Study of pulmonary function tests in multitransfused children with beta thalassemia major: an observational study
Keywords:Beta thalassemia major, Hemoglobinopathy, Pulmonary function tests
Background: Thalassemia is a hemoglobinopathy requiring multiple blood transfusions leading to iron deposition in various organs. Abnormalities in pulmonary function in thalassemia major patients have been found in various studies. Our aim was to study pulmonary function tests (PFT) in multitransfused children with beta thalassemia major.
Methods: PFT was done in forty beta thalassemia major (TM) patients and forty normal healthy age and sex matched individuals and the results were analyzed.
Results: Out of 40 thalassemia major patients, 20 (50%) had normal PFT and the rest 20 (50%) had an abnormal PFT out of which 18 (90%) had restrictive PFT and 2 (10%) had obstructive PFT. Out of 40 controls, all 40 (100%) had normal PFT.
Conclusions: Alteration of pulmonary function can be seen even in well-chelated TM patients. The respiratory system should be evaluated annually by PFTs even in asymptomatic patients to prevent pulmonary sequelae.
Higgs DR, Engel JD, Stamatoyannopoulos G. Thalassemia. Lancet. 2012;379:373-82.
AK Jain. Textbook of Physiology, 6th edition, vol 1-2, Avichal Publishing Company. 2016.
Kliegman RM, Behrman RE, Bonita HJ, Stanton F. Nelson. Textbook of Pediatrics, 20th edition, Saunders. 2007;462;10:2349.
Taher A, Vichinsky E, Musallam K, Cappellini MD, Viprakasit V, Weatherall D. Guidelines for the Management of Non-Transfusion Dependent Thalassaemia (NTDT). Nicosia, Cyprus, 2013, Thalassaemia Internation Federation.
Vichinsky E, Levine L. Standard of care guidelines for thalassemia, CA, Children’s Hospital and research center Oakland. 2012;1-28.
Barrett KE, Barman SM, Boitano S, Brooks HL. Ganong’s Review of Medical Physiology, 25th edition, McGraw-Hill Education/Medical. 2015;768.
Guidelines for Pulmonary Function Tests. American Academy of Paediatrics. 2015.
Alyasin S, Moghtaderi M, Amin R, Kashef S, Karimi M. Pulmonary function test in transfusion-dependent beta-thalassemia major patients: a pilot study. Pediatr Hematol Oncol. 2011;28:329-33.
Cooper DM, Mansell AL, Weiner MA, Berdon WE, Chetty-Baktaviziam A, Reid L. Low lung capacity and hypoxemia in children with thalassemia major. Am Rev Respir Dis. 1980;121(4):639-46.
Boddu A, Kumble A, Mahalingam S, Baliga BS, Achappa B. Pulmonary dysfunction in children with beta thalassemia major in relation with iron overload-a cross sectional hospital-based study. Asian J Med Sci. 2015;6(5):47-50.
Bourli E, Dimitriadou M, Economou M, Vlachaki E, Christoforidis A, Maratou E et al. Restrictive pulmonary dysfunction and its predictors in young patients with β-thalassaemia major. Pediatric Pulmonol. 2012;47(8):801-7.
Azarkeivan A, Mehrvar A, Pour HS, Mehrvar N, Vosough P. Pulmonary function test in transfusion-dependent beta-thalassemia patients. Pediatr Hematol Oncol. 2008;25(6):598-606.
Kanj N, Shamseddine A, Gharzeddine W, Kanj M, Abi Nasr T, Koussa S et al. Relation of ferritin levels to pulmonary function in patients with thalassemia major and the acute effects of transfusion. Eur J Haematol. 2000;64(6):396-400.
Eidani I, Keikhaei B, Rahim F, Bagheri A. Evaluation of pulmonary function in thalassemia major patients. Pak J Med Sci. 2009;25:749-54.
Factor JM, Pottipati SR, Rappoport I, Rosner IK, Lesser ML, Giardina PJ. Pulmonary function abnormalities in thalassemia major and the role of iron overload. Am J Respir Crit Care Med. 1994;149(6):1570-4.
Abu-Ekteish FM, Al-Rimawi HS, Al-Ali MK, Shehabi IM. Pulmonary function tests in children with beta-thalassemia major. Chron Respir Dis. 2007;4:19-22.
Parakh A, Dubey AP, Chowdhury V, Sethi GR, Jain S, Hira HS. Study of pulmonary function tests in thalassemic children. J Pediatr Hematol Oncol. 2007;29(3):151-5.
Li AM, Chan D, Li CK, Wong E, Chan YL, Fok TF. Respiratory function in patients with thalassaemia major: relation with iron overload. Arch Dis Child. 2002;87:328-30.
Jamal R, Baizura J, Hamidah A, Idris N, Jeffrey AH, Roslan H. Abnormalities in lung function among multiply-transfused thalassemia patients: results from a thalassemia center in Malaysia. Southeast Asian J Trop Med Public Health. 2005;36(1):265-9.
Tai DY, Wang YT, Lou J, Wang WY, Mak KH, Cheng HK. Lungs in thalassaemia major patients receiving regular transfusion. Eur Respir J. 1996;9(7):1389-94.
Ozyoruk D, Misirlioglu ED. Pulmonary Functions in Children with Thalassemia Major. J Pediatr Hematol Oncol. 2015;37(8):605-10.