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

Open Access
12.12.2025
Research

Erschienen in:

Verfasst von: Peisi Cheng

Peisi Cheng

Shantou University Medical College, 515041, Shantou, China; Tingyan He

Korrespondierender Autor Tingyan He

Department of Rheumatology and Immunology, Shenzhen Children’s Hospital affiliated Shantou University Medical College, Shantou University, 518038, Shenzhen, China

hetingyan2017@outlook.com; Jun Yang

Jun Yang

Department of Rheumatology and Immunology, Shenzhen Children’s Hospital affiliated Shantou University Medical College, Shantou University, 518038, Shenzhen, China
Erschienen in: Pediatric Rheumatology | Ausgabe 1/2026

Abstract

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.

Hinweise

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Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Background

Kawasaki disease (KD) is an acute systemic vasculitis predominantly affecting children under the age of five, and can potentially lead to severe complications, including coronary artery dilation, aneurysms, shock, and macrophage activation syndrome. The etiology of KD remains unclear, although both genetic and environmental factors are believed to contribute to its pathogenesis [1‐5].

Glucose-6-phosphate dehydrogenase deficiency (G6PDd) is an X-linked genetic disorder caused by defects in the erythrocyte enzyme G6PD, which plays a critical role in protecting erythrocyte from oxidative stress, through the generation of nicotinamide adenine dinucleotide phosphate (NADPH) [6, 7]. Emerging evidence suggests that G6PD deficiency is associated with elevated oxidative stress and dysregulated cytokine production, both of which can exacerbate inflammation in various pathological conditions [7‐9]. New clinical observations, including our preliminary data, suggest that a small proportion of children with Kawasaki disease also have G6PD deficiency. While the direct involvement of G6PD deficiency in the development of coronary artery lesions (CAL) in KD has not been established, these inflammatory pathways overlap with those implicated in CAL formation [2‐4], raising the possibility that G6PD deficiency may contribute to CAL progression in KD, warranting further investigation into this potential connection.

Futhermore, G6PD deficiency has been reported to impair myocardial function, influence angiogenesis, and promote atherosclerosis [10‐13], processes that are also relevant to KD-related cardiovascular complications [2‐4]. These shared mechanisms suggest that G6PD deficiency could potentially exacerbate CAL in KD patients, a hypothesis that remains largely unexplored.

Aspirin is a standard treatment for KD, aimed at controlling inflammation and reducing CAL progression [5]. However, its use is contraindicated in G6PD-deficient patients due to the risk of hemolysis [14], raising an important clinical question: does withholding aspirin therapy in this population affect CAL outcomes?

The primary objective of this study is to investigate whether G6PD deficiency constitutes an independent risk factor for persistent coronary artery lesions in children with KD. Given the withholding aspirin therapy in G6PD-deficient individuals, a secondary objective is to evaluate whether the absence of aspirin therapy influences CAL outcomes in this specific subgroup.

Patients and methods

We conducted a retrospective case-control study. KD patients with G6PDd were consecutively enrolled from January 2016 to September 2021 at Shenzhen Children’s Hospital. All eligible KD patients with G6PD deficiency were included. The control group was matched to cases at a 1:3 ratio by age, sex and hospitalization peroid, comprising KD patients with normal G6PD activity. The primary endpoint was the persistence of CAL at 8 weeks. Analyses included comparisons of clinical features, the proportion of high-risk KD patients, and the incidence of IVIG-resistant KD in two groups.

Study population and design

The study was approved by the hospital’s ethics committee, and written informed consent was obtained from the legal guardians of all patients. G6PD deficiency was confirmed by laboratory testing demonstrating extremely low glucose-6-phosphate dehydrogenase activity. KD, incomplete KD, and IVIG resistant KD were diagnosed based on the American Heart Association criteria [5]. High-risk KD was defined by the presence of one or more of the following: scoring according to the KOBAYASHI [15], SANO [16], or EGAMI [17] criteria, or the occurrence of complications such as Macrophage Activation Syndrome(MAS) or Kawasaki disease shock syndrome(KDSS). MAS was diagnosed according to the 2016 EULAR/ACR/PRINTO classification criteria in KD patients [18], whereas KDSS was defined as sustained systolic hypotension or clinical signs of poor perfusion [5]. Exclusion criteria included patients with underlying cardiac diseases (e.g., congenital or acquired heart disease, arrhythmias and pericardial disease), metabolic diseases (other than G6PDd), confirmed inborn immune errors (confirmed by relevant genetic abnormalities), or cases missing G6PD activity data. Also patients with hemolytic anemia unrelated to G6PD deficiency were also excluded.

