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العنوان
Serum Hepcidin Relation With Iron Overload In Beta Thalassemia Patients /
المؤلف
Botros, Ayman Shafek Samuel.
هيئة الاعداد
باحث / ايمن شفيق صموئيل بطرس
مشرف / ابراهيم يوسف عبد المسيح
مشرف / سها رؤوف يوسف
مشرف / مهيرة إسماعيل الموجى
الموضوع
Diagnosis, Laboratory
تاريخ النشر
2014.
عدد الصفحات
187 p. :
اللغة
الإنجليزية
الدرجة
ماجستير
التخصص
أمراض الدم
تاريخ الإجازة
1/1/2014
مكان الإجازة
جامعة عين شمس - كلية الطب - قسم الباثولوجيا الاكلينيكية
الفهرس
Only 14 pages are availabe for public view

from 187

from 187

Abstract

Beta thalassemia encompasses a group of monogenic diseases characterized by a genetic deficiency in the synthesis of beta-globin chains. The defects involved are extremely heterogeneous and give rise to a large phenotypic spectrum, starting with patients that are almost asymptomatic to others in which regular blood transfusions are required to sustain life. As a result of the inefficient synthesis of β-globin, the patients suffer from chronic anemia due to a process called ineffective erythropoiesis (IE). The sequelae of IE lead to extramedullary hematopoiesis with massive splenomegaly and dramatic iron overload, which in turn is responsible for many of the secondary pathology observed in thalassemic patients.
Tissue iron overload is the most important complication of beta thalassemia and is a major focus of therapeutic management. Hepcidin is a liver-synthesized hormone that inhibits the cellular efflux of iron by binding to ferroportin and its subsequent degradation. The main role of hepcidin is regulation of ferroportin expression and cell membrane function.
Hepcidin deficiency is one of the main contributing factors of iron overload in iron-loading anemias such as beta thalassemia. Hepcidin deficiency results from a strong suppressive effect of the high erythropoietic activity on hepcidin expression. Although in thalassemia major patients iron absorption contributes less to the total iron load than transfusions, in non-transfused thalassemia, low hepcidin and the consequent hyperabsorption of dietary iron is the major cause of systemic iron overload. Hepcidin diagnostics and future therapeutic agonists may help in management of iron overload in patients with beta thalassemia.
This study aimed to measure the level of serum hepcidin in patients with beta thalassemia and to correlate its level with iron profile . This provides better understanding of the role of hepcidin in thalassemia patients and whether its assay could be useful for the diagnosis and monitoring of iron status.
This study was conducted on sixty patients, 18 males and 42 females, who have beta thalassemia and thirty healthy individuals as a control group.The patients group was further subdivided into three groups, thalassemia major, thalassemia intermedia and thalassemia minor. All individuals in this study were subjected to full clinical assessment, complete blood count, iron profile tests and serum hepcidin level by ELISA (DRG International Inc., USA).
Results showed a significantly lower BMI, height and lower TIBC as well as a significantly higher percent of cases with delayed puberty & higher level of serum ferritin, serum iron among thalassemia patients compared to controls.
The study found that serum hepcidin ranged from 50 to 240 ng/ml in the control group. Serum hepcidin level was lower in patients with β- thalassemia Patients than controls. As well, serum hepcidin level had negative correlation with serum iron, transferrin saturation, serum ferritin in patients with beta thalassemia.
Hepcidin/ferritn index was significantly lower in beta thalassemia patients compared to control group. Low hepcidin/ferritin index was associated with high serum iron, increased frequency of blood transfusion and delayed puberty. A cut off hepcidin/ferritin index of 0.027 was suggested as a differentiating point between thalassemia major and thalassemia intermedia patients.
In conclusion, serum hepcidin level may be of significant value in future diagnostic and therapeutic approaches of beta thalassemia patients. Hepcidin / ferritin index may be a useful tool for discriminating different clinical types of beta thalassemia patients and follow up these patients.