دورية أكاديمية
أعرض في EDS

Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia.

التفاصيل البيبلوغرافية
العنوان: Elevated liver iron concentration is a marker of increased morbidity in patients with β thalassemia intermedia.
المؤلفون: Musallam KM; Department of Internal Medicine, Division of Hematology & Oncology, American University of Beirut Medical Center, Beirut, Lebanon., Cappellini MD, Wood JC, Motta I, Graziadei G, Tamim H, Taher AT
المصدر: Haematologica [Haematologica] 2011 Nov; Vol. 96 (11), pp. 1605-12. Date of Electronic Publication: 2011 Jul 26.
نوع المنشور: Clinical Trial; Journal Article; Multicenter Study
اللغة: English
بيانات الدورية: Publisher: Ferrata Storti Foundation Country of Publication: Italy NLM ID: 0417435 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1592-8721 (Electronic) Linking ISSN: 03906078 NLM ISO Abbreviation: Haematologica Subsets: MEDLINE
أسماء مطبوعة: Publication: 1999- : Pavia, Italy : Ferrata Storti Foundation
Original Publication: Pavia [etc.]
مواضيع طبية MeSH: Iron/*metabolism , Liver/*metabolism , beta-Thalassemia/*metabolism , beta-Thalassemia/*mortality, Adult ; Biomarkers/metabolism ; Blood Platelets/metabolism ; Blood Platelets/pathology ; Blood Transfusion ; Cross-Sectional Studies ; Erythrocyte Count ; Erythrocytes, Abnormal/metabolism ; Erythrocytes, Abnormal/pathology ; Female ; Ferritins/blood ; Fetal Hemoglobin/metabolism ; Humans ; Iron Overload/metabolism ; Iron Overload/mortality ; Iron Overload/pathology ; Liver/pathology ; Male ; Middle Aged ; Platelet Count ; Splenectomy ; beta-Thalassemia/pathology
مستخلص: Background: Patients with β thalassemia intermedia can have substantial iron overload, irrespectively of their transfusion status, secondary to increased intestinal iron absorption. This study evaluates whether iron overload in patients with β thalassemia intermedia is associated with morbidity.
Design and Methods: This was a cross-sectional study of 168 patients with β thalassemia intermedia treated at two centers in Lebanon and Italy. Data on demographics, splenectomy status, transfusion status, and presence of co-morbidities were retrieved. Laboratory values of serum ferritin, fetal and total hemoglobin levels, as well as platelet and nucleated red blood cell counts were also obtained. Iron burden was determined directly by measuring liver iron concentration using magnetic resonance imaging. Patients were subdivided according to transfusion and splenectomy status into groups with phenotypes of different severity.
Results: The mean age of the patients was 35.2 ± 12.6 years and 42.9% of them were male. The mean liver iron concentration was 8.4 ± 6.7 mg Fe/g dry weight. On multivariate logistic regression analysis, after adjusting for age, gender, splenectomy status, transfusion status, and laboratory indices, an increase in 1 mg Fe/g dry weight liver iron concentration was independently and significantly associated with higher odds of thrombosis, pulmonary hypertension, hypothyroidism, osteoporosis, and hypogonadism. A liver iron concentration of at least 7 and at least 6 mg Fe/g dry weight were the best thresholds for discriminating the presence and absence of vascular and endocrine/bone morbidities, respectively (area under the receiver-operating characteristic curve: 0.72, P<0.001). Elevated liver iron concentration was associated with an increased rate of morbidity in patients with phenotypes of all severity, with a steeper increase in the rate of vascular morbidity being attributed to aging, and an earlier appearance of endocrine and bone disease.
Conclusions: Elevated liver iron concentration in patients with β thalassemia intermedia is a marker of increased vascular, endocrine, and bone disease.
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المشرفين على المادة: 0 (Biomarkers)
9007-73-2 (Ferritins)
9034-63-3 (Fetal Hemoglobin)
E1UOL152H7 (Iron)
تواريخ الأحداث: Date Created: 20110728 Date Completed: 20120224 Latest Revision: 20220408
رمز التحديث: 20231215
مُعرف محوري في PubMed: PMC3208677
DOI: 10.3324/haematol.2011.047852
PMID: 21791471
قاعدة البيانات: MEDLINE
الوصف
تدمد:1592-8721
DOI:10.3324/haematol.2011.047852