An international patient-centred study of retroperitoneal fibrosis.

Autor:	Dattani R; West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK., Barwick TD; Department of Surgery and Cancer, Imperial College London, London, UK., El Wardany G; Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK., Gibbons N; Department of Urology, Imperial College Healthcare NHS Trust, London, UK., Mason JC; Imperial College London, National Heart and Lung Institute London, London, UK., Morgan P; Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK., Pusey CD; West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.; Imperial College London, Centre for inflammatory Disease London, London, UK., Tam FWK; West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.; Imperial College London, Centre for inflammatory Disease London, London, UK., Tomlinson JAP; West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.
Jazyk:	angličtina
Zdroj:	QJM : monthly journal of the Association of Physicians [QJM] 2022 Mar 22; Vol. 115 (3), pp. 148-154.
DOI:	10.1093/qjmed/hcaa327
Abstrakt:	Background: The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. Aim: To understand the physical and psychosocial impact that RPF has upon peoples' lives. Design: An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. Methods: An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. Results: A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. Conclusion: This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health. (© The Author(s) 2020. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For permissions, please email: journals.permissions@oup.com.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu