The clinical features and outcome of Kawasaki disease combined with G6PD deficiency.

PURPOSE: The characteristics and prognosis of Kawasaki disease (KD) patients with glucose-6-phosphate dehydrogenase deficiency (G6PDd) remain unknown. This study primarily aims to investigate whether G6PDd is an independent risk factor for persistent coronary artery lesions (CAL) in KD and secondarily to evaluate the prognosis of these patients without aspirin therapy.

METHODS

We conducted a retrospective case-control study. All KD patients with G6PDd were included. The control group, consisting of KD patients with normal G6PD activity, was matched by age, sex and hospitalization period at a 1:3 ratio. The clinical features, proportion of high-risk KD, and incidence of CAL between the two groups were compared.

RESULTS

This study included 54 and 162 patients in the G6PDd and the control group, respectively. No differences were observed between the two groups in most clinical symptoms and laboratory indicators(all p > 0.05). The univariate analysis showed that G6PD deficiency was significantly associated with high-risk SANO classification (OR 3.51, 95% CI 1.60–7.72, p = 0.002). However, both groups had a similar proportion of non-SANO high-risk KD(16.67%, OR, 1.42; 95% CI, 0.60–3.35; p = 0.420). There were no significant differences in the incidence of persistent CAL beyond 8 weeks between the G6PDd group (without aspirin) and the control group (with aspirin) (12.77% vs. 6.21%; OR, 2.21; 95% CI, 0.76–6.44). Similarly, the prevalence of IVIG-resistant KD were comparable between two groups (8.51% vs. 8.70%; OR, 0.98; 95% CI, 0.31–3.12).

CONCLUSION

G6PD deficiency may not likely be a significantly risk factor for CAL in KD. SANO criteria may not be suitable for KD patients with G6PDd. Although aspirin is not routinely applied to KD patients with G6PDd, it seems unlikely have a major effect on the short-term prognosis of this subgroup.