Haemophilia
Department of Hematology, La Paz University Hospital-IdiPAZ, Autonomous University of Madrid, Madrid, Spain.
Published: May 2024
Introduction: Tailored prophylaxis is the current treatment regimen for patients with severe haemophilia A. Recently, published guidelines describe two possible approaches, based on clinical characteristics or estimation of pharmacokinetic parameters. However, both have strengths and weaknesses, and their characteristics need to be integrated to optimize treatment appropriately. In this paper, we present a model that considers together the characteristics of prophylaxis and the relevance of each.
Methods: The age at initiation of prophylaxis, number of bleeding events, treatment regimen, therapeutic adherence, FVIII trough levels, and joint status were analyzed in 59 patients followed at La Paz University Hospital between January 2000 and December 2019.
Results: The mean duration of primary prophylaxis of 113.37 ± 57.79 months. Eighty-three percent (n = 49) had no joint status involvement at the end of follow-up (HJHS and HEAD-US = 0). The median ABR was 0.7 (IQR 0.2 -1.0) and 54.2% presented trough levels of FVIII during follow-up >1 IU/dL. 72,9% engaged in some type of physical activity and overall adherence was over 85% in all patients evaluated. The regression analysis performed, considering all these factors, showed that the initiation of prophylaxis before 21 months of age was the most relevant protective factor against the appearance of joint involvement (OR 88.33 p.031 CI 95% 1.49-5224.40) CONCLUSION: Early initiation of prophylaxis was the most relevant factor in the protection of joint status. More comprehensive analysis models adapted to the characteristics of each population, are needed to adequately individualize treatment.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1111/hae.15014 | DOI Listing |
Otol Neurotol
February 2025
Edwin L. Steele Laboratories, Department of Radiation Oncology, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts.
Background Introduction: Vestibular schwannoma (VS) tumors typically present with sensorineural hearing loss (SNHL). Losartan has recently demonstrated prevention of tumor-associated SNHL in a mouse model of VS through suppression of inflammatory and pro-fibrotic factors, and the current study investigates this association in humans.
Methods: This is a retrospective study of patients with unilateral VS and hypertension followed with sequential audiometry at a tertiary referral hospital from January 1994 to June 2023.
J Healthc Manag
January 2025
Department of Obstetrics and Gynecology, MedStar Washington Hospital Center, Washington, DC.
Goal: Burnout, decreased professional fulfillment, and resultant attrition across the medical professions are increasingly recognized as threats to sustainable and cost-effective healthcare delivery. While the skill level of leaders as perceived by their direct reports has been correlated with rates of burnout and fulfillment, no studies, to our knowledge, have directly evaluated whether intervention via leadership training impacts burnout and fulfillment among direct reports. The goal of this study was to evaluate the effectiveness of a leadership training intervention on direct reports' perceptions of the leadership skills of supervising residents and subsequently on the well-being of the direct reports.
View Article and Find Full Text PDFPLoS One
January 2025
Division of Emergency Medicine, University of Cape Town, Cape Town, South Africa.
To validate Palestine's previously derived emergency department quality standards (EDQS) using an e-Delphi survey. A two-round e-Delphi survey validated the EDQS, developed in an earlier study through a literature review and consensus-building among Palestinian emergency medicine and healthcare quality experts. The study purposively sampled 53 emergency department and healthcare quality experts with over 5 years of experience.
View Article and Find Full Text PDFHealthcare (Basel)
January 2025
Emergency Medical Teams, Country Readiness Strengthening Department, World Health Organization, 1211 Geneva, Switzerland.
: Failure mode and effect analysis (FMEA) is a valuable risk analysis tool aimed at predicting the potential failures of a system and preventing them from occurring. Since its initial use, it has also recently been applied to the healthcare setting, which has been made progressively more complex by technological developments and new challenges. Infection prevention and control (IPC) is an area that requires effective strategies.
View Article and Find Full Text PDFEpidemiol Infect
January 2025
Health Protection Operations, South West, UK Health Security Agency, Bristol, UK.
In September 2023, the UK Health Security Agency's (UKHSA) South West Health Protection Team received notification of patients with perichondritis. All five cases had attended the same cosmetic piercing studio and a multi-disciplinary outbreak control investigation was subsequently initiated. An additional five cases attending the same studio were found.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!
© LitMetric 2025. All rights reserved.