title: Peripheral expression of hepcidin gene in Egyptian β-thalassemia major.
abstract: Iron overload is the major cause of morbidity and mortality in transfusion dependent β-thalassemia major patients. There is a sophisticated balance of body iron metabolism of storage and transport which is regulated by several factors including the peptide hepcidin. Hepcidin is the main iron regulatory molecule; it is secreted mainly by the liver and other tissues including monocytes and lymphocytes. Expression of hepcidin in such cells is unclear and has been studied in few reports with controverted result. Peripheral expression of hepcidin was measured using quantitative real time PCR (qRT-PCR) in 50 β-thalassemia major patients, in addition to 20 healthy volunteers as a control group. Hepcidin levels in β-thalassemia major patients showed statistically significant decrease in comparison to the control group, and was correlated to cardiac iron stores (T2*). However, hepcidin level was not different among the patients according to the HCV status or whether splenectomized or not. In conclusion; peripheral expression of hepcidin, in iron overloaded β-thalassemia major patients, is a reflection of hepatic expression. It can be used as a molecular predictor for the severity of cardiac iron overload and can be used as a future target for therapy in β-thalassemia major patients.
authors: Clinical Pathology Department, Cairo University, Cairo, Egypt.
authors: Azza
authors: A
authors: Aboul-Enein
authors: Pediatric Department, Cairo University, Cairo, Egypt.
authors: Amal
authors: A
authors: El-Beshlawy
authors: Pediatric Department, Cairo University, Cairo, Egypt.
authors: Mona
authors: M
authors: Hamdy
authors: Clinical Pathology Department, Cairo University, Cairo, Egypt.
authors: Iman
authors: I
authors: Shaheen
authors: Clinical Pathology Department, Cairo University, Cairo, Egypt.
authors: Zainab
authors: Z
authors: El-Saadany
authors: Radiology Department, Ain Shams University, Cairo, Egypt.
authors: Ahmed
authors: A
authors: Samir
authors: Clinical Pathology Department, Cairo University, Cairo, Egypt.
authors: Hala Abd
authors: HA
authors: El-Samie
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D004534:Egypt; D005260:Female; D064451:Hepcidins / Q000032:analysis / Q000235:genetics*; D006801:Humans; D019190:Iron Overload / Q000209:etiology*; D008099:Liver / Q000378:metabolism; D008214:Lymphocytes / Q000378:metabolism; D008297:Male; D009000:Monocytes / Q000378:metabolism; D060888:Real-Time Polymerase Chain Reaction; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000235:genetics
chemical_list: D064451:Hepcidins
keywords: Cardiac iron (T2*); Hepcidin expression; qRT-PCR; β-Thalassemia