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Journal of the Korean Society of Pediatric Nephrology 2006;10(2): 109-118.
소아 용혈성요독증후군에서 ADAMTS13 활성도의 변화
이초애, 김남근, 장문주, 이준호, 정해일, 이선주, 박혜원, 오도연
1포천중문의과대학교 분당차병원 소아과학교실
2포천중문의과대학교 분당차병원 임상의학연구소
3포천중문의과대학교 분당차병원 내과학교실
4포천중문의과대학교 분당차병원 소아과학교실
5서울대학교 의과대학 소아과학교실
6포천중문의과대학교 분당차병원 임상의학연구소
7포천중문의과대학교 분당차병원 소아과학교실
8포천중문의과대학교 분당차병원 내과학교실
ADAMTS13 Activity in Childhood Hemolytic Uremic Syndrome(HUS)
Cho-Ae Lee, Nam-Keun Kim, Moon-Ju Jang, Jun-Ho Lee, Hae-Il Cheong, Sun-Ju Lee, Hye-Won Park, Do-Yeon Oh
1Department of Pediatrics, Pochon CHA University College of Medicine
2Institute for Clinic Research, Pochon CHA University College of Medicine
3Department of Medicine, Pochon CHA University College of Medicine
4Department of Pediatrics, Pochon CHA University College of Medicine
5Department of Pediatrics4, Seoul National University Children's Hospital
6Institute for Clinic Research, Pochon CHA University College of Medicine
7Department of Pediatrics, Pochon CHA University College of Medicine
8Department of Medicine, Pochon CHA University College of Medicine
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ABSTRACT
Purpose : HUS usually occurs in children after infection with shiga toxin-producing microorganism(D+HUS). In contrast, non-postdiarrheal(D-) HUS occurs at any age and has a high rate of relapse and a poor prognosis. The clinical presentation of D-HUS is similar to that of thrombotic thrombocytopenic purpura(TTP). Recently severe deficiencies of ADAMTS13 were reported not only in TTP and D- HUS but also in D+ HUS during their acute phase. The purpose of the study is to evaluate the plasma ADAMTS13 activity in D+ and D-HUS.
Methods : Nineteen children with HUS(D+ HUS 12 and D- HUS 7) were enrolled. The assays of plasma ADAMTS13 activity were performed during the acute stage in the D+ HUS and at various stages of relapsing courses in the D- HUS patients by multimer assay, based on electrophoresis.
Results : The median plasma activity of ADAMTS13 in D+ HUS and D- HUS were 80.9%(37.8-132.4%) and 53.9%(1.0-94.1%), respectively, which were not statistically significantly different from control(86.4%, 34.2-112.3%)(P>0.05). One boy with D- HUS had severe deficiency of ADAMTS13(1.0%). His platelet count was normalized temporarily by fresh frozen plasma infusion.
Conclusion : We have demonstrated that there is no significant difference of the plasma ADAMTS13 activity between D+ HUS, D- HUS and control. We detected severe deficiency of ADAMTS13 in one boy who presented with relapsing episodes of D- HUS. ADAMTS13 deficiency should be considered in the subgroup of D- HUS especially with early onset and recurrent courses. Plasma therapy can be beneficial in this subgroup.
Key words: ADAMTS13 | HUS | VWF | TMA | USS
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