From January 2004 to August 2019, 13 patients were diagnosed as a KDSS. Over the same time, 13 patients were admitted with a diagnosis of TSS and 16 patients were admitted with a diagnosis of septic shock. In addition, 93 patients were treated by a KD.
Demographics and clinical characteristics of each group are shown in Table 1. Symptoms, diagnosis, and treatments of each group are shown in Table 2. All patients in the KDSS and KD group, and 17 of 29 patients in the SS group received an echocardiographic assessment. Laboratory values and ejection fraction for KDSS group and control groups are shown in Figure 1 and supplement table 2.
KDSS vs. KD vs SS group
Patients with KDSS had a significantly older age (p = 0.00), longer fever duration (p = 0.008), longer hospital day (p = 0.00), longer intensive care unit (ICU), admission days (p = 0.00), and higher rate of using inotropic drugs (p = 0.00) than patients with KD. (Figure 1, Table 1)
KD group had significantly higher rate of initial diagnosis of KD than those in the KDSS group and KDSS group initially showed only 1-2 of typical Kawasaki symptoms. However, finally 3 or more symptoms were seen in all patients. Patients with KDSS had significantly higher rate of conjunctival injection (p = 0.00), oropharyngeal changes (p = 0.00), cervical lymphadenopathy (p = 0.00), and extremity changes (p = 0.001) than patients with SS group. (Table 2) KDSS group had significantly higher gastrointestinal symptoms (p = 0.00), respiratory symptoms (p = 0.00), neurologic symptoms (p = 0.013), pantalgia (p = 0.00), pleural effusion (p = 0.00), and organ damage (p = 0.00) than those in the KD group. In addition, patients with KDSS had a significantly higher rate of respiratory symptoms (p = 0.019) and pleural effusion (p = 0.029) than patients with SS group. (Table 2)
Compared to those in the KD group, patients in the KDSS group had lower hemoglobin level (p = 0.00), lower platelet counts (p = 0.00), higher CRP level (p = 0.00), higher total bilirubin level (p = 0.013), higher creatinine level (p = 0.002), higher BUN level (p = 0.00), lower albumin level (p = 0.00), and lower sodium level (p = 0.00). Compared with patients with KDSS, patients with septic shock group had higher levels of creatinine (p = 0.00). However, there were no significant differences in hemoglobin, platelet count, CRP level, liver function, BUN, albumin or sodium level between KDSS and SS groups. Compared to patients with KD, patients with septic shock group had lower ESR (p = 0.001). Patients in the KDSS group had significantly higher IVIG resistance rate than those in the KD group. (Figure 1, Table 2, Supplement table 3)
Compared with patients with KD, left ventricle dysfunction represented by reduced ejection fraction (< 55%) was more common in the KDSS group (p = 0.00). In addition, there was significantly (p = 0.038) more coronary artery aneurysm in the KDSS group than in the KD group. In the KDSS group, during the acute phase, five patients showed transient coronary artery aneurysm and three patients had persistent coronary artery aneurysm. In the KD group, during the acute phase of Kawasaki disease, 19 patients showed transient coronary artery aneurysm and four patients had persistent coronary artery aneurysm at the last echocardiography. There was no coronary artery aneurysm case in SS group. (Figure 1, Supplement Table 2)
KDSS vs TSS
There were no significant differences in age, sex-distribution, and laboratory findings between KDSS and TSS groups except for ESR and Creatinine. Patients with KDSS had a significantly higher ESR (p = 0.019) and significantly lower Creatinine (p=0.007) than patients with TSS. (Supplement Table 2) Receiver operation characteristic (ROC) curve revealed that the optimal ESR cut off value for determining the KDSS was 56.0 which had a sensitivity of 75.0% and a specificity of 100.0% (Figure 2, 3A, AUC, 0.894; 95% CI, 0.757-1.000, P=0.003) and the optimal Creatinine cut off value for determining the TSS was 0.695 which had as sensitivity of 76.9% and a specificity of 84.6% (Figure 2, 3B, AUC, 0.802; 95% CI, 0.620-0.983, P=0.009).