Data collection

Clinical data were collected from inpatient and outpatient medical records. The data included clinical manifestations, laboratory and imaging findings, treatment, and clinical outcomes. A list of clinical manifestations is provided in Table 1. The laboratory indicators included peripheral leukocyte counts, hemoglobin levels, platelet counts, urinary white blood cells, serum levels of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), aminotransferases, albumin, ferritin, fibrinogen, triglycerides, total bilirubin, serum sodium, and serum potassium. Management included intravenous immunoglobulin (IVIG), glucocorticoids, aspirin, dipyridamole, and antibiotics.

Table 1

Clinical characteristics and of G6PDda with KD patients

	With G6PDd (n = 54)	Without G6PDd (n = 162)	Z/χ2	p-value
Fever days^a	7(6,9)	6(5,7)	-2.682	0.007
Fever spike	40.0(39.5,40.2)	40.0(39.6,40.2)	-0.468	0.640
Polymorphous rash	45(83.33)	139(85.80)	0.196	0.658
Bilateral nonexudative conjunctivitis	50(92.59)	156(96.30)	0.559	0.455
Oral mucous membrane changes^b	47(87.04)	148(91.36)	0.862	0.353
Peripheral extremity changes^c	42(77.78)	126(77.78)	0.000	1.000
Cervical lymphadenopathy^d	44(81.48)	136(83.95)	0.018	0.673
Redness at the BCG inoculation site	8(14.81)	22(13.58)	0.052	0.820
Respiratory symptoms	32(59.26)	107(66.05)	0.814	0.367
Gastrointestinal symptoms	23(42.59)	68(41.98)	0.006	0.937
Limitation of neck movement	5(9.26)	16(9.88)	0.018	0.895
Central nervous symptoms	11(20.37)	17(10.49)	3.502	0.061

Data expressed as median (interquartile range) or n (%). (a) Variables such as fever days and spike were accessed by Wilcoxon Rank-Sum test. Others were analyzed by Chi-square test. (b) Oral mucous membrane changes: injected or fissured lips, injected pharynx, or strawberry tongue. (c) Peripheral extremity changes: erythema of palms or soles, edema of hands and periungual desquamation in convalescent phase. (d) Cervical lymphadenopathy: at least 1 lymph node > 1.5 cm in diameter. G6PDd Glucose-6-phosphate dehydrogenase deficiency, BCG Bacillus Calmette-Guerin

Statistical analysis

Normally distributed continuous variables are presented as mean ± standard deviation (SD) and compared using independent samples t-test. Non-normally distributed data are expressed as median (interquartile range, IQR) and compared using the Mann-Whitney U test. Categorical variables were analyzed using the Chi-square or Fisher’s exact test, with odds ratios and 95% confidence intervals (CIs) reported. Given the relatively small sample size, effect sizes with CIs were emphasized to provide more informative interpretation of the results. All statistical analyses were performed using SPSS version 26.0, and a a two-sided p-value < 0.05 was considered statistically significant.

Result

The clinical characteristics of G6PDd with KD patients

We compared 54 KD patients with G6PDd to 162 KD patients with normal G6PD activity. The clinical characteristics of both groups are summarized and in Table 1. Except for the duration of fever, which was significantly longer in the G6PDd group (Z=-2.682, p = 0.007), most clinical features did not differ significantly between the two groups. These included classic KD manifestations such as fever spikes, polymorphous rash, bilateral nonexudative conjunctivitis, and oral mucous membrane changes (e.g. cracked, red lips and “strawberry tongue”) (all p > 0.05). Others features, including peripheral extremity changes (indurated edema on the dorsum of the hands and feet, diffuse erythema on the palms and soles, and periungual desquamation), cervical lymphadenopathy, redness at the BCG inoculation site, respiratory and gastrointestinal symptoms, limitation of neck movement, and central nervous system symptoms, were similarly distributed across both groups (all p > 0.05) (Table 1).

