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Serum Hepcidin as a Diagnostic Marker of Severe Iron Overload in Beta-thalassemia Major
Ahmed Maher Kaddah1, Amina Abdel-Salam2, Marwa Salah Farhan3
1Department of Pediatrics, Faculty of Medicine, Cairo University, Ali Ibrahim Basha St., Cairo, Egypt.
Insights
Serum hepcidin is elevated in children with beta-thalassemia, particularly in those with severe iron overload. This suggests hepcidin may serve as a valuable diagnostic marker for iron overload in thalassemia major patients.
Area of Science:
- Biochemistry
- Pediatric Hematology
Background:
- Beta-thalassemia is a genetic blood disorder requiring frequent transfusions, leading to iron overload.
- Accurate diagnosis of iron overload is crucial for managing complications in children with beta-thalassemia.
Purpose of the Study:
- To evaluate the diagnostic utility of serum hepcidin levels for detecting iron overload in pediatric beta-thalassemia patients.
- To compare serum hepcidin with other markers in assessing iron overload severity.
Main Methods:
- Serum hepcidin levels were measured using ELISA in 30 thalassemia major (TM), 30 thalassemia intermedia (TI) patients, and 60 healthy controls.
- Correlations between hepcidin, age, disease duration, transfusion history, hemoglobin, and ferritin levels were analyzed.
Main Results:
- Children with beta-thalassemia exhibited significantly higher serum hepcidin than controls.
- Thalassemia major patients showed higher hepcidin and ferritin levels compared to thalassemia intermedia patients.
- Elevated serum hepcidin was independently associated with severe iron overload (serum ferritin ≥ 1500 ng/ml) in TM patients.
Conclusions:
- Serum hepcidin is elevated in children with beta-thalassemia, with higher levels observed in TM patients experiencing severe iron overload.
- Hepcidin shows potential as a biomarker for severe iron overload in thalassemia major.
- Further research is recommended to compare hepcidin and ferritin in predicting severe iron overload under various clinical conditions.
Objectives:
To investigate potential usefulness of serum hepcidin in the diagnosis of iron overload in children with β-thalassemia.
Methods:
A study was conducted on 30 thalassemia major (TM), 30 thalassemia intermedia (TI) and 60 healthy children as controls. Serum hepcidin was measured by Human Hepcidin, ELISA Kit.
Results:
β-thalassemia patients had a higher serum hepcidin compared to the controls (p < 0.001). TM group had higher hepcidin and ferritin compared to the TI group (p = 0.034; < 0.001, respectively). Among controls, hepcidin did not correlate with age (r = 0.225, p = 0.084). Among β-thalassemia patients, it correlated positively with age (r = 0.4; p = 0.001), disease duration (r = 0.5; p < 0.001), transfusion frequency (r = 0.35; p = 0.007), total number of transfusions (r = 0.4; p = 0.003), and ferritin (r = 0.3; p = 0.027). Total hemoglobin and serum ferritin were significantly related to hepcidin, which tended to increase by 0.514 ng/ml with each 1 g/dl rise in hemoglobin (p = 0.023) and by 0.002 ng/ml with each 1 ng/ml rise in serum ferritin (p = 0.002). Iron overload [serum ferritin (SF) ≥ 1500 ng/ml] was independently associated with TM (p = 0.001) and elevated serum hepcidin (p = 0.02). The overall predictability of serum hepcidin in severe iron overload was statistically significant when compared to hepcidin to serum ferritin ratio.
Conclusions:
Serum hepcidin is elevated in children with β-thalassemia; but this elevation is more evident in TM patients with severe iron overload. Thus, hepcidin can be a potential marker of severe iron overload in patients with TM. Further studies are recommended to compare serum hepcidin and serum ferritin in the prediction of severe iron overload in steady state and during infection or inflammation.
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