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Plasma Exchange and N-Acetylcysteine Therapy in a Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting With Acute Renal Failure.
J Pediatr Hematol Oncol
January 2025
Departments of Pediatric Hematology.
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 thrombotic microangiopathy (TMA) episodes.
View Article and Find Full Text PDFPlasmapheresis for extracorporeal membrane oxygenation (ECMO)-induced hemolysis in infants.
J Extra Corpor Technol
December 2024
Division of Pediatric Nephrology, Joe DiMaggio Children's Hospital, 1131 N35th Ave, Hollywood, FL 33021, USA - Charles E. Schmidt College of Medicine at Florida Atlantic University, 777 Glades Rd BC-71, Boca Raton, FL 33431, USA.
Background: Intravascular hemolysis is a known complication of extracorporeal membrane oxygenation (ECMO). Characterized by elevated plasma-free hemoglobin (PFH), intravascular hemolysis is associated with cytotoxic effects leading to renal replacement therapy (RRT), longer ECMO runs, and mortality. Therapeutic plasma exchange (TPE) in tandem with ECMO was described as a therapy for various pathologic conditions, but there are no Extracorporeal Life Support Organization (ELSO) guidelines for the treatment of ECMO-induced hemolysis.
View Article and Find Full Text PDFAssociation of febrile neutropenia episodes and blood product transfusions in children with acute leukemia.
Transfus Apher Sci
December 2024
Department of Pediatric Hematology, Dokuz Eylül University, İzmir, Turkey. Electronic address:
Objective: Transfusion is vital supportive therapy in leukemias, but has significant potential febrile complications. In this study, we aimed to reveal the possible effects of blood product transfusions on febrile neutropenia episodes (FNEs) in children with acute leukemia.
Methods: We obtained the relevant clinical and laboratory data from the medical records.
A child with factor V deficiency with a novel F5 gene mutation misdiagnosed as a left iliac fossa abscess: A case report.
Medicine (Baltimore)
November 2024
Department of Pediatrics, West China Second Hospital, Sichuan University, Chengdu, People's Republic of China.
Rationale: Congenital factor V deficiency is classified as a rare bleeding disorder that is expressed in an autosomal recessive manner and generally occurs in 1 in a million people. This disorder is accompanied by a variety of clinical manifestations, which can easily lead to misdiagnosis. This is the first report to identify the factor V gene variant c.
View Article and Find Full Text PDFPostoperative bleeding outcome of fresh frozen plasma prime in pediatric cardiac surgery: A systematic review & meta-analysis.
Perfusion
November 2024
Department of Cardiology and Vascular Medicine, University of Gadjah Mada/Dr. Sardjito Hospital, Yogyakarta, Indonesia.
Introduction: Bleeding after cardiac surgeries holds risk of mortality and morbidity in pediatrics. This systematic review aimed to evaluate postoperative blood loss and blood transfusion requirements for pediatric patients undergoing cardiac surgery with fresh frozen plasma (FFP) priming.
Methods: In 2024, the search reviewed four databases on randomized trials (RCTs) examining the impact of FFP prime intervention on 24-h postoperative blood loss and transfusion requirements in pediatric cardiac surgeries.
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