The laboratory parameters between KD patients with G6PDd and the control KD patients

Hemoglobin levels(t=-5.343, p < 0.001) were significantly lower in KD patients with G6PDd, whereas ESR (Z=-1.982, p = 0.048), ferritin (Z=-5.280, p < 0.001) and total bilirubin levels(Z=-3.577, p < 0.001) were significantly elevated. However, no significant differences were observed between the two groups regarding other laboratory parameters, including peripheral white blood cell count, neutrophil percentage, platelet count, urinary white blood cells, serum CRP, procalcitonin (PCT), alanine aminotransferase (ALT), aspartate transaminase (AST), albumin, and serum sodium (all p > 0.05) (Table 2).

Table 2

Laboratory findings

	With G6PDd (n = 54)	Without G6PDd (n = 162)	Z/t	p-value
WBC count (×10¹²/µL)	15.6(12.4,19.6)	15.4(12.6,19.3)	−0.175	0.861
Hemoglobin (g/dL)^a	96.1 ± 15.32	107.8 ± 8.58	−5.343	0.000
Neutrophil percentile (%)^a	67.9 ± 15.73	67.06 ± 14.87	0.391	0.696
Platelet (×10⁶ /µL)	431.0(308.5,504.5)	371.5(315.0,464.5)	−1.057	0.290
CRP (mg/L)	59.6(29.6,114.8)	63.0(37.4,101.9)	−0.646	0.518
ESR (mm/h)	78.0(55.5,89.0)	66.0(47.0,83.0)	−1.982	0.048
PCT (ng/ml)	0.4(0.2,2.0)	0.5(0.3,1.7)	−0.219	0.827
ALT (IU/L)	39.5(20.0,77.5)	42.0(16.0,96.3)	−0.298	0.766
AST (IU/L)	30.0(22.8,41.3)	32.0(23.0,49.0)	−0.541	0.589
Ferritin (ng/mL)	254.0(162.4,393.9)	136.1(100.0,203.1)	−5.280	0.000
Triglyceride (mg/dL)	1.4(1.1,1.8)	1.3(1.1,1.7)	−1.062	0.288
Fibrinogen (mg/dL)^a	5.23 ± 1.47	5.50 ± 1.51	−1.124	0.262
Total bilirubin (umol/L)	10.8(6.2,21.4)	6.6(4.7,9.9)	−3.577	0.000
Unconjugated bilirubin (umol/L)	6.1(3.4,12.2)	4(2.7,6.5)	−3.280	0.001
Albumin (g/L)	34.4(30.2,37.3)	34.95(32.4,38.0)	−1.862	0.063
Sodium (mmol/L)	135.8(134.2,137.2)	135.6(133.9,137.1)	−0.111	0.912
Potassium (mmol/L)^a	4.14 ± 0.63	4.24 ± 0.60	−1.042	0.299

Data expressed as mean, median (interquartile range) or n (%). a. Variables such as neutrophil percentile, hemoglobin, fibrinogen, and potassium were acessed by T-test. Others were analyzed by Wilcoxon rank sum test. WBC white blood cell, CRP C-reactive protein, ESR erythrocyte sedimentation rate, PCT procalcitonin, ALT alanine aminotransferase, AST aspartate aminotransferase

Proportion of high-risk KD

The proportion of high-risk KD, as defined by the SANO criteria, was significantly higher in the G6PD deficiency group (27.78%, χ²=10.55, p = 0.001; OR, 3.52, 95% CI, 1.62–7.66). In contrast, no significant differences were observed between groups for high-risk KD based on the KOBAYASHI criteria(14.81%, OR 1.31, 95% CI 0.54–3.18, p = 0.553), Non-SANO high-risk KD (16.67%, OR 1.42, 95% CI 0.60–3.35, p = 0.420), incomplete KD (11.11%, OR 1.23, 95% CI 0.45–3.35, p = 0.691), MAS(1.85%, OR 1.00, 95% CI 0.10–9.95, p = 1.000), and KDSS (5.56%, OR 3.11, 95% CI 0.61–15.85, p = 0.167) (Table 3).

