Browsing by Author "Male, Christoph (7003604062)"

Jean St-Louis MD, FRCPC 1,2| Audrey Abad BSc 3| Sharon Funk DPT 4|Merlyn Tilak MPT 5| Stephen Classey PT 6| Nichan Zourikian BSc, PT 7|Paul McLaughlin PT, MSc, MCSP 8| Sébastien Lobet PT, PhD 9|Grace Hernandez PT10| Stacie Akins PT11| Anna J. Wells PT, MSc, MCSP, SRP12|Marilyn Manco-Johnson MD4| Judy John MD5| Steve Austin MBBS BMedSci FRACP FRCPA6| Pratima Chowdary MD8| Cedric Hermans MD9| Diane Nugent MD10|Nihal Bakeer MD11| Sarah Mangles MD, MBBS, MD (Res), FRCP, FRCPath12|Pamela Hilliard BSc, PT3| Victor S. Blanchette MB, BChir (Cantab), FRCPC13,14|Brian M. Feldman MD, MSc, FRCPC15,161Department of Hematology, CHU Sainte-Justine and Hôpital Maisonneuve-Rosemont, Montréal, Québec, Canada2Department of Medicine, Université de Montréal, Montréal, Québec, Canada3Child Health Evaluative Sciences, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada4Hemophilia and Thrombosis Center, University of Colorado, Denver, Colorado, USA5Department of Physical Medicine & Rehabilitation, Christian Medical College, Vellore, India6Haemostasis and Thrombosis Centre, St Thomas’ Hospital, London, UK7Pediatric/Adult Comprehensive Hemostasis Center, CHU Sainte-Justine, Montréal, Québec, Canada8Katharine Dormandy Haemophilia and Thrombosis Centre, The Royal Free Hospital, London, UK9Haemostasis and Thrombosis Unit, Division of Haematology, Cliniques Universitaires Saint- Luc, Brussels, Belgium10The Center for Inherited Blood Disorders (CIBD), Orange County, California, USA11Indiana Hemophilia & Thrombosis Center, Indianapolis, Indiana, USA12Haemophilia, Haemostasis & Thrombosis Centre, Hampshire Hospitals NHS Foundation Trust, Basingstoke, UK13Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada14Department of Pediatrics, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada15Division of Rheumatology, The Hospital for Sick Children, Toronto, Ontario, Canada16Department of Pediatrics, Faculty of Medicine, Institute of Health Policy, Management and Evaluation, The Dalla Lana School of Public Health, University of Toronto, Toronto, Ontario, Canada The authors regret a technical error in the graph-generating software resulted in an inaccurate visual representation for the moderate group in Figure 1. The bar graphs were not representative of the actual data obtained in the moderate hemophilia group. The correct bar graphs for the moderate group should display a Median HJHS total score of 27.5 in those 30 to 40 years; 29.5 in those 41 to 50 years, and 29.0 in those > 50 years. This corrected figure does not alter the original data, analysis, or conclusions.[Formula presented] The authors would like to apologise for any inconvenience caused. © 2023

The authors regret the affiliation of Clinical Pathology Department, Faculty of Medicine, Mansoura University, Egypt was omitted for Maha Othman. This has been corrected and added as below. The authors would like to apologise for any inconvenience caused. © 2023

Severe congenital protein C deficiency (SCPCD) is rare and there is currently substantial variation in the management of this condition. A joint project by three Scientific and Standardization Committees of the ISTH: Plasma Coagulation Inhibitors, Pediatric/Neonatal Thrombosis and Hemostasis, and Women’s Health Issues in Thrombosis and Hemostasis, was developed to review the current evidence and help guide on diagnosis and management of SCPCD. We provide a summary of the clinical presentations, differential diagnoses, appropriate investigations to confirm the diagnosis, approaches for management of the acute situation, and options for long-term management including subsequent pregnancies. We finally provide a set of recommendations to help in this regard. © 2022 The Authors. Journal of Thrombosis and Haemostasis published by Wiley Periodicals LLC on behalf of International Society on Thrombosis and Haemostasis.

Objective To characterise heart failure (HF) maintenance pharmacotherapy for children across Europe and investigate how angiotensin-converting enzyme inhibitors (ACE-I) are used in this setting. Methods A Europe-wide web-based survey was conducted between January and May 2015 among European paediatricians dedicated to cardiology. Results Out of 200-eligible, 100 physicians representing 100 hospitals in 27 European countries participated. All participants reported prescribing ACE-I to treat dilated cardiomyopathy-related HF and 97% in the context of congenital heart defects; 87% for single ventricle physiology. Twenty-six per cent avoid ACE-I in newborns. Captopril was most frequently selected as first-choice for newborns (73%) and infants and toddlers (66%) and enalapril for children (56%) and adolescents (58%). Reported starting and maintenance doses varied widely. Up to 72% of participants follow formal creatinine increase limits for decision-making when up-titrating; however, heterogeneity in the cut-off points selected existed. ACE-I formulations prescribed by 47% of participants are obtained from more than a single source. Regarding symptomatic HF maintenance therapy, 25 different initial drug combinations were reported, although 79% select a regimen that includes ACE-I and diuretic (thiazide and/or loop), 61% ACE-I and aldosterone antagonist; 44% start with beta-blocker, 52% use beta-blockers as an add-on drug. Of the 89 participants that prescribe pharmacotherapy to asymptomatic patients, 40% do not use ACE-I monotherapy or ACE-I-beta-blocker two-drug only combination. Conclusions Despite some reluctance to use them in newborns, ACE-I seem key in paediatric HF treatment strategies. Use in single ventricle patients seems frequent, in apparent contradiction with current paediatric evidence. Disparate dosage criteria and potential formulation-induced variability suggest significant differences may exist in the risk-benefit profile children are exposed to. No uniformity seems to exist in the drug regimens in use. The information collected provides relevant insight into real-life clinical practice and may facilitate research to identify the best therapeutic options for HF children. © 2019 BMJ Publishing Group Limited.