Elevated Levels of Platelet Activating Factor and Its Acetylhydrolase Indicate High Risk of Kawasaki Disease

Kawasaki disease (KD) is a systemic vasculitis in children, which is related to inflammation and abnormal activation of immune system. Platelet activating factor (PAF) and its acetylhydrolase (PAF-AH) may play an important role in the pathogenesis of KD. This study aimed to investigate diagnosis and prognostic value of serum PAF and PAF-AH in KD. One hundred thirteen KD children were divided into coronary artery lesion (CAL) KD, noncoronary artery lesion (NCAL) KD, intravenous immunoglobulin (IVIG)-responsive KD, and IVIG-nonresponsive KD group. Seventy cases of fever control (F) group and 71 cases of normal control (N) group were set up. Peripheral venous blood was collected to detect serum PAF and PAF-AH levels, combined with other inflammatory mediators. Results showed that the serum levels of PAF and PAF-AH were significantly elevated in the KD group compared with F group and N group (P < 0.05). And the levels of conventional inflammatory mediators in KD group were significantly higher than those of F group (P < 0.05). In children with fever (KD group and F group), the area under the receiver operating characteristic curve (AUC) for PAF in prediction of KD was 0.804, and the estimated sensitivity and specificity were 79.6% and 74.3% with a cutoff of PAF >201.77 ng/mL, respectively; the AUC for PAF-AH in prediction of KD was 0.587, and the estimated sensitivity and specificity were 61.9% and 55.7% with a cutoff of PAF-AH >0.153 mu mol/min/mL, respectively. Compared with NCAL group, PAF and C-reactive protein were higher in CAL group (P < 0.05). The AUC for PAF in prediction of CAL KD was 0.679, and the estimated sensitivity and specificity were 96.0% and 40.9% with a cutoff of PAF >225.52 ng/mL, respectively. Thus, serum levels of PAF and PAF-AH were significantly elevated in the acute phase of KD. Serum PAF and PAF-AH contributed to the diagnosis of KD, and serum PAF has a greater diagnostic value for KD. At the same time, elevated serum PAF has a certain predictive value for the occurrence of coronary artery lesions in Kawasaki disease rather than IVIG-nonresponsive KD.