NDLI: Intensive plasma exchange increases a disintegrin and metalloprotease with thrombospondin motifs-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure*

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Intensive plasma exchange increases a disintegrin and metalloprotease with thrombospondin motifs-13 activity and reverses organ dysfunction in children with thrombocytopenia-associated multiple organ failure*

Content Provider	Scilit
Author	Nguyen, Trung C. Han, Yong Y. Kiss, Joseph E. Hall, Mark W. Hassett, Andrea Cortese Jaffe, Ron Orr, Richard A. Janosky, Janine Carcillo, Joseph A.
Copyright Year	2008
Description	Journal: Critical Care Medicine
Abstract	Background: Thrombocytopenia-associated multiple organ failure (TAMOF) is a poorly understood syndrome in critically ill children. A disintegrin and metalloprotease with thrombospondin motifs (ADAMTS-13), formerly known as von Willebrand factor (VWF) cleaving protease, is decreased in adults with VWF-mediated thrombotic microangiopathy, and intensive plasma exchange (PEx) both replenishes ADAMTS-13 and improves outcome in these patients. Objectives: To determine whether: 1) critically ill children with TAMOF syndrome have decreased ADAMTS-13 activity, 2) ADAMTS-13 activity correlates with platelet counts and VWF antigen, 3) the autopsies from patients who died with reduced ADAMTS-13 activity have VWF-rich microthrombi, and 4) intensive PEx will restore ADAMTS-13 activity and facilitate organ failure resolution. Design: First study: Observational. Second study: Randomized control trial. Setting: Single center university pediatric intensive care unit. Patients: First study: thirty-seven consecutive children (17 males and 20 females; ages ranging from 9 days to 23 years) identified with ≥2 organs dysfunction were enrolled. Seventy-six percent of these children had thrombocytopenia (platelet counts <100,000/mm^{3). Five additional critically ill children without MOF were also enrolled. In the second study, children with severe TAMOF (platelet counts 3 organ failure) were randomized to PEx or standard therapy. Primary physicians and parents agreed to enrollment in 10 of the 20 eligible patients with ages ranging from 1 year to 18 years. Five patients received PEx and 5 patients received standard therapy. Results: First study: children with TAMOF (n = 28) had decreased ADAMTS-13 activity, but similar plasminogen activator inhibitor-1 activity and prothrombin time compared to children with MOF without thrombocytopenia (n = 9, p < 0.05). All non-survivors (n = 7) had TAMOF, reduced ADAMTS-13 activity, and VWF-rich microvascular thromboses at autopsy. In the second study, PEx (n = 5, median 12 days, 4–28 days) restored ADAMTS-13 activity and organ function, compared to standard therapy (n = 5, p < 0.05). Conclusions: Children with TAMOF syndrome can have VWF-mediated thrombotic microangiopathy. Similar to adult experience, PEx can replenish ADAMTS-13 activity and reverse organ failure.}
Related Links	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2772176/pdf
Ending Page	2887
Page Count	10
Starting Page	2878
ISSN	00903493
e-ISSN	15300293
DOI	10.1097/ccm.0b013e318186aa49
Journal	Critical Care Medicine
Issue Number	10
Volume Number	36
Language	English
Publisher	Ovid Technologies (Wolters Kluwer Health)
Publisher Date	2008-10-01
Access Restriction	Open
Subject Keyword	Journal: Critical Care Medicine Critical Care Medicine Von Willebrand Factor Multiple Organ Failure Thrombotic Microangiopathy
Content Type	Text
Resource Type	Article
Subject	Critical Care and Intensive Care Medicine

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