• BLOOD RESEARCH
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• 제53권4호
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• pp.314-319
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• 2018

Background Iron overload is a risk factor affecting all patients with thalassemia intermedia (TI). We aimed to determine whether there is a relationship of serum ferritin (SF) and alanine aminotransferase (ALT) with liver iron concentration (LIC) determined by R2 magnetic resonance imaging (R2-MRI), to estimate the most relevant degree of iron overload and best time to chelate in patients with TI. Methods In this cross-sectional study, 119 patients with TI (mean age years) were randomly selected and compared with 120 patients who had a diagnosis of thalassemia major (TM). Correlations of LIC, as determined by R2-MRI, with SF and ALT levels, were assessed in all participants. A P-value <0.05 was considered statistically significant. Results SF and LIC levels were lower in patients with TI than in those with TM; only ferritin values were significant. We found a statistically significant relationship between SF and LIC, with cut-off estimates of SF in patients with TI who had splenectomy and those who entered puberty spontaneously (916 and 940 ng/mL, respectively) with LIC >5 mg Fe/g dry weight (P<0.0001). A significant relationship was also found for patients with TI who had elevated ALT level (63.5 U/L), of 3.15 times the upper normal laboratory limit, using a cut-off for LIC ${\geq}5mg\;Fe/g\;dry\;weight$. Conclusion We determined the cut-off values for ALT and SF indicating the best time to start iron chelation therapy in patients with TI, and found significant correlations among iron overload, SF, and ALT.

• Clinical and Experimental Pediatrics
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• 제45권5호
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• pp.659-663
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• 2002

저자들은 상염색체 우성으로 유전되는 경한 저색소성 소구성 빈혈을 보이고 ${\beta}$ 유전자의 127번째 코돈 이 CAG에서 CGG로 치환되는 과오돌연변이로 인하여 매우 불안정한 베타 사슬 변이체를 만드는 우성유전 베타 지중해빈혈을 경험하였기에 문헌고찰과 함께 보고하는 바이다.