急性熱性皮膚粘膜リンパ節症候群(MCLS)におけるプロテインCおよびプロテインSの推移について

Abstract

Plasma levels of protein C (PC) and protein S (PS) were investigated in 34 children with mucocutaneous lymphnode syndrome (MCLS). These patients were categorized into two groups :, Group A, aged from 3 months to less than 1 year, and Group B, from 1 year to 14 years. In the first week of the acute stage of the clinical course, both levels of PC activity (PC : Act) assayed by A-PTT using PC-immunodepleted plasma, and Protac®, and PC antigen (PC : Ag) assayed by ELISA, were markedly decreased ; Group A, 42.0±13.8 U/dl and 51.9±20.5 U/dl, and Group B, 51.4±19.5 U/dl and 68.2±18.6 U/dl, respectively. The values gradually returned to normal range after 3-5 weeks of the clinical course. Similarly, levels of PS activity (PS : Act) assayed by A-PTT using PS-immunodepleted plasma and Protac®, total PS antigen (T-PS : Ag) and PS antigen complexed with C4b-binding protein (C4bp-S : Ag) assayed by ELISA were significantly decreased in the first week of the clinical course ; Group A, 52.2±11.7 U/dl, 62.0±12.0 U/dl and 53.0±15.4 U/dl, and Group B, 73.6±14.7 U/dl, 82.4±16.5 U/dl and 78.8±21.3 U/dl. They were restored to normal range within the following one to 4 weeks. Free-PS antigen (F-PS : Ag) levels in both groups were almost normal or slightly decreased during the first week of the clinical course ; Group A, 71.7±15.6 U/dl and Group B, 75.8±22.4 U/dl. No precursor forms of PC : Ag and PS : Ag (PIVKAs-PC and -PS) were detected by crossed immunoelectrophoresis. Neither PC levels nor PS levels showed a significant difference between the patients with and without coronary aneurysm, whereas the former patients required 1-2 weeks longer to restore the lowered plasma levels than the latter. These results indicate that the duration of the decreased levels of plasma PC and PS is important to assess the development of anticipated thromboembolic complications in MCLS.