Objective: To evaluate causes of hospitalization (due to complications of iron overload and other causes) in beta-Thalassemic patients. Methodology: This study was performed on 244 patients with major beta-Thalassemia admitted in Taleghani hospital of Gorgan between 2000 and 2007. Causes of hospitalization (due to complications of iron overload and other causes) were evaluated. Data were analyzed with SPSS software. Results: The most common causes of hospitalization due to iron overload were diabetes mellitus (31.6%) and heart failure (16.4%). The most common clinical findings were weakness and fatigue. Conclusion: We perceive increased frequency of diabetes mellitus in this center compared to other studies in Iran and abroad. Therefore glucose tolerance test and genotypic research for IVS II nt 745 are recommended in Thalassemic patient in this area

Jahazi, A.

Mirbehbahani, N.

Rahnama, M.S.

English

Golestan University of Medical Sciences Repository

816   Pak J Med Sci   2011   Vol. 27   No. 4      www.pjms.com.pkOriginal ArticleHospitalization causes due to iron overloadin beta-Thalassemia in Gorgan, IranNarges Mirbehbahani1, Azam Jahazi2, Mohammad Sadegh Rahnama3ABSTRACTObjective: To evaluate causes of hospitalization (due to complications of iron overload andother causes) in beta-Thalassemic patients.Methodology: This study was performed on 244 patients with major beta-Thalassemiaadmitted in Taleghani hospital of Gorgan between 2000 and 2007. Causes of hospitalization(due to complications of iron overload and other causes) were evaluated. Data were analyzedwith SPSS software.Results: The most common causes of hospitalization due to iron overload were diabetesmellitus (31.6%) and heart failure (16.4%). The most common clinical findings were weaknessand fatigue.Conclusion: We perceive increased frequency of diabetes mellitus in this center compared toother studies in Iran and abroad. Therefore glucose tolerance test and genotypic research forIVS II nt 745 are recommended in Thalassemic patient in this area.KEY WORDS: Thalassemia, Cardiac disease, Diabetes mellitus, Iron overload.Pak J Med Sci   July - September 2011   Vol. 27   No. 4    816-818How to cite this article:Mirbehbahani N, Jahazi A, Rahnama MS. Hospitalization causes due to iron overload inbeta-Thalassemia in Gorgan, Iran. Pak J Med Sci 2011;27(4):816-8181. Narges Mirbehbahani,Hematologist, A faculty member of Golestan University of MedicalSciences, Hematology-Oncology Research Center, ThaleghaniHospital, Golestan University of Medical Sciences, Gorgan, Iran.2. Azam Jahazi,Master Science of Midwifery, Faculty Member,Islamic Azad University Gorgan Branch, Gorgan, Iran.3. Mohammad Sadegh Rahnama, Physician,Correspondence:Azam Jahazi,Islamic Azad University Gorgan Branch, Gorgan - Iran.E-Mail: ajahazi@gorganiau.irajahazi@gmail.com  * Received For Publication: February 24, 2011  * Accepted: May 16, 2011INTRODUCTIONBlood transfusion therapy significantly increaseslife expectancy of beta-Thalassemic patients by con-trolling anemia and its squeals. But this treatmentleads to progressive deposition of iron in tissues,posing significant risk of severe cardiac, endocrino-logical, and hepatic complications, which are fatal ifnot prevented.1 Today many of the complications ofThalassemia seen in developed countries are theresult of iron overload. fibrosis and cirrhosis in theliver, diabetes mellitus in the beta cells of the pan-creas, growth retardation and hypogonadotropic hy-pogonadism in the pituitary, testis, and ovaries, hy-pocalcaemia and osteoporosis in the parathyroid, andarrhythmias, myocarditis, and intractable cardiacfailure in the heart.2 Thus, iron overload, whetherthrough increased iron absorption caused by eryth-ropoietic activity or as a result of blood transfusion,constitutes the most important complications andmajor focus of clinical management inbeta-Thalassemia.Most of the pathologic processes develop slowlyand usually are not apparent until the 2nd decade oflife. In untransfused patients, these abnormalities de-velop more slowly. Diabetes mellitus is a frequentand often under recognized complication of Thalas-semia, which is due both to pancreatichypoproduction and (at least in some cases) toinsulin resistance.3Considering that Gorgan is a city located besideCaspian sea and is assumed as one of the prevalentareas of beta Thalassemia, statistical rendering of iron   Pak J Med Sci   2011   Vol. 