A nomogram for predicting immunoglobulin-resistant Kawasaki disease in children.

OBJECTIVE: This case-control study focused on the establishment and internal validation of a risk nomogram for intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) using the Kawasaki Disease Database. METHODS: The Kawasaki Disease Database is the first public database for KD researchers. A prediction nomogram for IVIG-resistant KD was constructed using multivariable logistic regression. Then, the C-index was used to assess the discriminating ability of the proposed prediction model, a calibration plot was drawn to evaluate its calibration, and a decision curve analysis was adopted to assess its clinical usefulness. Bootstrapping validation was performed for interval validation. RESULTS: The median ages of IVIG-resistant and -sensitive KD groups were 3.3 and 2.9 years, respectively. Predicting factors incorporated into the nomogram were coronary artery lesions, C-reactive protein, percentage of neutrophils, platelets, aspartate aminotransferase, and alanine transaminase. Our constructed nomogram exhibited favorable discriminating ability (C-index: 0.742; 95% confidence interval: 0.673-0.812) and excellent calibration. Moreover, interval validation achieved a high C-index of 0.722. CONCLUSIONS: The as-constructed new IVIG-resistant KD nomogram that incorporated C-reactive protein, coronary artery lesions, platelets, percentage of neutrophils, alanine transaminase, and aspartate aminotransferase may be adopted for predicting the risk of IVIG-resistant KD.

A Combination Therapy for Kawasaki Disease with Severe Complications: a Case Report.

Kawasaki disease (KD) is a form of acute multisystem vasculitis that presents with various complications, including coronary artery aneurysm. Heart failure and brain damage are rare, but life-threatening complications are associated with KD. Here, we describe a 4-year-old girl who developed intravenous immunoglobulin-resistant KD with both left ventricular failure and acute encephalopathy. On day 8 of the illness, the low left ventricular ejection fraction, mitral regurgitation, and low blood pressure, which required continuous administration of dobutamine, were observed during the treatments for KD, including intravenous immunoglobulin. She also appeared unconscious, where the electroencephalogram showed slow waves of activity in all regions of the brain. The cardiac performance improved after she received plasma exchange for three days. However, her unconsciousness with slow waves of activity on electroencephalogram and fever continued after the plasma exchange. Therefore, she was treated with methylprednisolone pulse, followed by prednisolone, as well as intravenous immunoglobulin. Finally, she recovered without any cardiac or neurological sequelae not only at the time she was discharged, but also throughout the follow-up period. The combination therapy using plasma exchange and methylprednisolone pulse may be a treatment option for severe KD with left ventricular failure and acute encephalopathy complications.

Clinical Outcomes of Low-Dose Methotrexate Therapy as a Second-Line Drug for Intravenous Immunoglobulin-Resistant Kawasaki Disease.

PURPOSE: Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease (KD). However, there is still no standard treatment for IVIG-resistant KD. This study aimed to evaluate the efficacy of low-dose methotrexate (MTX) as a treatment for IVIG-resistant KD. MATERIALS AND METHODS: We retrospectively analyzed 10-year data for patients with IVIG-resistant KD who were administered MTX at Severance Children's Hospital. RESULTS: The subjects included 75 patients with KD aged 5 months to 9.2 years who had been administered MTX. Their maximum body temperatures decreased significantly within 24 h of therapy. The patients' C-reactive protein levels were significantly lower 1 week after administering the first dose of MTX than those before treatment. No adverse effect for MTX was observed. CONCLUSION: MTX treatment of IVIG-resistant KD resulted in rapid defervescence, improvement of clinical symptoms, and normalization of acute-phase reactants in all patients. Thus, MTX could be a candidate treatment for IVIG-resistant KD.

Clinical Outcomes of Low-Dose Methotrexate Therapy as a Second-Line Drug for Intravenous Immunoglobulin-Resistant Kawasaki Disease

PURPOSE: Intravenous immunoglobulin (IVIG) is the standard treatment for Kawasaki disease (KD). However, there is still no standard treatment for IVIG-resistant KD. This study aimed to evaluate the efficacy of low-dose methotrexate (MTX) as a treatment for IVIG-resistant KD. MATERIALS AND METHODS: We retrospectively analyzed 10-year data for patients with IVIG-resistant KD who were administered MTX at Severance Children's Hospital. RESULTS: The subjects included 75 patients with KD aged 5 months to 9.2 years who had been administered MTX. Their maximum body temperatures decreased significantly within 24 h of therapy. The patients' C-reactive protein levels were significantly lower 1 week after administering the first dose of MTX than those before treatment. No adverse effect for MTX was observed. CONCLUSION: MTX treatment of IVIG-resistant KD resulted in rapid defervescence, improvement of clinical symptoms, and normalization of acute-phase reactants in all patients. Thus, MTX could be a candidate treatment for IVIG-resistant KD.

Efficacy of Dexamethasone Therapy for Coronary Lesion after Immunoglobulin-retreated Kawasaki Disease

PURPOSE: To evaluate the outcome of coronary lesions for efficacy of dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD). METHODS: Retrospective studies were performed on 438 cases of KD treated with one or two episode of high-dose IVIG and 24 cases with dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD) at this hospital from June 2000 to January 2004. 2D echocardiogram was done at admission, 2 months later and every 2 or 3 months when coronary lesion had improved more than 2 months later. RESULTS: In 108(24.7%) of 438 cases with IVIG therapy only, 10(41.7%) of 24 cases with dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD), coronary abnormality had been shown by initial 2D-echocardiogram examined at acute stage. In 10(2.3%) of 438 patients with IVIG therapy only, and in 3(12.5%) of 24 cases with additional IVIG retreatment, coronary lesions were still remained at follow-up echocardiogram. Even though it had tendency to increase the coronary lesions in the group with dexamethasone therapy after additional immunoglobulin(IVIG) retreated in initial IVIG-resistant Kawasaki disease(KD), there was no significant differences about the incidence of coronary lesions between two groups. There was no significant differences in age, sex, and other clinical findings between two groups. CONCLUSION: The combination therapy with high doses of IVIG and aspirin in KD is the standard treatment but not always effective. The dexamethasone therapy may be another treatment of choice after additional immunoglobulin(IVIG) retreated in IVIG- resistant Kawasaki disease(KD).