2 research outputs found

    Genetic Problems, Diagnosis, and Cardiovascular Manifestations of Loeys-Dietz Syndrome

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    Loeys-Dietz Syndrome (LDS) is an autosomal dominant connective tissue disorder with multisystem involvement of wide spectrum, found to be associated with transforming growth factor-β pathway. LDS is characterized by craniofacial, skeletal, cutaneous, vascular abnormalities along with aortic aneurysm and aortic dissection contributing to mortality and morbidity at a young age. Therefore, timely diagnosis and intervention in patients with LDS is vital. Several gene mutations have been described as contributing factors of LDS, causing widespread and aggressive vascular disease. Based on these gene mutations, 5 types of LDS have been described so far. Besides aortic aneurysm and dissection, some of the other cardiac manifestations of LDS involve cardiomyopathy, valvular abnormality, atrial fibrillation, patent ductus arteriosus, atrial septal defects, etc. Routine imaging of patients\u27 vasculatures and aggressive medical and surgical management are key factors in managing patients with LDS

    Development of Clinical Operating Guides (COGs) by the Clinical Variation Team at Jefferson Health

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    Project AIM: Create a multidisciplinary clinical variation team to develop Enterprise clinical operating guides (COGs) for high volume surgical procedures with the goal of creating three COGs by June 2022 for use by all surgical care team members
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