ABSTRACT
The aim of our study was to evaluate the efficacy of infliximab for the treatment of patients with refractory Kawasaki disease (KD) and investigate the dynamic changes of cytokines during infliximab treatment. We have performed a study of cytokine and proinflammatory molecule levels in 43 KD patients including 18 responders to IVIG, 14 nonresponders, and 11 patients treated with infliximab. We determined serum levels of soluble TNF receptor I (sTNFR I) and IL-6, as well as VEGF, damage associated molecular pattern (DAMP) molecules; myeloid-related protein (MRP)8/MRP14 and S100A12 sequentially. In eight patients, fever subsided immediately upon infliximab treatment. Four patients, who started infliximab after 12 d of illness, developed coronary artery lesions. Each of the cytokines was elevated before infliximab treatment in all patients. Although serum levels of proinflammatory cytokines decreased dramatically after infliximab treatment, DAMP molecules and VEGF and markers of local tissue damage were not suppressed. In contrast, in IVIG responders all cytokines decreased markedly after IVIG treatment. We show that infliximab is one of the adoptive therapies in refractory KD patients. Different behaviors of proinflammatory cytokines and DAMP molecules and VEGF after infliximab treatment suggest that infliximab is effective for suppression of cytokine-mediated inflammation, but could not completely block local vasculitis.
Subject(s)
Anti-Inflammatory Agents/therapeutic use , Antibodies, Monoclonal/therapeutic use , Cytokines/immunology , Endothelium, Vascular , Inflammation/drug therapy , Mucocutaneous Lymph Node Syndrome , Anti-Inflammatory Agents/immunology , Antibodies, Monoclonal/immunology , Biomarkers/metabolism , Child , Child, Preschool , Cytokines/blood , Endothelium, Vascular/cytology , Endothelium, Vascular/immunology , Endothelium, Vascular/pathology , Female , Humans , Immunoglobulins, Intravenous/immunology , Immunoglobulins, Intravenous/therapeutic use , Infant , Infliximab , Male , Mucocutaneous Lymph Node Syndrome/drug therapy , Mucocutaneous Lymph Node Syndrome/immunology , Mucocutaneous Lymph Node Syndrome/pathology , Treatment OutcomeABSTRACT
A newborn male was admitted with cyanosis and respiratory distress. Echocardiography showed a right heart isomerism associated with a single right ventricle, a double-outlet right ventricle, and pulmonary atresia. Chest X-ray demonstrated severe left upper lobe emphysema and a shift of the mediastinal structures to the right. Two-dimensional computed tomography (CT) exhibited left upper lobe emphysema and right upper lobe atelectasis. Three-dimensional (3D) spiral CT angiography showed a bilateral tracheal bronchus. The left tracheal bronchus branch was compressed between the descending aorta and the ductus arteriosus. After a right arteriopulmonary shunt operation, the patient's respiratory condition improved dramatically, with spontaneous closure of the ductus arteriosus. Subsequently, 3D-CT clearly exhibited the disappearance of tracheal compression. This combination of bilateral tracheal bronchus and congenital heart anomaly is extremely rare. The 3D-CT is a powerful noninvasive means for dynamically demonstrating the special relationships of arterial and tracheal anomalies.