27   No. 4      www.pjms.com.pk   817Hospitalization causes in beta-Thalassemiaoverload is important information for developingfacilities for patients.METHODOLOGYThe target population of this study was patientswith beta-Thalassemia major admitted to Taleghanihospital of Gorgan (a city located in the north of Iran)between 21 March 2000 and 20 March 2007.Causes of hospitalization due to complications ofiron overload (diabetes mellitus, cardiac disease andhypocalcaemia) and other causes such as respiratorytract infection, gastrointestinal infection, arthritis,cellulites and reaction to transfusion, age and sexwere evaluated. Data were analyzed with SPSS soft-ware (v: 13.0) and compared with other reports ob-tained from developing and developed countries.RESULTSOf our 244 cases, 126 cases (51.6%) were femaleand 118 cases (48.4%) were male. The range of pa-tients, age was between 2.5 months and 25 years.Diabetes mellitus was found in 31.6% of patients (77cases); arrhythmia and heart failure were found in16.4% of patients (40 cases) and hypocalcaemia wasfound in 11.9% of patients (29 cases) (Figure-1). Noneof patients were less than 5 years old had diabetesmellitus and hypocalcaemia and none of patientswere less than 2 years old had cardiac disease(Table-I). The youngest patient (2.5 months old) hadpneumonia and the oldest patient (25 years old) haddiabetes mellitus. Frequency of other causes ofhospitalization is shown in Figure-2 and Table-II.DISCUSSIONThe Thalassemias are common monogenic disor-ders of hemoglobin synthesis. Beta-Thalassemias arethe most important among the Thalassemia syn-dromes and have become a world wide clinical prob-lem due to an increasing immigrant population.Blood transfusion and increased gastrointestinal ironabsorption result in iron overload and tissuedamage.4Patients with Thalassemia major have traditionallysuccumbed to the cardiac complications of iron over-load. Recurrent pericarditis distinguished by char-acteristic pain, fever, and a friction rub may be ini-tial manifestation of myocardial iron deposition andoccasionally requires pericardectomy to relieve con-striction, ventricular tachycardia and fibrillation orsever refractory congestive heart failure.3Cardiac complication is one of the major causes ofdeath in patients with Thalassemia major. Heart rateFig-1: Frequency of hospitalization of beta-Thalassemiawith causes of iron overload.Fig-2: Frequency of hospitalization of beta-Thalassemiawithout causes of iron overload.Table-I: Frequency of hospitalization of beta-Thalassemiawith causes of iron overload in age groups.                  Age (year)    Causes < 2 2-5 5-15 >15of  hospitalizationDiabetes mellitus 0 0 33.8 66.2Heart disease 0 2.5 47.5 50Hypocalcemia 0 0 62.1 37.9Table-II: Frequency of hospitalization ofbeta-Thalassemia without causes of ironoverload in age groups.                 Age (year)Causes < 2 2-5 5-15 >15of  hospitalizationRespiratory 23.1 0 43.6 33.3  tract infectionGI tract infection 9.1 18.2 57.6 15.1Arthritis and 0 0 29.4 70.6  CellulitisReaction to 0 0 71.4 28.6  transfusion818   Pak J Med Sci   2011   Vol. 27   No. 4      www.pjms.com.pkNarges Mirbehbahani et al.variability is a noninvasive index of neuronal modu-lation of heart rate.5 Heart disease represents the maindeterminant of survival in beta-Thalassemia.6Aessopas assessed clinically andechocardiographically 202 Thalassemia major pa-tients aged 27.3±6.3 years and 75 age and sex-matched healthy controls. Overt cardiac disease wasencountered in 14 patients (6.9%).7 There were 211patients included in study of “Durong” in Thailand.Their ages ranged from 2.6 to 18.2 years. Cardiac in-volvement was found in 26 patients (12.3%).8 In ourstudy cardiac involvement was found in 40 patients(16.4%). That is higher than world wide range. Al-though results of our study are similar to Hong Kongstudy.9Cunningham performed a cross-sectional study incancer institute and Harvard medical school on 342patients in the registry of the National Institutes ofHealth-Sponsored Thalassemia clinical research net-work. None of patients were 15 years old or youngerand 5% of patients aged 16-24 years had heartdisease requiring medication.10In our study none of the patients were 2 years oldor younger, 6.2% of patients aged 2-5 years, 16.5% ofpatients aged 5-15 years and 22.2% of patients agedhigher than 15 years had disease requiring medica-tion. These finding may be results of poor