10 results match your criteria: "Amsterdam University Medical Centers Amsterdam The Netherlands.[Affiliation]"

Introduction: High-density (HD) substrate mapping may increase success of catheter ablation targeting ventricular tachycardia (VT). However, despite its use, recurrent VT is not uncommon. We aim to investigate factors that are associated with outcomes after HD mapping-guided substrate ablation procedures for VT in patients with ischemic cardiomyopathy.

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Background: Cardiac surgery triggers sterile innate immune responses leading to postoperative complications. Clonal hematopoiesis (CH) is associated with short-term inflammation-mediated outcomes after cardiac surgery. The impact of CH on long-term postoperative outcomes remains unknown.

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Riboflavin transporter deficiency in young adults unmasked by dietary changes.

JIMD Rep

July 2024

Department of Pediatrics, Division of Metabolic Disorders Emma Children's Hospital, Gastroenterology, Endocrinology & Metabolism, Amsterdam University Medical Centers Amsterdam The Netherlands.

Riboflavin transporter deficiency (RTD) is a genetic disorder of reduced riboflavin (vitamin B2) uptake that causes progressive, multifocal neurological dysfunction. Most patients present in early childhood; if patients present later in life, symptoms usually develop more gradually. We report three previously healthy young adults, who developed rapidly progressive neurological symptoms after decreasing dietary intake of meat and dairy.

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Background: There is inconsistency in outcomes collected in renal cell cancer (RCC) intervention effectiveness studies and variability in their definitions. This makes critical summaries of the evidence base difficult and sub-optimally informative for clinical practice guidelines and decision-making by patients and healthcare professionals. A solution is to develop a core outcome set (COS), an agreed minimum set of outcomes to be reported in all trials in a clinical area.

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We report a unique case of an infant with a severe dilated cardiomyopathy as the clinical presentation of sialidosis type II (OMIM 256550), a rare autosomal recessive inherited lysosomal storage disease that is characterized by partial or complete deficiency of α-neuraminidase, following mutations in the gene neuraminidase 1 (), located on the short arm of chromosome 6 (6p21.3). Accumulation of metabolic intermediates leads to severe morbidity, especially myoclonus, gait disturbances, cherry-red macules with secondary loss of visual acuity, impaired color vision and night blindness, and sometimes additional neurological findings such as seizures.

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Article Synopsis
  • Desmopressin is a helpful treatment for people with nonsevere hemophilia A because it costs less and doesn't have the same risks as other treatments.
  • The study looked at how well desmopressin worked on bleeding compared to other treatments from 2009 to 2020.
  • It found that many patients can respond well to desmopressin, but most still use factor concentrates, which means it might not be used as well as it could be.
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We reviewed the literature (January 2010-June 2021) on the effectiveness of debulking strategies before venetoclax initiation in patients with chronic lymphocytic leukemia to reduce tumor burden, downgrade tumor lysis syndrome (TLS) risk, and avoid hospitalization. Low TLS incidence and reduced TLS risk based on tumor burden were reported following debulking in clinical trials. Real-world observational studies reporting debulking regimens recorded no TLS events, and those without debulking strategies had greater TLS incidence.

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Background: Impulsivity is a core feature of attention-deficit/hyperactivity disorder (ADHD). Previous work using the delay discounting task to assess impulsivity reveals that adolescents with ADHD tend to prefer a smaller-immediate reward over a larger-delayed reward, and this relates to problematic choices in daily life. To gain a better understanding of daily decision-making in adolescence, it is important to examine the social context, as peers have a major influence on decisions.

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Objective: Insulin resistance develops prior to the onset of overt type 2 diabetes, making its early detection vital. Direct accurate evaluation is currently only possible with complex examinations like the stable isotope-based hyperinsulinemic euglycemic clamp (HIEC). Metabolomic profiling enables the detection of thousands of plasma metabolites, providing a tool to identify novel biomarkers in human obesity.

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