Table 3

High-Risk Kawasaki disease

	With G6PDd(n = 54)	Without G6PDd(n = 162)	χ²	p-value	OR(95% CI)
Criteria variables
KOBAYASHI criteria^[12]	8(14.81)	9(11.73)	0.353	0.553	1.31(0.54–3.18)
SANO criteria^[13]	15(27.78)	16(9.88)	10.558	0.001	3.52(1.62–7.66)
EGAMI criteria^[11]	0(0)	4(2.47)	-	0.574	-
Criteria positive^a	15(27.78)	25(15.43)	4.091	0.043	2.11(1.04–4.28)
Incomplete KD^[5]	6(11.11)	15(9.26)	0.158	0.691	1.23(0.45–3.35)
MAS^[14]	1(1.85)	3(1.85)	-	1.000	1.00(0.10–9.95)
KDSS^[5]	3(5.56)	3(1.85)	-	0.167	3.12(0.61–15.85)
Non-SANO high-risk KD^c	9(16.67)	290(12.35)	0.651	0.420	1.42(0.60–3.35)
High-risk KD^[5]d	16(29.63)	26(16.05)	4.768	0.029	2.21(1.09–4.50)

Data was presented with n(%). Variables were analyzed by Chi-square test. (a) Criteria positive including KOBAYASHI, SANO and EGAMI criteria. (b) Non-SANO criteria including KOBAYASHI criteria and EGAMI criteria. (c) Non-SANO high-risk KD including KOBAYASHI criteria, EGAMI criteria, MAS and KDSS. (d) High-risk KD including all criteria, IVIG-resistant KD, MAS and KDSS. KD Kawasaki disease, MAS macrophage activation syndrome, KDSS Kawasaki disease shock syndrome

Multivariable analysis of SANO risk classification

We conducted sequential logistic regression to identify predictive factors for high-risk classification according to the SANO criteria (Table 4). G6PD deficiency was significantly associated with SANO high-risk in univariate analysis (OR 3.51, 95% CI 1.60–7.72, p = 0.002), while this association was fully attenuated and lost statistical significance after adjusting for total bilirubin (OR 2.47, 95% CI 0.68–9.03, p = 0.172). In contrast, bilirubin remains a strong independent predictor (OR 1.089, 95% CI 1.055–1.125, p < 0.001).

Table 4

Multivariable logistic regression analysis of SANO High-Risk classification

	Model 1 OR(95% CI), p	Model 2 OR(95% CI), p	Model 3 OR(95% CI), p
G6PD deficiency	3.51(1.60–7.72), p = 0.002	2.47(0.68–9.03), p = 0.172	3.01 (0.68–13.30), p = 0.147
Total bilirubin(µmol/L)	-	1.089 (1.055–1.125), p < 0.001	1.084(1.049–1.121), p < 0.001
Hemoglobin (g/dL)	-	0.993(0.946–1.043), p = 0.772	1.005 (0.949–1.063), p = 0.874
CRP(mg/L)	-	-	1.017 (1.005–1.029), p = 0.007
Platelet count(×10⁹/L)	-	-	1.002 (0.998–1.006), p = 0.327
Age (months)	-	-	1.015 (0.985–1.047), p = 0.332
Sex (male)	-	-	1.922 (0.526–7.027), p = 0.323

Model fit statistics

Model 1: Hosmer-Lemeshow test p = NA; Overall accuracy = 85.6%; Sensitivity = 0%

Model 2: Hosmer-Lemeshow test p = 0.010; Overall accuracy = 92.6%; Sensitivity = 58.1%

Model 3: Hosmer-Lemeshow test p = 0.658; Overall accuracy = 94.0%; Sensitivity = 64.5%

Abbreviations: OR, odds ratio; CI, confidence interval; CRP, C-reactive protein

Note: Model 1: Unadjusted; Model 2: Adjusted for bilirubin and hemoglobin; Model 3: Fully adjusted model

This pattern persisted in the fully adjusted model, which included hemoglobin, CRP, platelet count, age, and sex. In this model, G6PD deficiency remained non-significant (OR 3.01, 95% CI 0.68–13.30, p = 0.147), while both bilirubin (OR 1.084, 95% CI 1.049–1.121, p < 0.001) and CRP (OR 1.017, 95% CI 1.005–1.029, p = 0.007) retained independent predictive value. The final model demonstrated good calibration (Hosmer-Lemeshow p = 0.658) and high predictive accuracy (94%).

Management and prognosis

A total of 7 KD patients with G6PDd received low-dose aspirin treatment, whereas the remaining 47 patients in the G6PDd group did not receive aspirin (Table 5). Except for aspirin, dipyridamole and glucocorticoids, no other antiplatelet or anti-inflammatory agents were administered. In the control group, 161 patients were treated with aspirin and one was not. The length of hospital stays was comparable between the G6PDd group without aspirin and the control group with aspirin (p = 0.142) (Table 6). There were no significant differences in the proportion of CAL that persisted beyond 8 weeks between the G6PDd group without aspirin and the control group with aspirin (161 patients) (12.77% vs. 6.21%; OR 2.2, 95% CI 0.758–6.438, p = 0.241) or in the prevalence of IVIG resistant KD (8.51% vs. 8.70%; OR 0.98, 95% CI 0.306–3.122, p = 1.000) (Table 6).

