title: Elevation of Serum Acid Sphingomyelinase Activity in Acute Kawasaki Disease.
abstract: Kawasaki disease (KD) is an acute systemic vasculitis that affects both small and medium-sized vessels including the coronary arteries in infants and children. Acid sphingomyelinase (ASM) is a lysosomal glycoprotein that hydrolyzes sphingomyelin to ceramide, a lipid, that functions as a second messenger in the regulation of cell functions. ASM activation has been implicated in numerous cellular stress responses and is associated with cellular ASM secretion, either through alternative trafficking of the ASM precursor protein or by means of an unidentified mechanism. Elevation of serum ASM activity has been described in several human diseases, suggesting that patients with diseases involving vascular endothelial cells may exhibit a preferential elevation of serum ASM activity. As acute KD is characterized by systemic vasculitis that could affect vascular endothelial cells, the elevation of serum ASM activity should be considered in these patients. In the present study, serum ASM activity in the sera of 15 patients with acute KD was determined both before and after treatment with infusion of high-dose intravenous immunoglobulin (IVIG), a first-line treatment for acute KD. Serum ASM activity before IVIG was significantly elevated in KD patients when compared to the control group (3.85 ± 1.46 nmol/0.1 ml/6 h vs. 1.15 ± 0.10 nmol/0.1 ml/6 h, p < 0.001), suggesting that ASM activation may be involved in the pathophysiology of this condition. Serum ASM activity before IVIG was significantly correlated with levels of C-reactive protein (p < 0.05). These results suggest the involvement of sphingolipid metabolism in the pathophysiology of KD.
authors.affiliation: Department of Pediatrics, Akita University Graduate School of Medicine.
authors.forename: Yuuki
authors.initials: Y
authors.lastname: Konno
authors.forename: Ikuko
authors.initials: I
authors.lastname: Takahashi
authors.forename: Ayuko
authors.initials: A
authors.lastname: Narita
authors.forename: Osamu
authors.initials: O
authors.lastname: Takeda
authors.forename: Hiromi
authors.initials: H
authors.lastname: Koizumi
authors.forename: Masamichi
authors.initials: M
authors.lastname: Tamura
authors.forename: Wataru
authors.initials: W
authors.lastname: Kikuchi
authors.forename: Akira
authors.initials: A
authors.lastname: Komatsu
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Tamura
authors.forename: Satoko
authors.initials: S
authors.lastname: Tsuchida
authors.forename: Atsuko
authors.initials: A
authors.lastname: Noguchi
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D000293:Adolescent; D000328:Adult; D015415:Biomarkers / Q000097:blood; D002097:C-Reactive Protein / Q000032:analysis; D002648:Child; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use; D015850:Interleukin-6 / Q000097:blood; D008297:Male; D008875:Middle Aged; D009080:Mucocutaneous Lymph Node Syndrome / Q000097:blood* / Q000628:therapy; D018570:Risk Assessment; D013107:Sphingolipids / Q000378:metabolism; D013108:Sphingomyelin Phosphodiesterase / Q000097:blood*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D015415:Biomarkers; C508600:IL6 protein, human; D016756:Immunoglobulins, Intravenous; D015850:Interleukin-6; D013107:Sphingolipids; D002097:C-Reactive Protein; D013108:Sphingomyelin Phosphodiesterase