Some aspects of thyroid dysfunction in thalassemia major patients with severe iron overload

  • Cynthia Rindang Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Jose R. L. Batubara Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Pustika Amalia Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
  • Hindra Satari Department of Child Health, University of Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
DOI: https://doi.org/10.14238/pi51.2.2011.66-72
Keywords: thala ssemia major, endocrine, primary hypothyroidism, iron overload, iron chelation therapy

Abstract

Background Severe iron overload due to recurrent transfusions for chronic anemia and inadequate iron chelation therapy in thalassemia major patients result in various complications, including hypothyroidism. Currently, there has been no data on the prevalence of hypothyroidism in thalassemia major patients at the Thalassemia Centers, Department of Child Health, Cipto
Mangunkusumo Hospital (DCH CMH).

Objective To study the prevalence of primary hypothyroidism in thalassemia major patients in the Thalassemia Center, DCH MCH.

Methods We performed a cross-sectional, descriptive study. All thalassemia major subjects aged Oô€¬18 years with severe iron overload underwent thyroid functionexamination. Primary hypothyroidism was defined as either normal (compensated) or decreased (decompensated) free T4 (FT4) levels, along with elevated sensitive thyroidô€¬stimulatinghonnone (TSH)levels. Results 179 subjects enrolled this study Mth male: female ratio of 1: 1.6. The prevalence of primary hypothyroidism in thalassemia major
patients Mth severe iron overloadws26.8% (48/179). Of those 48,45 had compensated hypothyroidism and 3 had decompensated hypothyroidism, 25.1% and 1.7% of the total subjects, respectively. Compensated hypothyroidism was observed in 17 subjects aged ≤1O years and in 28 subjects aged> 10 years. All 3 decompensated hypothyroidism cases were> 10 years of age. No relationship was found between the occurrence of primary hypothyroidism and mean pre-tr811sfusion Hb levels (P=0.481, OR 1.30; 95% CI 0.63 to 2.68), elevated serum ferritin levels (P=0.74, OR 0.89; 95% CI 0.46 to 1.75), and compliance to iron chelation therapy (P=0.570, OR 0.76; 95% CI 035 to 1.65). Based on multivariate analysis, only age of <10 year-old (P=O.029, OR 0.469; 95% CI 0.23 to 0.93) was significantly associated Mth primary hypJthyroidism. Further analysis using receiver operator curve (ROC) technique found that age of 8.5 year-old was the cutoff value to predict the risk of hypothyroidism. 

Conclusion The prevalence of primary hypothyroidism in our study is high. The occurrence of hypothyroidism is associated with age.

