A Polymorphism in Plasma Platelet-activating Factor Acetylhydrolase is Involved in Resistance to Immunoglobulin Treatment in Kawasaki Disease - 21/08/11
, Hiroyuki Suzuki, MD, PhD, Takashi Takeuchi, MD, Shigeru Uemura, MD, PhD, Junko Sugatani, PhD, Norishige Yoshikawa, MD, PhD
Reprint requests: Dr Takaomi Minami, Department of Pediatrics, Wakayama Medical University, 811-1 Kimiidera, Wakayama 641-0012, Japan.Abstract |
Objective |
To investigate whether reduced levels of plasma platelet-activating factor acetylhydrolase (PAF-AH) as a result of a genetic polymorphism are involved in the pathogenesis of Kawasaki disease (KD).
Study design |
The frequency of a V279F polymorphism (G/T transversion) in the PAF-AH gene was quantified in 76 Japanese children with KD and 112 healthy Japanese adults using the allele-specific polymerase chain reaction (PCR). Associations between genotype, clinical features, and resistance to intravenous immunoglobulin (IVIG) were investigated in the patients with KD. Plasma PAF-AH activity was measured by using [3H]-acetyl-PAF.
Results |
There were no significant differences in genotype frequency between patients and controls (P=.51). Compared with the GG (normal genotype) group, significantly more patients in the GT (heterozygous) +TT (homozygous deficient) group required additional IVIG (52% vs 14%, P=.001). The duration of fever and maximum serum C-reactive protein (CRP) levels also were significantly increased in the GT+TT group (P=.012 and .036, respectively), whereas plasma PAF-AH activity was significantly lower (P <.0001).
Conclusion |
We conclude that the V279F polymorphism in the plasma PAF-AH gene and consequent enzymatic deficiency is one of the factors for IVIG nonresponse in Japanese patients with acute KD.
Le texte complet de cet article est disponible en PDF.Mots-clés : CAL, CRP, IL, IVIG, KD, PAF, PAF-AH, PCR, TNF, WBC
Plan
| This work was supported by a Grant-in-Aid from the Ministry of Education, Culture, Sports, Science and Technology, Japan (2002-2004). |
Vol 147 - N° 1
P. 78-83 - juillet 2005 Retour au numéro
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