Childhood Kidney Diseases
- Volume 10 Issue 2
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- Pages.109-118
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- 2006
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- 2384-0242(pISSN)
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- 2384-0250(eISSN)
ADAMTS13 Activity in Childhood Hemolytic Uremic Syndrome(HUS)
소아 용혈성요독증후군에서 ADAMTS13 활성도의 변화
- Lee, Cho-Ae (Department of Pediatrics, Pochon CHA University College of Medicine) ;
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Kim, Nam-Keun
(Institute for Clinic Research, Pochon CHA University College of Medicine) ;
- Jang, Moon-Ju (Department of Medicine, Pochon CHA University College of Medicine) ;
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Lee, Jun-Ho
(Department of Pediatrics, Pochon CHA University College of Medicine) ;
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Cheong, Hae-Il
(Department of Pediatrics4, Seoul National University Children's Hospital) ;
- Lee, Sun-Ju (Institute for Clinic Research, Pochon CHA University College of Medicine) ;
-
Park, Hye-Won
(Department of Pediatrics, Pochon CHA University College of Medicine) ;
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Oh, Do-Yeon
(Department of Medicine, Pochon CHA University College of Medicine)
- 이초애 (포천중문의과대학교 분당차병원 소아과학교실) ;
-
김남근
(포천중문의과대학교 분당차병원 임상의학연구소) ;
- 장문주 (포천중문의과대학교 분당차병원 내과학교실) ;
-
이준호
(포천중문의과대학교 분당차병원 소아과학교실) ;
-
정해일
(서울대학교 의과대학 소아과학교실) ;
- 이선주 (포천중문의과대학교 분당차병원 임상의학연구소) ;
-
박혜원
(포천중문의과대학교 분당차병원 소아과학교실) ;
-
오도연
(포천중문의과대학교 분당차병원 내과학교실)
- Published : 2006.10.31
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.