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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 PDFThe Creeping Creatinine in a Growing Child With a Kidney Transplant: Distinguishing Progressive Graft Dysfunction From Normal Growth in Pediatric Kidney Transplant Recipients.
Pediatr Transplant
February 2025
Division of Pediatric Nephrology, Hypertension and Apheresis, Washington University School of Medicine & St. Louis Children's Hospital, St. Louis, Missouri, USA.
Background: Pediatric kidney transplant recipients experience creeping creatinine, which is a slow increase in serum creatinine over time. Distinguishing between normal growth-related changes and possible allograft dysfunction becomes challenging when interpreting the increase in serum creatinine. We hypothesized that changes in BSA-indexed measured glomerular filtration rate (mGFR) or creatinine-estimated GFR (eGFR) might not be a true reflection of the renal function post-transplant and that for longitudinal follow-up a stable absolute mGFR is better.
View Article and Find Full Text PDFPediatrics cascade screening in inherited dyslipidemias: a lipoprotein apheresis center experience.
Endocrine
December 2024
U.O. Lipoapheresis and Center for Inherited Dyslipidemias, Fondazione Toscana Gabriele Monasterio, Via Moruzzi, Pisa, Italy.
Familial hypercholesterolemia (FH) is less rare than one might think and, despite highly effective lipid-lowering therapies (LLT), more than half of the patients treated do not reach the lipid target indicated by the guidelines. In these patients, lipoprotein apheresis (LA) is the most effective tool to lowering apo-B containing atherogenic lipoproteins. In own center, since 1994, thanks to routinely cascade testing performed in patients who start LA, we have identified a pediatric population (30 subjects) that we analyzed retrospectively.
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 PDFManagement of severe pediatric Guillain-Barré syndrome in a low-income country: efficacy and safety of therapeutic plasma exchange in pediatric patients: a retrospective study.
BMC Pediatr
December 2024
Faculty of Medicine, Al-Baath University, Al-Sham Street, Homs, Syria.
Background: Guillain-Barré syndrome (GBS) is an autoimmune disease that affects the peripheral nervous system leading to motor, sensory, and sometimes autonomic manifestations. Therapeutic plasma exchange (TPE), which involves the selective removal of pathological molecules, such as auto-antibodies, from plasma, has proven to be safe and effective in adults with GBS. However, its application in pediatric patients lacks sufficient evidence.
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