References

1. Mohammadian S. Endocrine gland abnormalities in thalassemia major: a brief review. J Pediatr Endocrinol Metab. 2003;16:957-64.
2. Potter A, Philips III JA. Genetic disorders in pediatric endocrinology. In: Pescovitz OH, Eugster EA, editors. Pediatric endocrinology: mechanism, manifestation, and management. 1st ed. Philadelphia: Lippincott Williams & Wilkins; 2004. p.l-33.
3. Weatherall DJ. Thalassemia in the next millenium. Ann N Y Acad Sci. 1998;850:1-9.
4. Olivieri NF, Brittenham GM.lron􀁊chelating therapy and the treatment of thalassemia. Blood. 1997;89:739-61.
5. Olivieri NF. T h e b e ta􀁊thalassemia. N Engl J Med. 2005;341:99-109.
6. Lo L, Singer ST. Thalassemia: current approach to an old disease. Pediatr Clin N Am. 2002;49:1165-91.
7. Hull D, Johnston Dr. Blood. In, Hull D, Johnston Dr, editors. Essential Paediatrics. 3rd ed. Edinburgh: Churchill Livingstone; 1994. p.195-205.
8. Cappelini N, Cohen A, Elefttheriou A, Piga A, Porter J. Endocrine complications in thalassemia major. Guidelines for the clinical management of thalassaemia. Thalassemia International Federation, 2000.
9. Beutler E. Hemochromatosis. In: Beutler E, Hoffbrand AV, Cook JD, editors. Iron deficiency and overload. 1st ed. La Jolla: Hematology Am Soc Hematol Educ Program; 2003. p.40-61.
10. Cunningham Mj, Macklin EA, Neufeld Ej, Cohen AR. Complications of beta-thalassemia major in North America. Blood. 2004;104,34-9.
11. Zervas A, Katopodi A, Protonatoriou A, Livadas S, Karariorga M, Politis C, et al. Assessment of thyroid function in two hundred patients with beta􀁤thalassemia major. Thyroid. 2002;12:151-4.
12. Agarwal MB, Shah S, Vishwanathan C, Rajadhyaksha G, Bhave AA, Dube SR, et al. Thyroid dysfunction in multi-transfused iron􀁤loaded thalassemia patients. Indian Pediatr. 1992;29:997-1002.
13. Italian working group on endocrine complications in non-endocrine diseases. multicentre study on prevalence of endocrine complications in thalassemia major. Clin Endocrinol. 1995;42:581-6.
14. Roth C, Pekrun A, Bartz M, J arry H, Eber S, Lakomek M, et al. Short stature and failure of pubertal development in thalassemia major: evidence for hypothalamic neurosecretory dysfunction of growth hormone secretion and defective pituitary gonadotropin secretion. Eur J Pediatr. 1997;156:777-83.
15. Bharaktiya S, Orlander PR, Woodhouse W R, Davis AB. Hypothyroidism. [cited 03 Aug 2005]. Available from, http://www.emedicine.com.
16. Dallas JS, F ooley TP Jr. Hypothyroidism. In: Lifeshitz F, editor.
Pediatric endocrinology. yd ed. New York: Marcel Dekker; 1996. p. 391-9.
17. Larson EB, Metz R. Disorders of thyroid honnone production. In: Metz R, Larson EB, editors. Blue Book of Endocrinology. 1st ed. Philadelphia: W B Saunders Company; 1985. p. 12-42.
18. Data Pusat Thalasemia. Thalasemia Center, Medical School, Cipto Mangunkusumo Hospital, 2009.
19. Evered Dj, Ormston Bj, Smith PA, Hall R, Bird T. Grades of hypothyroidism. Br Med j. 1973;1:657-62.
20. LaFranchi S. Hypothyroidism. In: Berghman RE, Kleigman RM, Jenson HB, editors. Nelson's textbook of pediatrics. 17th ed. Philadelphia, W B Saunders; 2004. p. 1872-9.
21. Aydinok Y, Darcan S, Polat A, Kavakli N, Nisli G, Coker M, et al. Endocrine complications in patients 'With beta-thalassemia major. J Trop Pediatr. 2002;48:50-5.
22. Grundy RG, Woods KA., Savage MO, Evans JPM. Relationshipof endocrinopathy to iron chelation status in young patients with thalassemia major. Arch Dis Child. 1994;71:128-32.
23. Jain M, Sinha RSK, Ghellani H, Anand NK. Assessment on thyroid functions and its role in body growth in thalassemia major. Indian Pediatr. 1995;32:213-9.
24. Landau H, Matoth I, Landau􀁤Cordova Z, Goldfarb A, Rachmilewitz EA, Glaser B. Cross􀁤sectional and longitudinal study on the pituitary􀁤thyroid axis in patients with thalassemia major. Clin Endocrinol. 1993;38,55-61.
25. Masala A, Meloni T, Gallisai D, Alagna S, Rovasio Pp, Rassu S, et al. Endocrine functioning in multitransfused prepubertal patients 'With homozygous beta􀁤thalassemia. J Clin Endocrinol Metab. 1984;58,667-70.
26. Phenekos C, Karamerou A, Pipis P, Constantoulakis M, Lasaridis J, Detei S, et al. Thyroid function in patients with homozygous beta-thalassemia. Clin Endocrinol. 1984;20:445-50.
27. Sabato AR, De Sanctis V, Atti G, Capra L, Bagni B, Vullo C. Primary hypothyroidism and the low T3 syndrome in thalassaemia major. Arch Dis Child. 1983;58:120-7.
28. Shamsirsaz AA, Beikhernia MR, Kamgar M, Pourzahedgilani N, Bouzari N, Habibzadeh M, et al. Metabolic and endocrinologic complications in beta􀁤thalassemia major: a multicenter study in Tehran. BMC Endocrine Disorders. 2003;3;4-10.
29. Vullo C, De Sanctis V, Katz M, Wonke B, Hoffbrand AV, Bagni B, et al. Endocrine abnonnalities in thalassemia. Ann NY Acad Sci. 1990;612,293-310.
30. Al􀁤Hader AF, Bashir N, Hasan Z, Khatib S. Thyroid function in children 'With beta􀁤thalassemia major in North Jordan. J Trop Ped. 1993;39:107-10.
31. De Sanctis V. Growth and puberty and its management in thalassemia. Horm Res. 2002;58 (suppll);72-9.
32. Galanello R, Origa R. Beta􀁤thalassemia. Orphanet J Rare Dis. 2010;5:1-15.
33. Angelucci E, Brittenham GM, McLaren CE, Ripalti M, Baronciani D, Giardini C, et al. Hepatic iron concentration and total body iron stores in thalassemia major. N EnglJ Med. 2005;343:327-31
Published
2011-04-30
How to Cite
1.
Rindang C, Batubara J, Amalia P, Satari H. Some aspects of thyroid dysfunction in thalassemia major patients with severe iron overload. PI [Internet]. 30Apr.2011 [cited 22Nov.2024];51(2):66-2. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/889
Issue
Vol 51 No 2 (2011): March 2011
Section
Articles

Authors who publish with this journal agree to the following terms:

Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.

Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.

Received 2016-10-13
Accepted 2016-10-13
Published 2011-04-30