PURPOSE: Clinical and laboratory findings predict a severe outcome for mucocutaneous lymph node syndrome. This study aimed to define the clinical characteristics of Kawasaki disease (KD) patients with hyponatremia and to determine the factors associated with its development. METHODS: Retrospective studies were performed on 114 KD patients who received an initial high-dose intravenous immunoglobulin (IVIG, single 2 g/kg/dose) within 10 days of fever onset from January 2006 to February 2008. These patients were divided into 2 groups. Group 1 consisted of 30 (26.3%) patients with hyponatremia, and group 2 consisted of 84 (73.6%) patients without hyponatremia. Clinical manifestations, laboratory results, and echocardiographic findings were compared between the groups. RESULTS: Group 1 patients were more likely to have a coronary artery lesion (53.3% versus 20.2%, P=0.005) and suffered from diarrhea (41.3% versus 14.1%, P=0.007). There was a higher incidence of cardiovascular involvement in group 1 patients, including coronary dilatation (46.6%), valvular regurgitation (13.3%), pericardial effusion (6.7%) and medium-sized aneurysm (6.7%). There were no coronary aneurysms in group 2 patients. Serum C-reactive protein (CRP) was significantly higher in patients with hyponatremia (12.2+/-7.79 mg/ dL versus 7.3+/-4.7 mg/dL, P=0.003) and IVIG-resistant patients were more common in group 1 (13.3% versus 3.6%). CONCLUSION: These results indicate that hyponatremia in KD occurs in patients exhibiting severe inflammation and was significantly associated with the development of coronary disease. Further studies will be necessary to confirm the pathogenic mechanisms of hyponatremia in KD patients.