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Article

Plasma Exchange and N-Acetylcysteine Therapy in a Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting With Acute Renal Failure.

Journal of pediatric hematology/oncology - 1 Jan 2025

Coşkun Çağri, Aksu Tekin, Gülhan Bora, Düzova Ali, Ünal Şule

Abstract excerpt

Congenital thrombotic thrombocytopenic purpura (cTTP), which is associated with mutations in the gene for a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), is a chronic and lifelong disease. The clinical course is variable. Regularly using ADAMTS13-containing products such as fresh frozen plasma (FFP) for long-term prophylaxis is the most important treatment to prevent...

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Identifiers

PMID: 39737637
DOI: 10.1097/MPH.0000000000002963

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