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Large Variations in Phenylalanine Concentrations Associate Adverse Cardiac Remodelling in Adult Patients With Phenylketonuria-A Long-Term CMR Study.
J Cachexia Sarcopenia Muscle
February 2025
Department of Cardiology, Angiology and Intensive Care Medicine, Deutsches Herzzentrum der Charité, Berlin, Germany.
Background: Despite a phenylalanine (Phe) restrictive diet, most adult patients with 'classical' phenylketonuria (PKU) maintain life-long Phe concentrations above the normal range and receive tyrosine (Tyr) and protein-enriched diets to maintain acceptable concentrations and ensure normal development. While these interventions are highly successful in preventing adverse neuropsychiatric complications, their long- term consequences are incompletely explored. We observed early cardiomyopathic characteristics and associated hemodynamic changes in adult PKU patients and present here the results of a longitudinal evaluation of cardiac phenotype.
View Article and Find Full Text PDFPrecise diagnosis and effective management of highly vascular anterior mediastinal paragangliomas: A case report.
Int J Surg Case Rep
January 2025
Department of Thoracic surgery, National University Hospital, Damascus University, Damascus, Syria.
Introduction: Mediastinal paragangliomas are rare neoplasms arising from extra-adrenal neural crest cells, presenting as either functional or nonfunctional tumors. Clinical manifestations range from catecholamine-related symptoms to physical compression effects. Accurate recognition of these tumors is crucial for diagnosis and management due to their rarity and association with vital mediastinal structures.
View Article and Find Full Text PDFHigher Aircraft Noise Exposure Is Linked to Worse Heart Structure and Function by Cardiovascular MRI.
J Am Coll Cardiol
December 2024
UCL MRC Unit for Lifelong Health and Ageing, University College London, London, United Kingdom; UCL Institute of Cardiovascular Science, University College London, London, United Kingdom; Centre for Inherited Heart Muscle Conditions, Cardiology Department, Royal Free Hospital, London, United Kingdom. Electronic address:
Background: Aircraft noise is a growing concern for communities living near airports.
Objectives: This study aimed to explore the impact of aircraft noise on heart structure and function.
Methods: Nighttime aircraft noise levels (L) and weighted 24-hour day-evening-night aircraft noise levels (L) were provided by the UK Civil Aviation Authority for 2011.
Successful thoracoscopic-assisted resection of a functional paraganglioma in the mediastinum with extracorporeal circulation: a case report.
J Cardiothorac Surg
January 2025
Department of Thoracic Surgery, The Second Xiangya Hospital of Central South University, Changsha, Hunan, P. R. China.
Background: Paragangliomas are rare neoplasms arising from extra-adrenal chromaffin cells, with mediastinal paragangliomas representing an exceptionally rare subset. This report details the surgical management of a complex mediastinal paraganglioma case, presenting with refractory hypertension and invasion of critical surrounding structures. A comprehensive review of the current literature is included to underscore existing cases, enhance clinical awareness, and share our insights and experience in the diagnosis and treatment of this challenging condition.
View Article and Find Full Text PDFCommentary: Review of Mandatory Maintenance of Certification in the USA.
Eval Health Prof
January 2025
Jewish General Hospital, Canada.
The goal of maintenance of certification (MOC) activities is to ensure physicians are up to date on current practices and demonstrate the knowledge and skills required to provide patients with optimal care. The program's aim is to promote professional development, lifelong learning and quality assurance for the public and medical community. However, physicians are not happy with the current structure of the program, claiming it to be time-consuming, expensive and ineffective for their practice.
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