<p>Long-term blood transfusion (BT) therapy in sickle cell disease (SCD) is inevitably complicated by iron overload (IO). Around 7% of SCD cases’ mortality is attributed to IO. Therefore, monitoring of body iron is crucial. In the present study, we aimed to assess liver iron concentration (LIC) by hepatic T2* MRI and its correlation with iron metabolism markers. Ninety pediatric SCD patients, aged 6–18 years, were enrolled in this study. All participants underwent history taking, clinical examination, laboratory assessment (including serum ferritin (SF), transferrin saturation %, serum hepcidin and its gene expression), and measurement of LIC by hepatic FerriScan (T2*) MRI (positive Ferriscan means LIC &gt; 2&#xa0;mg/g). Patients were classified into 2 groups based on the FerriScan positivity: group I (80 SCD cases with positive Ferriscan) and group II (10 SCD cases with negative FerriScan). The continuum of iron overload severity was assessed by the degree of positive liver FerriScan (T2*) findings. Thirty-seven (% 41.1%), 38(42.2%) and 5(5.6%) of the enrolled cases had mild, moderate, and severe +ve FerriScan (+ ve T2*) respectively. The continuum in the severity of iron overload was not significantly related to disease duration (<i>P =</i> 0.507). The SF level and ferritin/ hepcidin ratio were higher in cases with +ve T2* than -ve T2* cases (<i>P</i> &lt; 0.001). Logistic regression analysis showed significant association of frequency of blood transfusion (BT) with +ve FerriScan [<i>p</i> = 0.025 and OR 0.846 with CI (0.731–0.979)]. In addition, a positive correlation was found between SF and BT frequency in SCD patients with +ve T2* (<i>r</i> = 0.35, <i>p</i> = 0.001). Hepatic ferriscan is considered a non-invasive tool for early detection of iron overload, irrespective of disease duration. However, its role in monitoring remains questionable; further studies are recommended .Regular screening using both serum ferritin in conjunction with FerriScan analysis enables early detection of iron overload in SCD and timely therapeutic intervention.</p>

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Hepatic FerriScan in Evaluating Iron Overload in Pediatric Sickle Cell Disease

  • Eman A. Elbostany,
  • Eman A. Elghoroury,
  • Nehal Abdelhamid,
  • Hanan M. Hamed,
  • Eman H. Thabetb,
  • Hadeel M. Seif El Dein,
  • Amany M. Abd Al-Aziz,
  • Shereen H. Abd El Aziz,
  • Sonia A. Habib,
  • Eman Eltahlawy

摘要

Long-term blood transfusion (BT) therapy in sickle cell disease (SCD) is inevitably complicated by iron overload (IO). Around 7% of SCD cases’ mortality is attributed to IO. Therefore, monitoring of body iron is crucial. In the present study, we aimed to assess liver iron concentration (LIC) by hepatic T2* MRI and its correlation with iron metabolism markers. Ninety pediatric SCD patients, aged 6–18 years, were enrolled in this study. All participants underwent history taking, clinical examination, laboratory assessment (including serum ferritin (SF), transferrin saturation %, serum hepcidin and its gene expression), and measurement of LIC by hepatic FerriScan (T2*) MRI (positive Ferriscan means LIC > 2 mg/g). Patients were classified into 2 groups based on the FerriScan positivity: group I (80 SCD cases with positive Ferriscan) and group II (10 SCD cases with negative FerriScan). The continuum of iron overload severity was assessed by the degree of positive liver FerriScan (T2*) findings. Thirty-seven (% 41.1%), 38(42.2%) and 5(5.6%) of the enrolled cases had mild, moderate, and severe +ve FerriScan (+ ve T2*) respectively. The continuum in the severity of iron overload was not significantly related to disease duration (P = 0.507). The SF level and ferritin/ hepcidin ratio were higher in cases with +ve T2* than -ve T2* cases (P < 0.001). Logistic regression analysis showed significant association of frequency of blood transfusion (BT) with +ve FerriScan [p = 0.025 and OR 0.846 with CI (0.731–0.979)]. In addition, a positive correlation was found between SF and BT frequency in SCD patients with +ve T2* (r = 0.35, p = 0.001). Hepatic ferriscan is considered a non-invasive tool for early detection of iron overload, irrespective of disease duration. However, its role in monitoring remains questionable; further studies are recommended .Regular screening using both serum ferritin in conjunction with FerriScan analysis enables early detection of iron overload in SCD and timely therapeutic intervention.