Search Results - "Niss, Omar"
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Association between diffuse myocardial fibrosis and diastolic dysfunction in sickle cell anemia
Published in Blood (13-07-2017)“…Sickle cell anemia (SCA)–related cardiomyopathy is characterized by diastolic dysfunction and hyperdynamic features. Diastolic dysfunction portends early…”
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Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease
Published in JACC. Cardiovascular imaging (01-03-2016)“…Abstract Objectives The aim of this study was to identify a unifying cardiac pathophysiology that explains the cardiac pathological features in sickle cell…”
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Left atrial dysfunction in sickle cell anemia is associated with diffuse myocardial fibrosis, increased right ventricular pressure and reduced exercise capacity
Published in Scientific reports (04-02-2020)“…Increased extracellular volume (ECV) by CMR is a marker of interstitial myocardial fibrosis and is associated with diastolic dysfunction in sickle cell anemia…”
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IL-10/Janus kinase/signal transducer and activator of transcription 3 signaling dysregulates Bim expression in autoimmune lymphoproliferative syndrome
Published in Journal of allergy and clinical immunology (01-03-2015)“…Background Autoimmune lymphoproliferative syndrome (ALPS) is a human disorder of T cell homeostasis caused by mutations that impair FAS-mediated apoptosis…”
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The Spectrum of SPTA1 -Associated Hereditary Spherocytosis
Published in Frontiers in physiology (03-07-2019)“…Hereditary spherocytosis (HS) is the most common red blood cell (RBC) membrane disorder causing hereditary hemolytic anemia. Patients with HS have defects in…”
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Author Correction: Left atrial dysfunction in sickle cell anemia is associated with diffuse myocardial fibrosis, increased right ventricular pressure and reduced exercise capacity
Published in Scientific reports (12-03-2020)“…An amendment to this paper has been published and can be accessed via a link at the top of the paper…”
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Lymphopenia in adults after the Fontan operation: prevalence and associations
Published in Cardiology in the young (01-05-2020)“…Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an…”
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Hydroxyurea Optimization through Precision Study (HOPS): study protocol for a randomized, multicenter trial in children with sickle cell anemia
Published in Current controlled trials in cardiovascular medicine (27-11-2020)“…Abstract Background Sickle cell disease (SCD) is a severe and devastating hematological disorder that affects over 100,000 persons in the USA and millions…”
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Corrigendum: The Spectrum of SPTA1-Associated Hereditary Spherocytosis
Published in Frontiers in physiology (18-10-2019)Get full text
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A reappraisal of the mechanisms underlying the cardiac complications of sickle cell anemia
Published in Pediatric blood & cancer (01-11-2017)“…Anemia, hemolysis‐driven vasculopathy, and intrinsic myocardial injury have been proposed as predisposing factors to cardiac disease in sickle cell anemia…”
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Improving Transition of Emerging Adults with Sickle Cell Disease to Adult Care through a Multidisciplinary Process: The Development of a Transition Clinic to Support Transition Success
Published in Blood (02-11-2023)“…The transition period from pediatric to adult care is challenging for people with sickle cell disease (SCD). Emerging adults with SCD who do not successfully…”
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Early initiation of disease-modifying therapy can impede or prevent diffuse myocardial fibrosis in sickle cell anemia
Published in Blood (15-09-2022)“…Cardiovascular disease is a major cause of mortality in patients with sickle cell disease (SCD). Niss et al previously reported that cardiac magnetic resonance…”
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Early hydroxyurea use is neuroprotective in children with sickle cell anemia
Published in American journal of hematology (01-10-2022)“…Children with sickle cell disease (SCD) who began hydroyxurea before age five years scored no differently on a measure of cognitive funciton than age, sex, and…”
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Reversible Myelofibrosis in Pediatric Renal Osteodystrophy
Published in The Journal of pediatrics (01-11-2020)Get full text
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Applications of cardiac magnetic resonance imaging in sickle cell disease
Published in Blood cells, molecules, & diseases (01-09-2017)“…Cardiac magnetic resonance imaging (CMR) has evolved from an effective research tool to a non-invasive clinical modality with versatile applications. The…”
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Robust clinical and laboratory response to hydroxyurea using pharmacokinetically guided dosing for young children with sickle cell anemia
Published in American journal of hematology (01-08-2019)“…Hydroxyurea is FDA‐approved and now increasingly used for children with sickle cell anemia (SCA), but dosing strategies, pharmacokinetic (PK) profiles, and…”
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Early initiation of hydroxyurea (hydroxycarbamide) using individualised, pharmacokinetics‐guided dosing can produce sustained and nearly pancellular expression of fetal haemoglobin in children with sickle cell anaemia
Published in British journal of haematology (01-08-2021)“…Summary Hydroxyurea (hydroxycarbamide) is an effective treatment for sickle cell anaemia (SCA), but clinical responses depend primarily upon the degree of…”
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Sickle cell anemia mice develop a unique cardiomyopathy with restrictive physiology
Published in Proceedings of the National Academy of Sciences - PNAS (30-08-2016)“…Cardiopulmonary complications are the leading cause of mortality in sickle cell anemia (SCA). Elevated tricuspid regurgitant jet velocity, pulmonary…”
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Pharmacokinetics-Guided Dosing of Hydroxyurea Can Achieve Near-Pancellular Fetal Hemoglobin Expression in Sickle Cell Anemia: F-Cell Analysis As a Benchmark for Disease-Modifying Therapy
Published in Blood (13-11-2019)“…▪ Background: Hydroxyurea is the standard of care for children with sickle cell anemia (SCA). We have developed and prospectively validated a population…”
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