title: Study of serum hepcidin in hereditary hemolytic anemias.
abstract: The aim of this study was to assess the level of hepcidin in hereditary chronic hemolytic anemias and to correlate the serum hepcidin levels to the need for blood transfusions (frequency of blood transfusions and the serum ferritin level). Seventy pediatric patients with hereditary chronic hemolytic anemias, attending to hematology clinics of Cairo University and Misr University for Science and Technology (MUST) hospitals were the subjects of this study [53 patients with β-thalassemia major (β-TM), 10 patients with β-thalassemia intermedia (β-TI), four patients with congenital spherocytosis and three patients with sickle cell disease) (38 males and 32 females)]; their ages ranged from 1-14 years. Seventy normal children, age- and sex-matched, served as the control group. The results of this study revealed decreased hepcidin levels in patients (all types of congenital chronic hemolytic anemias) [mean ± SD (standard deviation) = 22.9 ± 6.0] compared to controls (mean ± SD = 132.4 ± 16.7) with highly significant statistical difference in between. Hepcidin levels were higher in β-TM patients (mean ± SD = 23.7 ± 6.2) than in β-TI patients (mean ± SD = 21.8 ± 4.0), the hepcidin to ferritin ratio was significantly less than one. In β-TM patients, the mean ± SD was 0.03 ± 0.004, and in β-TI patients the mean ± SD = 0.025 ± 0.002, with highly significant statistical difference with hepcidin-to-ferritin ratios in controls being mean ± SD = 2.3 ± 0.7. Hepcidin and hepcidin/ferritin ratios can be used as good markers of hemolytic anemia and iron overload as they have very high sensitivity (99.0 and 99.0%, respectively) and very high specificity (98.0 and 97.0%, respectively). Our findings highlight the potential usefulness of hepcidin measurement as a diagnostic tool. The use of hepcidin as an adjuvant therapy with iron chelators is important as it has a vital role in combating hemosidrosis.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Cairo University, Cairo, Egypt.
authors.forename: Amal
authors.initials: A
authors.lastname: El Beshlawy
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Alaraby
authors.forename: Mohamed S E M
authors.initials: MS
authors.lastname: Abdel Kader
authors.forename: Dina H
authors.initials: DH
authors.lastname: Ahmed
authors.forename: Hossam E M
authors.initials: HE
authors.lastname: Abdelrahman
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000628:therapy; D023181:Antimicrobial Cationic Peptides / Q000097:blood*; D001803:Blood Transfusion; D002097:C-Reactive Protein; D002648:Child; D002675:Child, Preschool; D005260:Female; D005293:Ferritins / Q000097:blood; D064451:Hepcidins; D006801:Humans; D007223:Infant; D007501:Iron / Q000097:blood; D019190:Iron Overload / Q000097:blood / Q000175:diagnosis / Q000209:etiology; D008297:Male; D013103:Spherocytosis, Hereditary / Q000097:blood* / Q000628:therapy; D017086:beta-Thalassemia / Q000097:blood* / Q000628:therapy
chemical_list: D023181:Antimicrobial Cationic Peptides; C578702:HAMP protein, human; D064451:Hepcidins; D002097:C-Reactive Protein; D005293:Ferritins; D007501:Iron