Plasma therapy in von!Willebrand factor protease deficiency

被引：13

作者：

Deschênes, G

Veyradier, A

Cloarec, S

Benoit, S

Desbois, I

Gruel, Y

Nivet, H

机构：

[1] Hop Gatien Clocheville, Unite Nephrol Pediat, F-37044 Tours, France

[2] Hop Antoine Beclere, Serv Hematol Biol, F-92141 Clamart, France

[3] Hop Bretonneau, Ctr Transfus Sanguine, F-37044 Tours, France

[4] Hop Trousseau, Hematol Lab, F-37044 Tours, France

[5] Hop Trousseau, Serv Nephrol Pediat, F-75571 Paris 12, France

来源：

PEDIATRIC NEPHROLOGY | 2002年 / 17卷 / 10期

关键词：

hemolytic uremic syndrome; thrombotic thrombocytopathic purpura; fresh-frozen plasma; Von Willebrand factor cleaving protease; recurrence of hemolytic uremic syndrome; renal transplant;

D O I：

10.1007/s00467-002-0944-9

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

We report a patient with relapsing hereditary hemolytic uremic syndrome (HUS) that began in the neonatal period with life-threatening jaundice and hemolytic anemia. He progressed to end-stage renal failure at 14 years of age and had a cerebrovascular accident while on dialysis. The cause of HUS was a constitutional deficiency in the von Willebrand factor cleaving protease. Hematological features of HUS significantly improved following bilateral nephrectomy. After renal transplantation, he had an early recurrence of HUS associated with two episodes of retinal and cerebral ischemia. Long-term treatment with fresh-frozen plasma exchanges prevented recurrence of HUS, cerebrovascular attacks, and early loss of the graft.

引用

页码：867 / 870

页数：4