Liver iron overload and hepatic function in children with thalassemia major

  • Pustika Amalia Wahidiyat Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta https://orcid.org/0000-0002-5513-006X
  • Stephen Diah Iskandar Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Ludi Dhyani Rahmartani Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Damayanti Sekarsari Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Keywords: thalassemia; liver iron overload; MRI; hepatic function test

Abstract

Background Routine blood transfusions and increased intestinal iron absorption lead to iron accumulation in various organs, especially the liver. To date, T2-star magnetic resonance imaging (T2*MRI) is a valuable tool to evaluate iron level in organs.

Objective To assess the degree of liver iron overload among children with thalassemia major (TM) and its possible correlations with hepatic function laboratory values.

Methods This cross-sectional study was conducted in Cipto Mangunkusumo Hospital. The degree of liver iron overload was evaluated by T2*MRI. Assessments of serum aspartate aminotransferase (AST), alanine aminotransferase (ALT), albumin, and bilirubin levels were done to evaluate liver function.

Results A total of 291 TM children were included in this study. The mean age of subjects was 12 years. Most of the subjects were diagnosed as β-thalassemia homozygote (54.6%) and β-thalassemia/HbE (41.2%). Deferiprone (DFP) was the most commonly used iron chelator. Less than 10% of the subjects had normal liver iron deposition. The AST and ALT values increased proportionally with the severity of liver iron overload, with significant, moderately negative correlation coefficients (r=-0.388 and -0.434, respectively). However, albumin level decreased proportionally with the severity of liver iron overload, with a significant, moderately positive correlation coefficient (r=0.323). Liver T2* MRI had no significant correlations with direct, indirect, and ratio of direct/total bilirubin levels.

Conclusion Most of the children with TM have mild to severe liver iron overload. Liver T2* MRI had significant, moderate correlations with AST, ALT, and albumin values. Bilirubin level has no correlation with T2* MRI. Our findings suggest that monitoring of AST, ALT, and albumin levels is important because they may reflect the severity of liver iron overload. However, they should not be used as the only predictors of iron overload.

References

Fucharoen S, Winichagoon P. Haemoglobinopathies in Southeast Asia. Indian J Med Res 2011;134:498-506.

Langhi D, Ubiali EMA, Marques JFC, Verissimo MA, Loggetto SR, Silvinato A, et al. Guidelines on Beta-thalassemia major – regular blood transfusion therapy: Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular: project guidelines: Associação Médica Brasileira – 2016. Rev Bras Hematol Hemoter 2016;38:341–5.

Anderson ER, Shah YM. Iron hemostasis in the liver. Compr Physiol 2013;3:315-30.

Echeverria JMA, Castiella A, Emparanza JI. Quantification of iron concentration in the liver by MRI. Insights Imaging 2012;3:173-80.

Sirlin CB, Reeder SB. Magnetic resonance imaging quantification of liver iron. Magn Reson Imaging Clin N Am 2010;18:359-ix.

Kohgo Y, Ikuta K, Ohtake T, Torimoto Y, Kato J. Body iron metabolism and pathophysiology of iron overload. Int J Hematol 2008;88:7-15.

Imam MU, Zhang S, Ma J, Wang H, Wang F. Antioxidants mediate both iron hemostasis and oxidative stress. Nutrients 2017;9:671.

Gowda S, Desai PB, Hull VY, Math AAK, Vernekar SN, Kulkarni SS. A review on laboratory liver function tests. Pan Afr Med J 2009;3:17.

Kasarala G, Tillmann HL. Standard liver tests. Clin Liver Dis 2016;8:13-18.

Giannini EG, Testa R, Savarino V. Liver enzyme alteration: a guide for clinicians. CMAJ 2005;172:367-79.

Bernardi M, Maggioli C, Zaccherini G. Human albumin in the management of complication of liver cirrhosis. Crit Care 2012;16:211.

Garcovich M, Zocco MA, Gasbarrini A. Clinical use of albumin in hepatology. Blood Transfus 2009;7:268-77.

Thapa RR, Walia A. Liver function tests and their interpretation. Indian J Pediatr 2007;74:663-71.

Published
2018-09-27
How to Cite
1.
Wahidiyat P, Iskandar S, Rahmartani L, Sekarsari D. Liver iron overload and hepatic function in children with thalassemia major. PI [Internet]. 27Sep.2018 [cited 25Apr.2024];58(5):233-. Available from: https://www.paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/1885
Section
Pediatric Hemato-Oncology
Received 2018-06-13
Accepted 2018-09-25
Published 2018-09-27