People with rare lysosomal storage diseases face challenges in their care that arise from disease complexity and heterogeneity, compounded by many healthcare professionals being unfamiliar with these diseases. These challenges can result in long diagnostic journeys and inadequate care. Over 30 years ago, the Rare Disease Registries for Gaucher, Fabry, Mucopolysaccharidosis type I and Pompe diseases were established to address knowledge gaps in disease natural history, clinical manifestations of disease and treatment outcomes. Evidence generated from the real-world data collected in these registries supports multiple stakeholders, including patients, healthcare providers, drug developers, researchers and regulators. To maximise the impact of real-world evidence from these registries, engagement and collaboration with the patient communities is essential. To this end, the Rare Disease Registries Patient Council was established in 2019 as a partnership between the Rare Disease Registries and global and local patient advocacy groups to share perspectives on how registry data are used and disseminated. The Patient Council has resulted in a number of patient initiatives including patient representation at Rare Disease Registries advisory boards; development of plain language summaries of registry publications to increase availability of real-world evidence to patient communities; and implementation of digital innovations such as electronic patient-reported outcomes, and patient-facing registry reports and electronic consent (in development), all to enhance patient engagement. The Patient Council is building on the foundations of industry-patient advocacy group collaboration to fully integrate patient communities in decision-making and co-create solutions for the rare disease community.
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http://dx.doi.org/10.1186/s13023-024-03262-2 | DOI Listing |
J Orthop Surg Res
January 2025
Center of Medical Genetics, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, 610041, China.
Background: Delta large-channel endoscopy and unilateral biportal endoscopy (UBE) are prominent minimally invasive techniques for treating lumbar spinal stenosis, known for minimal tissue damage, clear visualization, and quick recovery. However, rigorous controlled research comparing these procedures is scarce, necessitating further investigation into their respective complications and long-term effectiveness. This randomized controlled trial aims to compare their perioperative outcomes, focusing on postoperative recovery and complications over time.
View Article and Find Full Text PDFHealth Qual Life Outcomes
January 2025
Department of Human Sciences, LUMSA University, Rome, 00193, Italy.
Background: The number of people living with congenital heart disease (CHD) in 2017 was estimated to be 12 million, which was 19% higher than that in 1990. However, their death rate declined by 35%, emphasizing the importance of monitoring their quality of life due to its impact on several patient outcomes. The main objective of this study is to analyze how parents' psychosocial factors contribute to children's and adolescents' perceptions of their QoL, focusing on their medical condition.
View Article and Find Full Text PDFBMC Pregnancy Childbirth
January 2025
Department of Clinical Genetics, Rennes University Hospital, Rennes, France.
Background: Mucopolysaccharidosis type I (MPS I - IDUA gene) is a rare autosomal recessive lysosomal storage disorder. Clinical symptoms, including visceral overload, are progressive and typically begin postnatally. Descriptions of hepatosplenomegaly associated with lysosomal pathology are uncommon during the prenatal period.
View Article and Find Full Text PDFMed Biol Eng Comput
January 2025
Department of Computer Science and Engineering, Shri Shankaracharya Institute of Professional Management and Technology, Raipur, (C.G.), India.
This study presents an advanced methodology for 3D heart reconstruction using a combination of deep learning models and computational techniques, addressing critical challenges in cardiac modeling and segmentation. A multi-dataset approach was employed, including data from the UK Biobank, MICCAI Multi-Modality Whole Heart Segmentation (MM-WHS) challenge, and clinical datasets of congenital heart disease. Preprocessing steps involved segmentation, intensity normalization, and mesh generation, while the reconstruction was performed using a blend of statistical shape modeling (SSM), graph convolutional networks (GCNs), and progressive GANs.
View Article and Find Full Text PDFTransplant Proc
January 2025
Department of Thoracic Surgery and Lung Transplantation, University Hospital Reina Sofía, Córdoba, Spain; Instituto Maimónides de Investigación Biomédica de Córdoba, IMIBIC, University of Córdoba, Córdoba, Spain; Group for the Study of Thoracic Neoplasms and Lung Transplantation, IMIBIC (Instituto Maimónides de Investigación Biomédica de Córdoba), University of Córdoba, Córdoba, Spain. Electronic address:
Introduction: Mucorales infections in the airways of lung transplant (LT) patients are rare but have a rising incidence in transplanted lungs.
Objective: We present our experience with LT in immediate postoperative infections due to mucormycosis.
Methods: Review of 767 LT performed in Andalusia between 2000 and 2023 identifying Mucorales through microbiological results and histological findings.
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