1,408 results match your criteria: "Our Lady-s Children's Hospital[Affiliation]"

Background And Aim: There is emerging evidence of cardiovascular remodeling and functional impairment in individuals conceived via Assisted Reproductive Technologies (ART). The aim of this study was to serially assess myocardial function and pulmonary hemodynamic measurements in infants conceived via ART over the first year of age and to compare them to a cohort of spontaneously conceived controls.

Methods: This was a prospective, observational study.

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Von Willebrand factor-inflammation crosstalk in deep vein thrombosis.

J Thromb Haemost

March 2023

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland; National Children's Research Centre, Our Lady's Children's Hospital, Dublin, Ireland.

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Y-27632 inhibits Rho-associated coiled-coil-containing protein kinase (ROCK) signaling, which is involved in various embryonic developmental processes, including angiogenesis, by controlling actin cytoskeleton assembly and cell contractility. Administration of Y-27632 impairs cytoskeletal arrangements in post-gastrulation chick embryos, leading to ventral body wall defects (VBWDs). Impaired angiogenesis has been hypothesized to contribute to VBWDs.

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Background: Early detection of cognitive disability is challenging. We assessed the domain-specific, concurrent validity of the ages and stages questionnaire (ASQ-3) and the Bayley Scales of Infant and Toddler Development (BSID-III), and their ability to predict cognitive delay at school age.

Methods: Within a longitudinal birth cohort study, a nested cohort of children was assessed using ASQ-3 and BSID-III at 24 months, and at 5 years using the Kaufmann brief IQ test (KBIT).

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Second allogeneic stem cell transplantation can rescue a significant proportion of patients with JMML relapsing after first allograft.

Bone Marrow Transplant

May 2023

Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

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Endothelial cell activation, Weibel-Palade body secretion, and enhanced angiogenesis in severe COVID-19.

Res Pract Thromb Haemost

February 2023

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland.

Background: Severe COVID-19 is associated with marked endothelial cell (EC) activation that plays a key role in immunothrombosis and pulmonary microvascular occlusion. However, the biological mechanisms through which SARS-CoV-2 causes EC activation and damage remain poorly defined.

Objectives: We investigated EC activation in patients with acute COVID-19, and specifically focused on how proteins stored within Weibel-Palade bodies may impact key aspects of disease pathogenesis.

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Macrophage Galactose Lectin Contributes to the Regulation of FVIII (Factor VIII) Clearance in Mice-Brief Report.

Arterioscler Thromb Vasc Biol

April 2023

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons, Dublin, Ireland (S.E.W., T.G., C.B., P.L., J.M.O., D.D., A.C., J.F., R.J.S.P., J.S.O.).

Background: Although most plasma FVIII (Factor VIII) circulates in complex with VWF (von Willebrand factor), a minority (3%-5%) circulates as free-FVIII, which is rapidly cleared. Consequently, 20% of total FVIII may be cleared as free-FVIII. Critically, the mechanisms of free-FVIII clearance remain poorly understood.

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Purpose: The aims of our study were to investigate with cardiovascular magnetic resonance (CMR) the role of Epicardial Fat Volume (EFV) and distribution in patients with Takotsubo cardiomyopathy (TTC). Moreover, we explored EFV in patients with TTC and related this to comorbidities, cardiac biomarkers, and cardiac function.

Methods: This retrospective study performed CMR scans in 30 consecutive TTC patients and 20 healthy controls.

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Background: Bleeding assessment tools are key screening tests used in the evaluation of patients with suspected inherited bleeding disorders. The International Society on Thrombosis and Haemostasis-Scientific and Standardization Committee endorsed Bleeding Assessment Tool (ISTH-BAT) has differing reference ranges for adult males (0-3), adult females (0-5), and children (0-2), reflecting differing bleeding symptoms and exposure to hemostatic challenges in these healthy population subgroups. Age is known to markedly impact bleeding score in individuals with von Willebrand disease.

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Diploid triploid mosaicism (DTM) is a rare genetic condition where there is an extra haploid set of chromosomes in mosaic form. We describe an infant for whom DTM was detected antenatally through amniocentesis. Prenatal counselling suggested a guarded prognosis.

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Dysregulated haemostasis in thrombo-inflammatory disease.

Clin Sci (Lond)

December 2022

Irish Centre of Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland.

Inflammatory disease is often associated with an increased incidence of venous thromboembolism in affected patients, although in most instances, the mechanistic basis for this increased thrombogenicity remains poorly understood. Acute infection, as exemplified by sepsis, malaria and most recently, COVID-19, drives 'immunothrombosis', where the immune defence response to capture and neutralise invading pathogens causes concurrent activation of deleterious prothrombotic cellular and biological responses. Moreover, dysregulated innate and adaptive immune responses in patients with chronic inflammatory conditions, such as inflammatory bowel disease, allergies, and neurodegenerative disorders, are now recognised to occur in parallel with activation of coagulation.

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Background: Internationally, a single standard chemotherapy treatment for Ewing sarcoma is not defined. Because different chemotherapy regimens were standard in Europe and the USA for newly diagnosed Ewing sarcoma, and in the absence of novel agents to investigate, we aimed to compare these two strategies.

Methods: EURO EWING 2012 was a European investigator-initiated, open-label, randomised, controlled phase 3 trial done in 10 countries.

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Evaluation of miRNA Expression in 3D In Vitro Scaffold-Based Cancer Models.

