Kawasaki Disease: Laboratory Findings and an Immunopathogenesis on the Premise of a ""Protein Homeostasis System

Lee, Kyung-Yil; Rhim, Jung-Woo; Kang, Jin-Han

echanism that underlies the predilection for coronary artery involvement in KD are largely unknown. 2, [11] [12] [13] [14] [15] [16] Laboratory parameters are used for the diagnosis and evaluation of conditions of patients for any inflammatory disease. As for laboratory findings in KD, many inflammatory indices change throughout the disease process; elevated levels of C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), leukocyte count with neutrophilia (lymphopenia), platelet count, alanine aminotransferase (ALT), aspartate aminotransferase (AST) and other inflammation associated enzymes, as well as decreased levels of lymphocytes, albumin, hemoglobin, sodium, potassium and total cholesterol including high density lipoprotein cholesterol (HDL-cholesterol) have been detected. 2 The severity of inflammation in KD is reflected by inflammatory param-have been implicated. 15, 36 Recently as a more precise genetic tool, a genome-wide linkage analysis and follow-up association study (genome-wide associated study) for the potential loci and genes of susceptibility to KD have also been performed in several countries. [37] [38] [39] [40] The results of the genetic studies may have some limitations resulting from small sample size, failure to replicate results in subsequent studies, and lack of precise phenotype of KD patients based on CAL outcomes and response to IVIG therapy. 36, 41 In Japan, Onouchi, et al. 37, 42 reported that the ITPKC gene which is a negative regulator of T-cell activation was associated with KD susceptibility and an increased risk of CALs. Although the association of the ITPKC gene in replication studies of other populations is controversial, 39, 40, 43 this finding suggests a relevant clue for the genetic study of KD in which immune reaction of T cells may have a crucial role in the immunopathogenesis of the disease. Recently, the International Kawasaki Disease Genetics Consortium has been organized and has identified many candidate genes potentially related to inflammation, apoptosis and cardiovascular pathology. 38, 41 Although susceptibility to KD is polygenic, further studies are necessary to determine relationships between the candidate genes and functional consequences that lead to KD or CALs.