Ming-Tai Lin1, Chin-Hao Chang2, Li-Chuan Sun3, Hsin-Min Liu1, Huei-Wen Chang4, Chun-An Chen1, Shuenn-Nan Chiu1, Chun-Wei Lu1, Luan-Yin Chang1, Jou-Kou Wang1, Mei-Hwan Wu5. 1. Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan. 2. Department of Medical Research, National Taiwan University Hospital, Taipei, Taiwan. 3. Department of Pediatrics, Cardinal Tien Hospital, Taipei, Taiwan. 4. National Translational Medicine and Clinical Trial Resource Center, Taipei, Taiwan. 5. Department of Pediatrics, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan. Electronic address: wumh@ntu.edu.tw.
Abstract
BACKGROUND/ PURPOSE: Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients. METHODS: We enrolled 248 KD (development dataset: 181, validation: 67) patients who received IVIG within 10 days after fever onset. IVIG unresponsiveness was defined by persistent fever beyond 24 hours after IVIG or recrudescent fever with KD symptoms. RESULTS: From the development dataset (181 patients), IVIG unresponsiveness was noted in 22 patients (12.1%). The preIVIG levels of albumin, percentage of neutrophils, and positive lymphadenopathy were identified with highest risk for IVIG unresponsiveness. These three variables were used to construct a three-variable logistic regression model, which yielded an area under the receiver-operating-characteristics curve of 0.87. These three variables were further used to generate a composite scoring model (Formosa score) which yielded a sensitivity of 90.9% and specificity of 81.3% for a cut-off point of three or more. Validation in an independent cohort (67 KD patients) yielded sensitivity and specificity of 71.4% and 81.0%, respectively. CONCLUSION: We have established a simple three-variable Formosa score for KD patients to identify early those at risk of IVIG unresponsiveness for timely aggressive immunomodulation initially.
BACKGROUND/ PURPOSE:Kawasaki disease (KD) is the most common pediatric vasculitis. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KDpatients. METHODS: We enrolled 248 KD (development dataset: 181, validation: 67) patients who received IVIG within 10 days after fever onset. IVIG unresponsiveness was defined by persistent fever beyond 24 hours after IVIG or recrudescent fever with KD symptoms. RESULTS: From the development dataset (181 patients), IVIG unresponsiveness was noted in 22 patients (12.1%). The preIVIG levels of albumin, percentage of neutrophils, and positive lymphadenopathy were identified with highest risk for IVIG unresponsiveness. These three variables were used to construct a three-variable logistic regression model, which yielded an area under the receiver-operating-characteristics curve of 0.87. These three variables were further used to generate a composite scoring model (Formosa score) which yielded a sensitivity of 90.9% and specificity of 81.3% for a cut-off point of three or more. Validation in an independent cohort (67 KDpatients) yielded sensitivity and specificity of 71.4% and 81.0%, respectively. CONCLUSION: We have established a simple three-variable Formosa score for KDpatients to identify early those at risk of IVIG unresponsiveness for timely aggressive immunomodulation initially.
Authors: Yu Rang Park; Jae-Jung Kim; Young Jo Yoon; Young-Kwang Yoon; Ha Yeong Koo; Young Mi Hong; Gi Young Jang; Soo-Yong Shin; Jong-Keuk Lee Journal: Database (Oxford) Date: 2016-07 Impact factor: 3.451