diseasecontrol and management in early life in our center.Endocrine disorders commonly associated withThalassemia in the United States today are gener-ally thought to be secondary to the effects of chroniciron loading.3 Fasting blood glucose levels are sig-nificantly elevated even in patients aged 5-10 yearswhose iron burden is 5 to 209. When glucose toler-ance tests are performed as many as 50% of Thalas-semic patients have chemical diabetes defined byglucose tolerance.3Galati prospectively evaluated glucose tolerancein 84 patients over one year. 7.9% of patients haddiabetes or impaired glucose tolerance.11 In HongKong 232 patients were studied at a median age of15.5 years (range 1.4-30.3 years). Cardiomyopathyand diabetes mellitus occurred in 15.1% and8.6% of patients with Thalassemia majorrespectively.9In Egyptian beta-Thalassemic patients the preva-lence of diabetes was 10.4% (5 of 48).12 To identifyprevalence of endocrine dysfunction in Iranian withbeta-Thalassemia, Karami assessed pancreatic func-tion in 150 beta-Thalassemia patients aged 10-22years at the pediatrics unit, Shiraz University ofMedical Sciences. Type 1 diabetes mellitus was foundin 7.3% with age 13.9±2.8 years.13In our study diabetes mellitus was found in 31.6%of patients (77 cases); therefore, we perceive an in-creased frequency of diabetes mellitus in our centercompared to other studies in Iran and abroad. Thehigh frequency of diabetes mellitus in our study maybe a result of poor disease control and management.On the other hand, glucose intolerance generally cor-relates with genetic predisposition and an associa-tion between diabetes and genotyping IVS II nt 745has found. Therefore test of blood glucose andglucose tolerance every 6 months and genotypicalresearch for IVS II nt 745 are recommended inThalassemia patients in our center to detect earlyoccurrence of diabetes.REFERENCES1. Olivery NF, Weatherall DJ. Thalassemias. In: John Lilley man,Jan Hann and victor Blanchette. Pediatric Hematology. 2ndEd. London: Churchill. Livingston. 1999: 307-27.2. Quirolo K, Vichinsky E. Hemoglobin disorders. In: BehrmanRe, Kliegman Rm, Jenson HB. Nelson Textbook of pediat-rics. 17th Ed, Philadelphia, Pennsylvania: WB SaundersCompany: 2004: 1630.3. Orkin SH, Nathan DG. The Thalassemia. In: Nathan andOskies: Hematology of infancy and childhood. 5th Ed.Philadelphia, Pennsylvania: W.B Saunders Company:1998: 858-9.4. Hahalis G, Alexopoulos D, Kremastinos DT, Zoumbos NC.Heart failure in beta-Thalassemia syndromes: A decade ofprogress. Am J Med 2005;118(9):957-967.5. Gurses D, Ulger Z, Levent E, Ayodinok Y, Ozyurek AR, Timedomain heart rate variability analysis in patients with Thalas-semia major. Acta Cardiol 2005;60(5):477-481.6. Aessopas A, Farmakis D, Deftereos S, Tsironi M,Tassiopoulos S, Moyssakis I, et al. Thalassemia heart dis-ease: A comparative evaluation of Thalassemia major andThalassemia intermedia. Chest 2005;127(5):1523-1530.7. Aessopas A, Farmakis D, Hatziliami A, Fragodimitri C,Karabatsos F, Joussef J, et al.  Cardiac status in well-treatedpatients with Thalassemia major. Eur J Heamatol2004;73(5):359-366.8. Durongpisitkul K, Kruasukon S, Kangkagate C, TanphaichitrVS. Early detection of cardiac involvement inbeta- Thalassemia children. J Med Assoc Thai 2002;85(Supple2):5667-73.9. Li CK, Luk CW, Ling SC, Chik KW, Yuen HL, Li CK, et al.Morbidity and mortality Patterns of Thalassemia major pa-tients in Hong Kong: Retrospective study. Hong Kong MedJ 2002;8(4):255-260.10. Cunningham Mj, Macklin EA, Neufeld EJ, Cohen AR. Com-plications of beta-Thalassemia major in North America.Blood 2004;104(1):34-39.11. Gulati R, Bhatia V, Agarwal SS. Early onset of endocrineabnormalities in beta-Thalassemia major in a developingcountry. J Pediatr Endocrine Metab 2000;13(6):651-656.12. Khalifa AS, Salem M, Mounir E, EI-Tawil MM, EI-Sawy M,Al-Aziz MM. Abnormal glucose tolerance in Egyptian beta-Thalassemic patients. Possible Association with GenotypingPediatr Diabetes 2004;5(3):126-132.13. Karamifar H, Shahriari M, Sadjadian N. Prevalence of endo-crine complications in beta-Thalassemia major in the IslamicRepublic of Iran. East Mediterr Health J 2003;9(1-2):55-60.

Hospitalization causes due to iron overload in beta-Thalassemia in Gorgan, Iran

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Hospitalization causes due to iron overload in beta-Thalassemia in Gorgan, Iran

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