Predictive value of coagulation profiles for Kawasaki Disease Shock Syndrome: a prospective cohort study

Abstract

Abstract Background Kawasaki disease (KD) is an acute febrile inflammatory disorder that can progress to a more severe condition known as Kawasaki disease shock syndrome (KDSS). The aim of this study is to comprehend the clinical characteristics of KDSS and investigate the predictive value of the coagulation profiles in the occurrence of KDSS. Method Patients with KD were prospectively enrolled and divided into the KDSS group (n = 29) and non-KDSS group (n = 494). Multivariate logistic regression analysis was employed to ascertain the relationship between coagulation profiles and KDSS. Furthermore, ROC curve analysis was conducted to assess the predictive value of the coagulation profile in the occurrence of KDSS. Result Among the KDSS patients, the median age was higher and the proportion of cervical lymph node involvement was greater compared to the non-KDSS group. Adverse events including pericardial effusion, valve regurgitation, cardiac enlargement, coronary artery lesions (CALs), and Intravenous immunoglobulin (IVIG) resistance were also significantly higher in the KDSS group than in non-KDSS group. Notably, Prothrombin time (PT), activated partial thromboplastin time (APTT), D-dimer, and fibrin degradation products (FDP) were significantly elevated in the KDSS group compared to the non-KDSS group. Conversely, total thrombin time (TT), fibrinogen, and antithrombin III (ATIII) activity were significantly reduced. Multivariate logistic regression analysis revealed that PT, APTT, D-dimer, and ATIII were independent risk factors for predicting KDSS occurrence. ROC curve analysis established critical values for PT, D-dimer, FDP, and ATIII as 13.45 s, 2.03 mg/L, 7.45 µg/mL, and 77.5%, respectively. Sensitivity for predicting KDSS occurrence was 76%, 79%, 83%, and 76%, while specificity was 51%, 72%, 63%, and 80%, respectively. Conclusion Characteristic features of KDSS include older age, a greater likelihood of experiencing pericardial effusion, valve regurgitation, cardiac enlargement, CALs, and IVIG resistance. KD patients with a hypercoagulable state during the acute phase are at a higher risk of developing KDSS.