EXPLORE B : A prospective, long-term natural history study of patients with acute hepatic porphyria with chronic symptoms

التفاصيل البيبلوغرافية
العنوان: EXPLORE B : A prospective, long-term natural history study of patients with acute hepatic porphyria with chronic symptoms
المؤلفون: David Cassiman, Raili Kauppinen, Susana Monroy, Ming‐Jen Lee, Herbert L. Bonkovsky, Manish Thapar, Encarna Guillén‐Navarro, Anna‐Elisabeth Minder, Cecilia Hale, Marianne T. Sweetser, Aneta Ivanova
المساهمون: HUS Internal Medicine and Rehabilitation, Clinicum, Department of Medicine, Helsinki University Hospital Area
سنة النشر: 2022
مصطلحات موضوعية: COMPLICATIONS, QUESTIONNAIRE, Pain, hepatic complications, EORTC QLQ-C30, chronic symptoms, DIAGNOSIS, ACUTE INTERMITTENT PORPHYRIA, prospective studies, RECOMMENDATIONS, Porphyrias, Hepatic, disease burden, QUALITY-OF-LIFE, Porphyria, Acute Intermittent, 3121 General medicine, internal medicine and other clinical medicine, Genetics, Quality of Life, porphyria attack, MANAGEMENT, Humans, Hemin, VALIDITY, Genetics (clinical), ATTACKS, porphyrias
الوصف: One-year data from EXPLORE Part A showed high disease burden and impaired quality of life (QOL) in patients with acute hepatic porphyria (AHP) with recurrent attacks. We report baseline data of patients who enrolled in EXPLORE Part B for up to an additional 3 years of follow-up. EXPLORE B is a long-term, prospective study evaluating disease activity, pain intensity, and QOL in patients with AHP with ≥1 attack in the 12 months before enrollment or receiving hemin or gonadotropin-releasing hormone prophylaxis. Data were evaluated in patients with more (≥3 attacks or on prophylaxis treatment) or fewer (
وصف الملف: Print-Electronic
اللغة: English
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::51ba88da64a03ddf281cdff0f121d88dTest
http://hdl.handle.net/10138/354211Test
حقوق: OPEN
رقم الانضمام: edsair.doi.dedup.....51ba88da64a03ddf281cdff0f121d88d
قاعدة البيانات: OpenAIRE