Complications of Transfusion-Dependent β-Thalassemia Patients in Sistan and Baluchistan, South-East of Iran
AbstractBackground:Thalassemia syndromes are among prevalent hereditary disorders imposing high expenses on health-care system worldwide and in Iran. Organ failure represents a life-threatening challenge in transfusion- dependent β-thalassemia (TDT) patients. The purpose of the present study was to determine the frequency of organ dysfunctions among TDT patients in Sistan and Baluchistan province in South-East of Iran. Materilas and Methods: Laboratory and clinical data were extracted from medical records as well as by interviews. Standard criteria were applied to recognize cardiac, gonadal, endocrine and renal dysfunctions. The collected data were analyzed using the SPSS statistics software (Ver.19). Results: A total of 613 TDT patients (54.3% males and 45.7% females) were included in this study. The mean age of patients was 13.3± 7.7 years old. Cardiac events comprised the most encountered complications (76.4%), following by hypogonadism (46.8%), parathyroid dysfunction (22%), thyroid abnormalities (8.3%), diabetes (7.8%) and renal disease (1.8%). Hypogonadism comprised the most identified complication in patient <15 years old, while the cardiac complications were the most frequent sequela in patients >15 years old (P<0.01). Conclusion: As cardiac events are significantly more common among TDT patients, close monitoring of the heart function is recommended for identifying patients with cardiac problems.
Miri-Moghaddam E, Naderi M, Izadi S, et al. Causes of new cases of major thalassemia in sistan and balouchistan province in South-East of iran. Iran J Public Health. 2012; 41(11):67-71.
Roudbari M, Soltani-Rad M, Roudbari S. The survival analysis of beta thalassemia major patients in South East of Iran. Saudi Med J. 2008; 29(7):1031-5.
Olivieri NF, Brittenham GM. Iron-chelating therapy and the treatment of thalassemia. Blood. 1997; 89(3):739-61.
Patel HV, Qari M, Mousa SA, et al. Iron Balance in β-thalassemia: Maintaining an Antioxidant/Oxidant Ratio. J Appl Hematol. 2012; 3(1):4-11.
Abolghasemi H, Eshghi P, Rahiminejhad S, et al. Evaluation and cost-effectiveness analysis of prevention program of major thalassemia in Sistan-Balouchestan and Fars provinces. Hakim. 2006; 8(4):8-14.
Miri-Moghaddam E,Fadaiy Raieny M,Izadie Sh. Lack of Knowledge of Being Minor Thalasemic is the Main Cause of Major Thalassemia Incidence in Zahedan, the South-East of Iran.Hakim Health Sys Res. 2011; 14(3):174-179.
Miri-Moghaddam E, Zadeh-Vakili A, Nikravesh A, et al. Sistani population: a different spectrum of β-thalassemia mutations from other ethnic groups of Iran. Hemoglobin. 2013; 37(2):138-47.
Tanner J M, RHWhitehouse. Clinical longitudinal standards for height, weight, height velocity, weight velocity, and stages of puberty. Arch Dis Child. 1976;51(3):170-179.
Di Marco V, Capra M, Angelucci E, et al.Management of chronic viral hepatitis in patients with thalassemia: recommendations from an international panel. Blood. 2010; 116(16):2875-83.
Vahidi AA, Parvaresh S, Torabinegad MH, et al. The Frequency of β-thalasemia Major Complications in Patients Referred to Kerman Center for Special Diseases during 6 Months. Journal of Kerman University of Medical Sciences. 2011; 18(4):318-329.
Mohsenzadeh A, Varkuhi A, Shahkarami K, et al. Evaluation of heart disease in patients with beta-thalessemia major referred to Shahid Madanni Hospital in Khorram ABAD in 2003. Yafteh. 2004; 6(1):69-72.
Rahimi Bashar F, Jafroudi M, Zari Doust A. survey of cardiac complications in beta thalassemia major patients in 10-20 years old. The Journal of Guilan University of Medical Sciences. 2008; 16(64):16-23.
Farhangi H, Zandian KH, Pedram M, et al. Evaluation of acquired cardiac complications in major β-thalassemic patients referred to Ahvaz thalassemia center. Jundishapur Scientific Medical Journal. 2010; 8(4): 391-405.
Albu A, Barbu CG, Antonie L, et al. Risk factors associated with hypogonadism in β-thalassemia major patients: predictors for a frequent complication of a rare disease. Postgrad Med. 2014; 126(5):121-7.
Adil A, Sobani ZA, Jabbar A, et al. Endocrine complications in patients of beta thalassemia major in a tertiary care hospital in Pakistan. J Pak Med Assoc. 2012; 62(3):307-10.
Toumba M, Sergis A, Kanaris C, et al. Endocrine complications in patients with Thalassaemia Major. Pediatr Endocrinol Rev. 2007; 5(2):642-8.
Gamberini M, De Sanctis V, Gilli G. Hypogonadism, diabetes mellitus, hypothyroidism, hypoparathyroidism: incidence and prevalence related to iron overload and chelation therapy in patients with thalassaemia major followed from 1980 to 2007 in the Ferrara Centre. Pediatr Endocrinol Rev. 2008; 6 Suppl 1:158-69.
Isik P, Yarali N, Tavil B, et al. Endocrinopathies in Turkish children with Beta thalassemia major: results from a single center study. Pediatr Hematol Oncol. 2014; 31(7):607-15.
Habeb AM, Al-Hawsawi ZM, Morsy MM, et al. Endocrinopathies in beta-thalassemia major. Prevalence, risk factors, and age at diagnosis in Northwest Saudi Arabia. Saudi Med J. 2013; 34(1):67-73.
Borgna‐Pignatti C, Cappellini M, De Stefano P, et al.Survival and complications in thalassemia. Ann N Y Acad Sci. 2005; 1054: 40-7.
Mostafavi H, Afkhamizadeh M, Rezvanfar MR. Endocrine disorders in patients with thalassemia major.
IJEM. 2005; 7(2):143-147.
Smolkin V, Halevy R, Levin C, et al. Renal function in children with β-thalassemia major and thalassemia intermedia. Pediatr Nephrol. 2008; 23(10):1847-51.
Ahmadzadeh A, Jalali A, Assar S, et al. Renal tubular dysfunction in pediatric patients with beta-thalassemia major. Saudi J Kidney Dis Transpl. 2011; 22(3):497-500