Table 5

Management

	With G6PDd (n = 54)	Without G6PDd (n = 162)	Z/χ²	p-value
IVIG (day)^a	6(5,8)	6(5,7)	−1.052	0.293
Aspirin	7	161	-	-
Glucocorticoids	16(29.63)	27(21.60)	1.446	0.229
Dipyridamole	51(94.44)	151(93.21)	0	1.000
Antibiotics	50(92.59)	154(95.06)	0.118	0.732
Vasoactive drugs	0(0)	2(1.23)	-	1.000

Data expressed as median (interquartile range) or n (%). a. IVIG means the day of administration of intravenous immunoglobulin(day). And IVIG was accessed by Wilcoxon rank sum test. Other variables were analyzed by Chi-square test

Table 6

Prognosis between G6PDd group and normal G6PD group

	G6PDd group (n = 47)	Normal G6PD group (n = 161)	Z/χ²	P value	OR (95% CI)
Hospital stays (ds)	6(5,10)	6(5,8)	-1.467	0.142
CAL > 8wks	6(12.77)	10(6.21)	1.375	0.241	2.2(0.758–6.438)
IVIG-R KD^[5]	4(8.51)	14(8.70)	0.000	1.000	0.98(0.306–3.122)

Data expressed as median (interquartile range) or n (%). (a) Hospital days was accessed by Wilcoxon Rank-Sum test. Others were analyzed by Chi-square test. (b) CAL > 8wks coronary artery lesions that persisted beyond 8 weeks. (c) IVIG-R KD intravenous immunoglobulin resistant Kawasaki disease. CAL coronary artery lesions

Discussion

This study provides a preliminary exploration of the potential interactions between G6PD deficiency and KD, two conditions with overlapping pathophysiological features such as oxidative stress and inflammatory dysregulation. Previous research has suggested that G6PD deficiency may enhance inflammatory responses through cytokine modulation [10, 11, 19] and endothelial injury mechanism [12, 13], theoretically increasing the risk of CAL in KD. However, in our study, the incidence of CAL persistent beyond 8 weeks show no significant differences (OR 2.21, 95% CI 0.758–6.438) between KD patients with low or normal G6PD activity. These findings suggest that G6PD deficiency may not substantially increase CAL risk in this population. Similarly, no significant differences were observed between the two groups in clinical manifestations, laboratory parameters, the proportion of high-risk KD (excluding SANO criteria), or the incidence of IVIG-resistant KD. Although, the study was sufficiently powered to detect large effects (e.g., risk ratio >2.0), the wide confidence intervals for these results limit the precision of these estimates, reflecting the challenges inherent to studying this rare patient population.

The neutral CAL outcome observe in G6PD-deficient patients may reflect a hypothesis of complex immunometabolic balance. Although G6PD deficiency increases oxidative stress, it is also associated with anti-inflammatory effects, including impaired T cell proliferation, reduced cytokine production (such as IFN-γ, IL-17), and weakened cytotoxic function, thereby suppressing adaptive immunity. This T-cell suppression inhibits M1 polarization and IFN-γ-dependent macrophage activation, thereby attenuating a key inflammatory pathway. Meanwhile, NADPH deficiency may inhibit NLRP3 inflammasome activation and promote the differentiation of anti-inflammatory M2 macrophages, supported by the activation of the Nrf2 pathway [20, 21]. In KD, vascular injury is mainly driven by innate immunity. Although G6PD deficiency may suppress adaptive immunity, this effect appears insufficient to alleviate the overall inflammatory response. Conversely, G6PD deficiency may also induce pro-inflammatory response under certain conditions. These opposing effects likely counterbalance one another, resulting in no net increase in CAL risk [11, 20, 21]. Further mechanistic studies are warranted to elucidate these complex interactions.

