ADAMTS13 Activity in Childhood Hemolytic Uremic Syndrome(HUS).
- Author:
Cho Ae LEE
1
;
Nam Keun KIM
;
Moon Ju JANG
;
Do Yeon OH
;
Jun Ho LEE
;
Hae Il CHEONG
;
Sun Ju LEE
;
Hye Won PARK
Author Information
1. Department of Pediatrics, Pochon CHA University College of Medicine, Sungnam, Korea. parkhyewon@dreamwiz.com
- Publication Type:Original Article
- Keywords:
ADAMTS13;
HUS;
VWF;
TMA;
USS
- MeSH:
Child;
Electrophoresis;
Humans;
Male;
Plasma;
Platelet Count;
Prognosis;
Recurrence
- From:Journal of the Korean Society of Pediatric Nephrology
2006;10(2):109-118
- CountryRepublic of Korea
- Language:Korean
-
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.
