소아 용혈성요독증후군에서 ADAMTS13 활성도의 변화
ADAMTS13 Activity in Childhood Hemolytic Uremic Syndrome (HUS)
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목 적 : HUS는 보통 shiga toxin을 생성하는 세균의 장내감염 후에 발생한다(D＋ HUS). 반면 설사 없이 발병하는 D- HUS는 어느 연령에서나 발생할 수 있으며, 재발의 경향이 높고, 예후가 불량하다. D- HUS의 임상 양상은 TTP와 유사한 경향을 보인다. 최근 ADAMTS13 활성도 감소가 TTP와 D- HUS의 병인으로 알려져 있으며, 또한 D＋ HUS에서도 급성기에 활성도의 감소가 보고되고 있다. 이에 저자들은 D＋, D- HUS 에서 ADAMTS13 활성도를 측정하였다. 방 법 : 소아 HUS 환아 19명(D＋ HUS 12명, D- HUS 7명)의 혈장을 전기영동을 이용한 다량체 분석으로 D＋ HUS의 급성기와 D- HUS의 재발기에 ADAMTS13 활성도를 측정하였다. 결 과 : D＋ HUS 환아의 ADAMTS13 활성도는 중앙값 80.9%(37.8-132.4%), D- HUS 환아는 53.9%(1.0-94.1%)로 대조군의 ADAMTS13 활성도 86.4%(34.2-1112.3%)에 비하여 유의한 차이를 보이지 않았다(P>0.05). 한 명의 D- HUS 남아에서 ADAMTS13의 심한 결핍(1.0%)을 관찰하였다. 환아는 신선냉동혈장 수혈로 일시적으로 혈소판 수치가 정상화되었다.
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.
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