Interestingly, KD patients with G6PDd presented with lower hemoglobin levels, higher ESR, elevated ferritin, and increased total bilirubin, consistent with stress-induced hemolysis. Furthermore, a higher proportion of these patients were classified as high-risk KD according to the SANO criteria, whereas the proportion of high-risk KD defined by non-SANO criteria was similar between groups. We hypothesize that this difference arises from the the inclusion of bilirubin (a marker that typically increases in hemolytic state [6]) as a key component of the SANO criteria, potentially leading to risk overestimation in G6PD-deficient patients. Multivariate logistic regression analysis supported this hypothesis. After adjusting for bilirubin, the statistical significance of G6PD status disappeared, while bilirubin remained strongly and was independently associated with SANO criteria. These findings suggest that the apparent increased risk in G6PD-deficient patients is primarily attributed to hemolysis-related bilirubin elevation, rather than the severity of KD, highlighting potential limitations of applying the SANO criteria in this population. This hypothesis warrants further validation in future studies.

Regarding aspirin therapy is controversial in G6PDd patients, our exploratory and observation analysis found no significant difference in CAL progression between patients who received aspirin and those who did not. This aligns with documented evidence from two cases [22, 23], who reported successful management and complete coronary recovery in G6PDd KD patients treated with IVIG alone. This observation, though limited by the sample size, this finding challenges concerns about withholding aspirin in this subgroup and suggests that the absence of aspirin therapy may not have a major impact on short-term outcomes. This align with prior studies demonstrating that high-dose aspirin does not improve the KD prognosis compared to median- or low-dose aspirin [24‐28], or even to withholding aspirin, which was supported by a randomized clinical trial [29]. We emphasize that confounding by indication remains a potential limitation, and well-designed randomized controlled trials are needed to more definitively assess the role of aspirin in this patient population.

Several limitations should be acknowledged. The small sample size, due to the rare coexistence of G6PD deficiency and KD, limits detection of smaller associations. This single-center cohort lacked longitudinal follow-up, restricting generalizability and evaluation of long-term cardiovascular outcomes.

G6PD deficiency is genetically heterogeneous, and varying detection methods precluded enzymatic subgrouping, highlighting the need for future studies integrating molecular genotyping with WHO-based activity classification [6]. Aspirin was largely withheld in G6PD-deficient patients, with a few receiving low-dose aspirin without safety concerns, reflecting real-world clinical practice.

Finally, while Ravelli’s criteria [30] for MAS perform reasonably in KD, overlapping features may cause diagnostic uncertainty, emphasizing the need to refine MAS criteria for KD. Multi-center prospective studies with larger cohorts and longer follow-up are warranted to validate these findings.

Conclusion

Despite these limitations, this study provides valuable preliminary evidence on the interaction between G6PD deficiency and KD. Our findings suggest that G6PD deficiency is unlikely to substantially worsen coronary outcomes in KD patients receiving standard treatment protocols. Moreover, the SANO criteria may not be appropriate for accurately assessing the risk in this specific subgroup. Although aspirin is not routinely administered to KD patients with G6PDd, our results suggest that its omission may be unlikely have a major effect on their short-term prognosis. However, these conclusions should be interpreted with caution due to the limited sample size and study design, they offer an important foundation for future research and warrant validation in larger, well-characterized cohorts with extended follow-up.

Acknowledgements

We thank all the patients and their families for their participation.

Declarations

All participating members were enrolled with the approval of the ethics committee of Shenzhen Children’s Hospital and provided written consent from legal guardians.

Written consent for publication of this anonymous information was obtained from patients’ legal guardians.

Competing interests

The authors declare no competing interests.

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Metadaten
Literatur

Titel: The clinical features and outcome of Kawasaki disease combined with G6PD deficiency
Verfasst von: Peisi Cheng
Tingyan He
Jun Yang
Publikationsdatum: 12.12.2025
Verlag: BioMed Central
Erschienen in: Pediatric Rheumatology / Ausgabe 1/2026
Elektronische ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-025-01179-z

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Springer Medizin

Abstract

Purpose

Methods

Results

Conclusion

Publisher’s note

Background

Patients and methods

Study population and design

Data collection

Statistical analysis

Result

The clinical characteristics of G6PDd with KD patients

The laboratory parameters between KD patients with G6PDd and the control KD patients

Proportion of high-risk KD

Multivariable analysis of SANO risk classification

Management and prognosis

Discussion

Conclusion

Acknowledgements

Declarations

Ethics approval and consent to participate

Consent for publication

Competing interests

Publisher’s note