160 research outputs found

    The Agony of Influence

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    Two Unusual Mind Diagrams in a Late Fifteenth-Century Manuscript (UPenn Schoenberg Collection, LJS 429)

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    University of Pennsylvania Libraries, Lawrence J. Schoenberg Collection, MS LJS 429, is a small booklet containing materials of natural philosophy, chiefly related to the effects of cosmic forces on human biology. Two of its diagrams illustrate the mentalizing process of the Aristotelian-Thomist psychology anima sensitiva, or the process through which sensory experience is formed as a mental perception. This essay points out the ways in which these diagrams differ from a standard (Thomist) medieval model of Mind. During the very late Middle Ages, the analysis of Mind as anima sensitiva and mens appears to shift from being action-based (analysed in terms of abilities and powers) to being substantive-based (analysed in terms of substantial agents using material tools). I will suggest that these two diagrams unusually model “faculty psychology” in a way that seems to foreshadow one we associate more with the time of Descartes, and even of Locke and Hume

    Dendara métropole

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    Le chantier « Dendara métropole » vise à étudier les divers aspects du temple d’Hathor dans son environnement, en portant les investigations sur l’étude architecturale des monuments ainsi que sur l’exploration archéologique des quartiers d’habitations et des cimetières. Outre la poursuite des travaux sur l’architecture monumentale, sur les secteurs associés aux fondations de Montouhotep II et sur la nécropole de l’Ancien Empire, la campagne 2019 a ouvert de nouvelles perspectives de recherche..

    Case Reports1. A Late Presentation of Loeys-Dietz Syndrome: Beware of TGFβ Receptor Mutations in Benign Joint Hypermobility

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    Background: Thoracic aortic aneurysms (TAA) and dissections are not uncommon causes of sudden death in young adults. Loeys-Dietz syndrome (LDS) is a rare, recently described, autosomal dominant, connective tissue disease characterized by aggressive arterial aneurysms, resulting from mutations in the transforming growth factor beta (TGFβ) receptor genes TGFBR1 and TGFBR2. Mean age at death is 26.1 years, most often due to aortic dissection. We report an unusually late presentation of LDS, diagnosed following elective surgery in a female with a long history of joint hypermobility. Methods: A 51-year-old Caucasian lady complained of chest pain and headache following a dural leak from spinal anaesthesia for an elective ankle arthroscopy. CT scan and echocardiography demonstrated a dilated aortic root and significant aortic regurgitation. MRA demonstrated aortic tortuosity, an infrarenal aortic aneurysm and aneurysms in the left renal and right internal mammary arteries. She underwent aortic root repair and aortic valve replacement. She had a background of long-standing joint pains secondary to hypermobility, easy bruising, unusual fracture susceptibility and mild bronchiectasis. She had one healthy child age 32, after which she suffered a uterine prolapse. Examination revealed mild Marfanoid features. Uvula, skin and ophthalmological examination was normal. Results: Fibrillin-1 testing for Marfan syndrome (MFS) was negative. Detection of a c.1270G > C (p.Gly424Arg) TGFBR2 mutation confirmed the diagnosis of LDS. Losartan was started for vascular protection. Conclusions: LDS is a severe inherited vasculopathy that usually presents in childhood. It is characterized by aortic root dilatation and ascending aneurysms. There is a higher risk of aortic dissection compared with MFS. Clinical features overlap with MFS and Ehlers Danlos syndrome Type IV, but differentiating dysmorphogenic features include ocular hypertelorism, bifid uvula and cleft palate. Echocardiography and MRA or CT scanning from head to pelvis is recommended to establish the extent of vascular involvement. Management involves early surgical intervention, including early valve-sparing aortic root replacement, genetic counselling and close monitoring in pregnancy. Despite being caused by loss of function mutations in either TGFβ receptor, paradoxical activation of TGFβ signalling is seen, suggesting that TGFβ antagonism may confer disease modifying effects similar to those observed in MFS. TGFβ antagonism can be achieved with angiotensin antagonists, such as Losartan, which is able to delay aortic aneurysm development in preclinical models and in patients with MFS. Our case emphasizes the importance of timely recognition of vasculopathy syndromes in patients with hypermobility and the need for early surgical intervention. It also highlights their heterogeneity and the potential for late presentation. Disclosures: The authors have declared no conflicts of interes
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