Abstract
An epidemiological study showed that a positive family history of Kawasaki disease (KD) was a risk factor for intravenous immunoglobulin (IVIG) therapy resistance, coronary artery lesions (CALs), and KD recurrence. However, real-world outcomes of KD patients with a family history remain unclear. The objective of this study was to elucidate the outcomes of KD patients with a family history in the era of 2 g/kg IVIG therapy. This retrospective study included data from 201 KD patients who underwent acute-phase treatment from January 2009 to June 2020, with 184 (91.5%) receiving 2 g/kg IVIG therapy. The patients were divided into 13 (family group) with and 188 (nonfamily group) without a family history of KD. The rates of IVIG resistance (8.3% vs. 22.1%, P = 0.315), rescue therapy (8.3% vs. 12.8%, P = 1.000), CALs (0.0% vs. 2.7%, P = 1.000), and KD recurrence (0.0% vs. 3.2%, P = 1.000) were similar between the family and nonfamily groups.
Highlights
Kawasaki disease (KD) is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected [1]
The main findings of this study were that the rates of intravenous immunoglobulin (IVIG) resistance, rescue therapy, coronary artery lesions (CALs), and KD recurrence were similar between the family and nonfamily groups (Table IV)
These findings suggested that a positive family history of KD is not a risk factor for IVIG therapy resistance, CAL development, and KD recurrence in the era of 2 g/kg IVIG therapy
Summary
Kawasaki disease (KD) is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected [1]. One study showed that the inositol 1, 4, 5triphosphate 3-kinase C gene is associated significantly with KD susceptibility and an increased risk of coronary artery lesions (CALs) [1]. Several evidences suggest the importance of genetic factors in disease susceptibility and outcome [1]. The incidence of KD is 10 to 20 times higher in Japan than in Western countries [2]. Compared with the general populations, the risk of KD in siblings of affected children is 10 times higher and the incidence in children born to parents with a history of KD is twice as high [3], [4]. Familial aggregation of the disease has been observed [5]
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