Prior computational and imaging studies described changes in flow patterns for patients with Marfan syndrome, but studies are lacking for related populations. This pilot study addresses this void by characterizing wall shear stress (WSS) indices for patients with Loeys-Dietz and undifferentiated connective tissue diseases. Using aortic valve-based velocity profiles from magnetic resonance imaging as input to patient-specific fluid structure interaction (FSI) models, we determined local flow patterns throughout the aorta for four patients with various connective tissue diseases (Loeys-Dietz with the native aorta, connective tissue disease of unclear etiology with native aorta in female and male patients, and an untreated patient with Marfan syndrome, as well as twin patients with Marfan syndrome who underwent valve-sparing root replacement). FSI simulations used physiological boundary conditions and material properties to replicate available measurements. Time-averaged WSS (TAWSS) and oscillatory shear index (OSI) results are presented with localized comparison to age- and gender-matched control participants. Ascending aortic dimensions were greater in almost all patients with connective tissue diseases relative to their respective control. Differences in TAWSS and OSI were driven by local morphological differences and cardiac output. For example, the model for one twin had a more pronounced proximal descending aorta in the vicinity of the ductus ligamentum that impacted WSS indices relative to the other. We are optimistic that the results of this study can serve as a foundation for larger future studies on the connective tissue disorders presented in this article.
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http://dx.doi.org/10.3389/fped.2022.772142 | DOI Listing |
Introduction: Sarcomas are rare cancers originating from mesenchymal tissues, manifesting in diverse anatomical locations, but notably in connective tissue, muscles and the skeleton. Thoracic sarcomas present a unique diagnostic and surgical challenge attributable to their rarity and pathoanatomy. Standard practice currently comprises wide surgical excision, often accompanied by adjuvant chemotherapy and/or radiotherapy.
View Article and Find Full Text PDFBone Res
January 2025
Institute of Life Course and Medical Sciences, Faculty of Health and Life Sciences, University of Liverpool, Liverpool, UK.
Low-density lipoprotein receptor-related protein 1 (LRP1) is a multifunctional endocytic receptor whose dysfunction is linked to developmental dysplasia of the hip, osteoporosis and osteoarthritis. Our work addresses the critical question of how these skeletal pathologies emerge. Here, we show the abundant expression of LRP1 in skeletal progenitor cells at mouse embryonic stage E10.
View Article and Find Full Text PDFZhonghua Yi Xue Za Zhi
February 2025
Department of Orthopedics, the First Hospital of Huaian City, Nanjing Medical University, Huaian 223300, China.
To investigate the effects of long non-coding RNA KLHL7-AS1 (LncRNA KLHL7-AS1) on the proliferation and apoptosis of nucleus pulposus cells under oxidative stress and its mechanisms. Human nucleus pulposus cells (HUM-iCell-s012) were divided into 4 groups, and unoxidized nucleus pulposus cells were transfected with an empty pcDNA vector (pcDNA-control) to serve as the blank control group. Based on previous studies on oxidative stress-induced nucleus pulposus cell senescence and preliminary experiments, oxidative stress was induced by treating nucleus pulposus cells with 400 μmol/L HO.
View Article and Find Full Text PDFEur J Vasc Endovasc Surg
January 2025
Universitat Autònoma de Barcelona (UAB), Bellaterra, Spain; Vall d'Hebron Institut de Recerca (VHIR), Barcelona, Spain; Department of Vascular, Endovascular Surgery and Angiology, Hospital Universitari Vall d'Hebron, Barcelona, Spain. Electronic address:
Objective: Abdominal aortic aneurysm (AAA) in a patient with an underlying heritable aortic disease (HAD) is rare, and evidence based recommendations for its management are lacking. This study aimed to generate a consensus from multidisciplinary specialists on the diagnosis, treatment, and surveillance of AAA associated with HAD and to define topics of interest for future research.
Methods: A Delphi consensus was designed involving European multidisciplinary specialists and reported using the ACcurate COnsensus Reporting Document (ACCORD) reporting guideline.
Eur J Oral Sci
January 2025
Department of Oral Biochemistry, Institute of Oral Bioscience, School of Dentistry, Jeonbuk National University, Jeonju-si, South Korea.
The periodontal ligament (PDL) is a connective tissue, and PDL cells have a potential to differentiate into cementoblasts, osteoblasts, and gingival fibroblasts. This study investigated whether transcription factor c-Myb could induce differentiation of PDL cells for periodontal regeneration. PDL cells were isolated from extracted teeth and cultured.
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