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Assessment and management of iron overload in β‐thalassaemia major patients during the 21st century: a real‐life experience from the Italian Webthal project

脱铁酮 去铁斯若 医学 去铁胺 铁质沉着 肌酐 内科学 铁蛋白 地中海贫血 胃肠病学 儿科
作者
Antonio Piga,Filomena Longo,Khaled M. Musallam,Maria Domenica Cappellini,Gian Luca Forni,Giovanni Quarta,Francesco Chiavilli,Francesca Valeria Commendatore,Sergio Mulas,Vincenzo Caruso,Renzo Galanello
出处
期刊:British Journal of Haematology [Wiley]
卷期号:161 (6): 872-883 被引量:32
标识
DOI:10.1111/bjh.12340
摘要

Summary We conducted a cross‐sectional study on 924 β‐thalassaemia major patients (mean age 30·1 years) treated at nine Italian centres using the webthal software, to evaluate real‐life application of iron overload assessment and management standards. S erum ferritin <2500 ng/ml was a risk factor for never having liver iron concentration ( LIC ) measurement, while absence of cardiac disease and siderosis were risk factors for a delay in LIC measurement >2 years. Patients who never had a cardiac MRI ( CMR ) T 2* measurement were <18 years, had iron intake ≤0·4 mg/kg per day, or a serum ferritin <2500 ng/ml. A history of normal CMR T 2* was the main risk factor for a delay in subsequent assessment of >2 years. Deferoxamine (22·8%) was more commonly used in patients with Hepatitis C Virus or high serum creatinine. Deferiprone (20·6%) was less commonly prescribed in patients with elevated alanine aminotransferase; while a deferoxamine + deferiprone combination (17·9%) was more commonly used in patients with serum ferritin >2500 ng/ml or CMR T2* <20 ms. Deferasirox (38·3%) was more commonly prescribed in patients <18 years, but less commonly used in those with heart disease or high iron intake. These observations largely echoed guidelines at the time, although some practices are expected to change in light of evolving evidence.
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