Abstract
BackgroundAs an acute febrile and inflammatory disease, Kawasaki disease (KD) could develop Kawasaki disease shock syndrome (KDSS) sometimes. However its pathogenesis was still not well known. This study was to learn more about the clinical features and evaluate the role of cytokines in the pathogenesis of KDSS.MethodsWe collected clinical and laboratory data retrospectively for all patients with KDSS(KDSS, n = 27)who were hospitalized at our hospital from Jan 2014 to Oct 2017. For patient with KDSS, we randomly identified 43 patients with KD as control subjects (KD, n = 43). Clinical features, laboratory evaluations were collected. Cytokines IL-2, IL-4, IL-6, IL-10, TNF-α and IFN-γ in serum were assayed using flow cytometric bead array.ResultsThe patients with KDSS were older age (43.41 ± 31.42 vs 28.81 ± 21.51 months, P < 0.05), longer duration of fever (10.63 ± 5.12 vs 6.98 ± 2.45 days, P < 0.05), higher WBC count, neutrophils, CRP, ESR, PCT and D-dimer, and lower hemoglobin and albumin, more severe hyponatremia and hypokalemia, more refractory to IVIG therapy, more coronary artery abnormalities (CAAs), aseptic meningitis, and longer duration of hospitalization than patients with KD (all P < 0.05). The levels of serum IL-6 [184.1 (27.7–2577.3) vs 54.1 (4–425) pg/ml], IL-10 [42.6 (5–236.7) vs 9.4 (3–94) pg/ml], TNF-α [2.6 (1.0–23.4) vs 2.1 (1–6) pg/ml] and IFN-γ [18.3 (4.5–94.4) vs 6.7 (2–56) pg/ml] in KDSS patients were significant higher than KD patients (all P < 0.05). ROC curves showed that 66.7 pg/ml of IL-6, 20.85 pg/ml of IL-10 and 8.35 pg/ml of IFN-γ had sensitivity and specificity for identifying KDSS as 85.2 and 62.8%; 66.7 and 83.7%; 74.1 and 74.4% respectively. No fatality was recorded in this series.ConclusionsKDSS were characteristic as more cytokine production and prone to developing IVIG non-responsiveness and CAAs. KD patients with IL-6 above 66.7 pg/ml, IL-10 above 20.85 pg/ml, and IFN-γ above 8.35 pg/ml suggested higher risk for KDSS.
Highlights
As an acute febrile and inflammatory disease, Kawasaki disease (KD) could develop Kawasaki disease shock syndrome (KDSS) sometimes
Laboratory data of patients with KDSS or KD Significantly higher white blood cell count (WBC) counts (19.04 ± 8.53 vs 13.12 ± 5.22 × 109/L), Polymorphonuclear neutrophil (PMN) (79.44 ± 17.56 vs 60.76 ± 14.34%), C-reactive protein (CRP) (137.15 ± 54.80 vs 76.11 ± 50.66 mg/dl), erythrocyte sedimentation rate (ESR)[101 (24–140) vs 67 (6–135) mm/h], PCT [2.33 (0.46–19) vs 0.56 (0.001–21.88) ng/ml] and lower hemoglobin (99.37 ± 12.69 vs 110.44 ± 10.17 g/L) were observed in patients with KDSS compared to patients with KD
Receiver operating characteristic (ROC) curves showed that 66.7 pg/ml of IL-6,20.85 pg/ml of IL-10 and 8.35 pg/ml of IFN-γ had sensitivity and specificity for KDSS as 85.2 and 62.8%, 66.7 and 83.7%, 74.1 and 74.4%
Summary
As an acute febrile and inflammatory disease, Kawasaki disease (KD) could develop Kawasaki disease shock syndrome (KDSS) sometimes. Kawasaki disease (KD) is an acute, febrile vasculitis affecting children of younger than 5 years of age [1]. This disease often involved medium-sized arteries, especially coronary arteries [2]. The concept of Kawasaki Disease Shock Syndrome (KDSS) was further defined by Kanegaye in 2009 [10]. They found that this syndrome was associated with more severe laboratory markers of inflammation and greater risk of CAAs, mitral regurgitation and prolonged myocardial dysfunction [11,12,13,14,15].
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