FREQUENCY OF HYPOTHYROIDISM IN MULTI-TRANSFUSED IRON OVERLOADED β-THALASSEMIA SYNDROME PATIENTS
Abstract
Background: Beta thalassemia syndromes are heterogeneous inherited disorders. They represent the commonest inherited hemoglobinopathies and constitute a com-mon public health problem. Patients suffering from these disorders ultimately devel-op complications including endocrinopathies secondary to transfusional iron over-load. Thyroid dysfunction is well documented in this context; however there is great disparity in its prevalence across the world. This study was designed to determine the frequency of thyroid dysfunction in multi-transfused (> 12 regular transfusions) beta thalassemia syndrome patients in District Peshawar Pakistan.
Methodology: In this cross-sectional descriptive study, patients treated at Fatimid Foundation Peshawar were enrolled. One hundred and fifteen multi-transfused beta thalassemia syndrome patients >10 years of age were included. Data and blood samples were collected after taking written informed consent from patients/guardians. Venous blood (3ml) was collected in vacuette® (Griener, GmbH). After centrifugation, sera were separated and analyzed for T4, T3, TSH and ferritin levels by Chemiluminescence Immune Assay (CLIA) technique using Acculite Monobind Diagnostics on Lumax analyzer in IBMS.
Results: Primary subclinical hypothyroidism was detected in 25 (21.7%) pa-tients. All patients were iron overloaded as indicated by very high serum ferri-tin levels (ranging from 1500-27042ng/ml).
Conclusion: Hypothyroidism occurs mainly in subclinical state and is a fre-quent complication in iron overloaded beta thalassemia syndrome patients with risk of progression.
References
Sway Lay Thein, David Rees. In: Postgraduate Haematolo-gy. Edited by A. Victor Hoffbrand, Daniel Catovsky, Edward G.D. Tuddenham, Anthony R. Green, 6th edn. London: Wi-ley-Blackwell; 2011: 83-108.
Weatherall DJ: Science, medicine, and the future: Single
gene disorders or complex traits: lessons from the thalas-
saemias and other monogenic diseases. BMJ: British Medi-cal Journal. 2000;321(7269):1117.
Mula-Abed W-A, Al Hashmi H, Al Muslahi M, Al Muslahi H, AlLamki M: Prevalence of endocrinopathies in patients with Beta-thalassaemia major-a cross-sectional study in oman. Oman medical journal. 2008;23(4):257.
Modell B, Darlison M: Global epidemiology of haemoglobin disorders and derived service indicators. Bulletin of the World Health Organization. 2008;86(6):480-487.
Galanello R, Origa R: Review: Beta-thalassemia. Orphanet J Rare Dis. 2010;5(11):
Adil A, Sobani ZA, Jabbar A, Adil SN, Awan S: Endocrine complications in patients of beta thalassemia major in a tertiary care hospital in Pakistan. Journal of the Pakistan Medical Association. 2012;62(3):307.
Schrier SL, Angelucci E: New strategies in the treatment of the thalassemias. Annu Rev Med. 2005;56(157-171.
Clara Camaschella, A. Victor Hoffbrand. In: Postgraduate Haematology. Edited by A. Victor Hoffbrand, Daniel Ca-tovsky, Edward G.D. Tuddenham, Green AR, 6th edn. Lon-don: Wiley-Blackwell; 2011: 47-60.
Porter JB: Practical management of iron overload. British journal of haematology. 2001;115(2):239-252.
De Sanctis V: Growth and puberty and its management in thalassaemia. Hormone Research in Paediatrics. 2004;58(Suppl. 1):72-79.
Zervas A, Katopodi A, Protonotariou A, Livadas S, Karagi-orga M, Politis C, Tolis G: Assessment of thyroid function in two hundred patients with β-thalassemia major. Thyroid.
;12(2):151-154.
Cunningham MJ, Macklin EA, Neufeld EJ, Cohen AR:
Complications of β-thalassemia major in North America.
Blood. 2004;104(1):34-39.
Hashemizadeh H, Noori R: Assessment of Hypothyroidism in Children with Beta-Thalassemia Major in North Eastern Iran. IRANIAN JOURNAL OF PEDIATRIC HEMATOLOGY AND ONCOLOGY. 2012;
De Sanctis V, Soliman AT, Elsedfy H, Skordis N, Kattamis C, Angastiniotis M, Karimi M, Yassin MADM, El Awwa A, Stoe-va I: Growth and endocrine disorders in thalassemia: The international network on endocrine complications in thal-assemia (I-CET) position statement and guidelines. Indian journal of endocrinology and metabolism. 2013;17(1):8.
Belhoul KM, Bakir ML, Saned M-S, Kadhim AM, Musallam KM, Taher AT: Serum ferritin levels and endocrinopathy in medically treated patients with β thalassemia major. An-nals of Hematology. 2012;91(7):1107-1114.
Borgna-Pignatti C, Rugolotto S, De Stefano P, Zhao H, Cap-pellini MD, Del Vecchio GC, Romeo MA, Forni GL, Gamber-ini MR, Ghilardi R: Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine. Haematologica. 2004;89(10):1187-1193.
Gulati R, Bhatia V, Agarwal S: Early Onset of Endocrine Ab-normalities in ß-Thalassemia Major in a Developing Coun-try. Journal of pediatric endocrinology and metabolism. 2000;13(6):651-656.
Cario H, Stahnke K, Sander S, Kohne E: Epidemiological situation and treatment of patients with thalassemia ma-jor in Germany: results of the German multicenter β-thal-assemia study. Annals of Hematology. 2000;79(1):7-12.
Najafipour F: Evaluation of endocrine disorders in pa-tients with thalassemia major. International Journal of Endocrinology and Metabolism. 2012;6(2):0-0.
Malik SA, Syed S, Ahmed N: Frequency of hypothyroid-ism in patients of beta-thalassaemia. Pak I Med Assoc. 2010;60(1):17-29.
Merchant RH, Shirodkar A, Ahmed J: Evaluation of growth, puberty and endocrine dysfunctions in relation to iron overload in multi transfused Indian thalassemia patients. The Indian Journal of Pediatrics. 2011;78(6):679-683.
Shamshirsaz AA, Bekheirnia MR, Kamgar M, Pourzahedg-ilani N, Bouzari N, Habibzadeh M, Hashemi R, Sham-shirsaz AA, Aghakhani S, Homayoun H: Metabolic and endocrinologic complications in beta-thalassemia major: a multicenter study in Tehran. BMC Endocrine Disorders. 2003;3(1):4.
Saffari F, Mahyar A, Jalilolgadr S: Endocrine and metabolic disorders in β-thalassemiamajor patients. Caspian Journal of Internal Medicine. 2012;3(3):466.
Baldini M, Marcon A, Cassin R, Ulivieri F, Spinelli D, Cap-pellini M, Graziadei G: Beta-Thalassaemia Intermedia: Evaluation of Endocrine and Bone Complications. BioMed Research International. 2014;2014(
Jaipuria R, Nigam R, Malik R, Shrivastava A, Balani S, Tripathi A: ASSESSMENT OF THYROID FUNCTION IN CHIL-
DREN WITH BETA-THALASSEMIA MAJOR AND ITS CORRELA-TION WITH SERUM FERRITIN AND TRANSFUSION INDEX. Journal of Evolution of Medical and Dental Sciences. 2014;3(4):847-854.
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2017 Maria Kamran
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Readers may “Share-copy and redistribute the material in any medium or format” and “Adapt-remix, transform, and build upon the material”. The readers must give appropriate credit to the source of the material and indicate if changes were made to the material. Readers may not use the material for commercial purpose. The readers may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.