Methods Mol Biol

November 2022

Department of Anatomy and Regenerative Medicine, Cancer Bioengineering Group, RCSI University of Medicine and Health Sciences, Dublin, Ireland.

Accumulating experimental evidence suggests that 3D in vitro cancer models strengthen our understanding of vital processes in the tumor microenvironment (TME) and accelerate the drug discovery pipeline. Previous studies examining the effects of specific miRNAs on cancer cells in vitro have involved ectopic expression of miRNA mimics in 2D in vitro culture. Assessment of cell viability and gene expression ensures that upregulation of the chosen miRNA and repression of its target genes have been achieved.

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Assessment of Basic Biological Functions Exerted by miRNAs.

Methods Mol Biol

November 2022

Department of Anatomy and Regenerative Medicine, Cancer Bio-Engineering Group, Royal College of Surgeons in Ireland, Dublin, Ireland.

To evaluate basic miRNA function, it is vital to assess various cellular processes, such as cell viability and proliferation, under different miRNA levels. Here we describe the process of overexpression of miRNA in vitro via transfection and subsequent downstream evaluation using the acid phosphatase assay.

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Background: Cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE) is a key diagnostic tool in the differential diagnosis between non-ischemic cause of cardiac chest pain. Some patients are not eligible for a gadolinium contrast-enhanced CMR; in this scenario, the diagnosis remains challenging without invasive examination. Our purpose was to derive a machine learning model integrating some non-contrast CMR parameters and demographic factors to identify Takotsubo cardiomyopathy (TTC) in subjects with cardiac chest pain.

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Objective: To assess the impact of milrinone administration on time spent on nitric oxide (iNO) in infants with acute pulmonary hypertension (aPH). We hypothesized that intravenous milrinone used in conjunction with iNO would reduce the time on iNO therapy and the time spent on invasive ventilation in infants ≥34 weeks gestation with a diagnosis of aPH. We aimed to assess the practicality of instituting the protocol and contributing to a sample size calculation for a definitive multicentre study.

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von Willebrand factor links primary hemostasis to innate immunity.

Nat Commun

November 2022

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Science (PBS), Royal College of Surgeons in Ireland, Dublin, Ireland.

The plasma multimeric glycoprotein von Willebrand factor (VWF) plays a critical role in primary hemostasis by tethering platelets to exposed collagen at sites of vascular injury. Recent studies have identified additional biological roles for VWF, and in particular suggest that VWF may play an important role in regulating inflammatory responses. However, the molecular mechanisms through which VWF exerts its immuno-modulatory effects remain poorly understood.

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Article Synopsis
  • Interleukin (IL)25 plays a key role in causing inflammation and maintaining tissue health at barrier surfaces in various organs, prompting the need to develop a neutralizing antibody targeting it.
  • Researchers created a novel antibody called 22C7, which effectively inhibits IL25's inflammatory effects in mouse models, including significant reductions in immune cell recruitment and skin inflammation.
  • The findings support 22C7 as a promising therapeutic option for treating conditions related to IL25, with further engineering aimed at making it a fully human antibody while preserving its effectiveness.
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Background: Prolonged recovery is common after acute SARS-CoV-2 infection; however, the pathophysiological mechanisms underpinning Long COVID syndrome remain unknown. VWF/ADAMTS-13 imbalance, dysregulated angiogenesis, and immunothrombosis are hallmarks of acute COVID-19. We hypothesized that VWF/ADAMTS-13 imbalance persists in convalescence together with endothelial cell (EC) activation and angiogenic disturbance.

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The protein C pathways.

Curr Opin Hematol

September 2022

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland.

Purpose Of Review: To provide an overview of the state-of-the-art in protein C (PC) pathway research.

Recent Findings: The PC pathway is crucial for maintaining hemostasis to prevent venous thromboembolism. This is evident from genetic mutations that result in impaired PC pathway activity and contribute to increased venous thromboembolism risk in affected individuals.

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The surgical burden of sebaceous naevus excision in childhood.

J Plast Reconstr Aesthet Surg

August 2022

Department of Plastic & Reconstructive Surgery, Our Lady's Children's Hospital and St. James's Hospital, Dublin, Ireland.

Unlabelled: Prophylactic excision of sebaceous naevi during childhood has been common practice due to the risk of malignant transformation into basal cell carcinoma (BCC). With incidence of BCC now recognised as 0.8%, a more conservative approach to management is being advocated.

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The human skin can be affected by a multitude of diseases including inflammatory conditions such as atopic dermatitis and psoriasis. Here, we describe how skin barrier integrity and immunity become dysregulated during these two most common inflammatory skin conditions. We summarise recent advances made in the field of the skin innate immune system and its interaction with adaptive immunity.

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Clinical Features and Natural History of Preadolescent Nonsyndromic Hypertrophic Cardiomyopathy.

J Am Coll Cardiol

May 2022

Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London, United Kingdom; Institute of Cardiovascular Sciences, University College London, London, United Kingdom. Electronic address:

Article Synopsis
  • Up to 50% of childhood cases of hypertrophic cardiomyopathy (HCM) occur before age 12, but this demographic hasn't been thoroughly studied.
  • The research aimed to analyze the clinical characteristics and progression of children diagnosed with nonsyndromic HCM under 12 years old, utilizing data from the International Paediatric Hypertrophic Cardiomyopathy Consortium involving 639 children.
  • Results showed that younger patients had similar symptoms and cardiac issues compared to older children; however, they experienced serious events like mortality at younger ages, with a notable difference in surgical and device interventions between the two age groups.
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