Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major

التفاصيل البيبلوغرافية
العنوان: Hepatic and Cardiac Iron-load in Children on Long-term Chelation with Deferiprone for Thalassemia Major
المؤلفون: Reena Das, Kushaljit Singh Sodhi, Richa Jain, Anmol Bhatia, Niranjan Khandelwal, Amita Trehan, Prateek Bhatia, Deepak Bansal, Sidharth Totadri, Alka Khadwal
المصدر: Indian Pediatrics. 55:573-575
بيانات النشر: Springer Science and Business Media LLC, 2018.
سنة النشر: 2018
مصطلحات موضوعية: Adult, Male, medicine.medical_specialty, Liver Iron Concentration, Iron Overload, Adolescent, Pyridones, Iron, Thalassemia, Myocardial iron, Iron Chelating Agents, Gastroenterology, Drug Administration Schedule, 030218 nuclear medicine & medical imaging, Young Adult, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Internal medicine, medicine, Cardiac iron, Humans, Deferiprone, In patient, Hepatic iron, Child, Serum ferritin, business.industry, Myocardium, beta-Thalassemia, medicine.disease, Cross-Sectional Studies, Treatment Outcome, Liver, chemistry, Pediatrics, Perinatology and Child Health, Female, business, Biomarkers, 030215 immunology
الوصف: OBJECTIVE To evaluate the efficacy of prolonged deferiprone monotherapy in patients with b-thalassemia major. METHODS This cross-sectional study included 40 patients (age range 9 to 38 years) with thalassemia major receiving deferiprone for ≥5 years. Serum ferritin, and myocardial iron concentration (MIC) and liver iron concentration (LIC) assessed by T2*MRI were recorded. RESULTS The patients were receiving deferiprone for a mean (SD) duration of 12.1 (4.7) years. The median (IQR) dose of deferiprone was 85 (74.3, 95) mg/kg/day. The MIC was normal or had a mild, moderate or severe elevation in 29 (72.5%), 3 (7.5%), 3 (7.5%), and 5 (12.5%) patients. The LIC was normal or had a mild, moderate or severe elevation in 2 (5%), 4 (10%), 11 (27.5%) and 23 (57.5%) patients. CONCLUSIONS The majority of patients receiving deferiprone had a moderate/severe hepatic but normal cardiac iron load. Prolonged deferiprone monotherapy was suboptimal for hepatic iron load in the majority.
تدمد: 0974-7559
0019-6061
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7edf1dc824aa7498d12e5fd108dc5c5eTest
https://doi.org/10.1007/s13312-018-1299-zTest
حقوق: OPEN
رقم الانضمام: edsair.doi.dedup.....7edf1dc824aa7498d12e5fd108dc5c5e
قاعدة البيانات: OpenAIRE