.217, p = 0.046 and R = 0.381, p < 0.0001, respectively)
A total of 49 (12.9%) KD patients did not respond to their initial IVIG treatment, 64 (16.8%) KD patients developed coronary artery lesions (CAL), and 16 (4.2%) patients developed coronary artery aneurysms (CAA)
Summary
A form of acute vasculitis, KD (Kawasaki disease) affects various systems, most often in children under the age of five years old [1]. Hepcidin , a protein for iron regulation, reduces iron level by inhibiting intestinal iron absorption and keeping iron storage in macrophages, leading to impaired hemoglobin synthesis [11]. It is essential for iron metabolism and for the pathogenesis of inflammation anemia [11]. We found that abnormally high levels of hepcidin could impair iron metabolism and subsequently correlate with reduced hemoglobin levels in KD patients [19, 20]. We aim to determine the role of HAMP in children’s susceptibility to KD, CAL formation, and CAA, as well as IVIG treatment responses, lengths of hospital stays, and albumin levels
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