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Evaluation of the effect of therapeutic plasma exchange on serum cytokine levels in pediatric intensive care unit.
Ther Apher Dial
January 2025
Department of Pediatric Rheumatology, Health Science University, Umraniye Research and Training Hospital, Istanbul, Turkey.
Introduction: Therapeutic plasma exchange (TPE) is crucial for saving lives when used appropriately. This study aimed to assess TPE's impact on tumor necrosis factor-like weak inducer of apoptosis (TWEAK) protein and IL-6 levels in critically ill pediatric patients.
Methods: Conducted between May 2022 and December 2022, the study observed pediatric intensive care unit (PICU) patients undergoing TPE, recording demographics, lab results, TWEAK, and IL-6 levels pre- and post-procedure.
Clinical Complications and Healthcare Resource Utilization Associated with Conventional Management of Sickle Cell Disease with Recurrent Vaso-occlusive Crises and Transfusion-Dependent β-Thalassemia in Germany.
Pharmacoecon Open
January 2025
Division of Pediatric Stem Cell Therapy, Department of Pediatric Oncology, Hematology and Clinical Immunology, Medical Faculty, Heinrich-Heine-University, Duesseldorf, Germany.
Objective: The purpose of this study was to describe clinical complications and healthcare resource utilization (HCRU) among patients with sickle cell disease (SCD) with recurrent vaso-occlusive crises (VOCs) and patients with transfusion-dependent β-thalassemia (TDT) in Germany.
Methods: The Betriebskrankenkasse (BKKs) Database was used to identify patients with SCD or TDT. To be eligible for inclusion, patients with SCD were required to have ≥ 2 VOCs/year in any two consecutive years and ≥ 12 months of available data before and after the index date (second VOC in the second consecutive year).
EEFSEC deficiency: A selenopathy with early-onset neurodegeneration.
Am J Hum Genet
January 2025
Institute of Medical Genetics and Applied Genomics, University of Tübingen, 72076 Tübingen, Germany; Center for Rare Disease, University of Tübingen, 72076 Tübingen, Germany; Genomics for Health in Africa (GHA), Africa-Europe Cluster of Research Excellence (CoRE).
Inborn errors of selenoprotein expression arise from deleterious variants in genes encoding selenoproteins or selenoprotein biosynthetic factors, some of which are associated with neurodegenerative disorders. This study shows that bi-allelic selenocysteine tRNA-specific eukaryotic elongation factor (EEFSEC) variants cause selenoprotein deficiency, leading to progressive neurodegeneration. EEFSEC deficiency, an autosomal recessive disorder, manifests with global developmental delay, progressive spasticity, ataxia, and seizures.
View Article and Find Full Text PDFLaparoscopic Warshaw Procedure for Solid Pseudopapillary Neoplasms in Children.
Am Surg
January 2025
Department of Pediatric Surgery, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN, USA.
Background: Solid pseudopapillary neoplasms (SPNs) arising in the body or tail of the pancreas can be amenable to laparoscopic distal pancreatectomy with or without concomitant splenectomy. The purpose of this study was to evaluate laparoscopic distal pancreatectomy for SPN using the Warshaw technique as a means to preserve spleens in children.
Methods: We reviewed our database of SPN patients 19 years and younger (January 2006-December 2023).
Putative biomarkers of hepatic dysfunction in critically ill sepsis patients.
Clin Exp Med
January 2025
Pediatrics, Western University, London, ON, Canada.
Sepsis is a major cause of morbidity and mortality worldwide. Among the various types of end-organ damage associated with sepsis, hepatic injury is linked to significantly higher mortality rates compared to dysfunction in other organ systems. This study aimed to investigate potential biomarkers of hepatic injury in sepsis patients through a multi-center, case-control approach.
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