Cholelithiasis in Thalassemia Major Patients: A Report from the South-East of Iran
Background: Cholelithiasis and its predisposing factors are less characterized in thalassemia syndromes. In the present study, we assessed the prevalence of gallstones and related-risk factors among thalassemia major (TM) patients in south-east of Iran.
Materials and Methods: The patients were recruited form a single center in Zabol city, south-east of Iran. Demographic and clinical information were retrieved from medical histories. Abdominal ultrasonography was performed to scrutinize gallstones and organ dimensions of liver, spleen, gallbladder and kidney.
Results: The study participants (n=127) consisted of 50 (39.4%) males and 77 (60.6%) females. The mean age of the patients was 15.2±7.9 years. Cholelithiasis was observed in 11 (8.7%) patients. Cholelithiasis was significantly associated with age (P=0.002) and splenectomy (P=0.001). The patients with cholelithiasis received a significantly higher blood volume than patients without cholelithiasis (546±108.7 ml and 425.1±134.7 ml, respectively, P=0.007). There were significant differences between cholelithiasis and non- cholelithiasis TM patients regarding the length of right and left liver lobes (P=0.001), as well as the length of gallbladder (P=0.006). Ferritin level was not associated with cholelithiasis in our patients. In multivariate analysis, age older than 15 (OR=10.4, 95% CI: 1.2-86.3, P=0.02) and 30 years old (OR=42.6, 95% CI: 2.9-613, P=0.006), and splenectomy (OR=8.7, 95% CI: 2.1-35.4, P=0.002) were significant risk factors for cholelithiasis.
Conclusion: Cholelithiasis is a relatively common complication among TM patients in our region. The most prominent risk factors of cholelithiasis were advanced age, splenectomy and large-volume blood transfusion.
Bazi A, Miri-Moghaddam E. Spectrum of beta-thalassemia Mutations in Iran, an Update. Iran J Ped Hematol Oncol. 2016;6(3):190-202.
Miri-Moghaddam E, Bahrami S, Naderi M, et al. Molecular Characterization of β-Thalassemia Intermedia in Southeast Iran. Hemoglobin. 2016;40(3):173-8.
Shahramian I, Razzaghian M, Ramazani AA, et al. The correlation between troponin and ferritin serum levels in the patients with major beta-thalassemia. Int Cardiovasc Res J. 2013;7(2):51-5.
Bazi A, Keramati MR, Shahramian I. Cardiac Hemosiderosis in Transfusion Dependent Thalassemia: A Mini-Review. Int J Basic Sci Med. 2017; 2(1):5-10.
Winichakoon P, Tantiworawit A, Rattanathammethee T, et al. Prevalence and Risk Factors for Complications in Patients with Nontransfusion Dependent Alpha- and Beta-Thalassemia. Anemia. 2015; 2015(1):1-7.
Dehghani SM, Efazati N, Shahramian I, et al. Evaluation of cholestasis in Iranian infants less than three months of age. Gastroenterol Hepatol Bed Bench. 2015;8(1):42-8.
Gumiero AP, Bellomo-Brandao MA, Costa-Pinto EA. Gallstones in children with sickle cell disease followed up at a Brazilian hematology center. Arq Gastroenterol. 2008;45(4):313-8.
Au WY, Cheung WC, Hu WH, et al. Hyperbilirubinemia and cholelithiasis in Chinese patients with hemoglobin H disease. Ann Hematol. 2005; 84(10):671-4.
Lotfi M, Keramati P, Assdsangabi R, et al. Ultrasonographic assessment of the prevalence of cholelithiasis and biliary sludge in beta-thalassemia patients in Iran. Med Sci Monit. 2009; 15(8):CR398-402.
Galanello R, Piras S, Barella S, et al. Cholelithiasis and Gilbert's syndrome in homozygous β‐thalassaemia. Br J Haematol. 2001;115(4):926-8.
Sherlock S, Dooley J. Diseases of the liver and biliary system. Gut. 2003; 52(4): 615.
Mishra AK, Tiwari A. Iron overload in beta thalassaemia major and intermedia patients. Maedica (Buchar). 2013;8(4):328-32.
Khavari M, Hamidi A, Haghpanah S, et al. Frequency of Cholelithiasis in Patients With Beta-Thalassemia Intermedia With and Without Hydroxyurea. Iran Red Crescent Med J. 2014; 16(7): e18712.
Karami H, Vahidshahi K, Kosarian M, et al. Relationship between Use of Hydroxyurea and Cholelithiasis in Patients with Major and Intermediate β-thalassemia. J Mazandaran Univ Med Sci. 2007; 17(61):62-68.
Bazi A, Miri-Moghaddam E, Moudi Z. The High Birth Rate of Thalassemia Major and the Associated Problems in Sistan and Baluchistan Province, Iran. Gene, Cell and Tissue. 2016; 3(4): e38670.
Shahramian I, Akhlaghi E, Ramezani A, et al. A study of leptin serum concentrations in patients with major beta-thalassemia. Iran J Ped Hematol Oncol. 2013; 3(2):59-63.
Rosenberg H, Markowitz R, Kolberg H, et al. Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol. 1991; 157(1):119-21.
Borgna-Pignatti C, Rigon F, Merlo L, et al. Thalassemia minor, the Gilbert mutation, and the risk of gallstones. Haematologica. 2003; 88(10):1106-9.
Rafsanjani KA, Mafi N, Tafreshi RI. Complications of beta-thalassemia intermedia in Iran during 1996-2010 (single-center study). Pediatr Hematol Oncol. 2011;28(6):497-508.
Karami H, Kianifar HR, Karami S. Cholelithiasis in Children: A Diagnostic and Therapeutic Approach. J Pediat Rev. 2017; 5(1): e9114.
Origa R, Galanello R, Perseu L, et al. Cholelithiasis in thalassemia major. Eur J Haematol. 2009;82(1):22-5.
Portincasa P, Moschetta A, Berardino M, et al. Impaired gallbladder motility and delayed orocecal transit contribute to pigment gallstone and biliary sludge formation in beta-thalassemia major adults. World J Gastroenterol. 2004;10(16):2383-90.
Papadaki MG, Kattamis AC, Papadaki IG, et al. Abdominal ultrasonographic findings in patients with sickle-cell anaemia and thalassaemia intermedia. Pediatr Radiol. 2003; 33(8):515-21.
Nasr MR, Shaker M, Mahdy H, et al. Gall bladder contractility in children with beta-thalassaemia. East Mediterr Health J. 2009; 15(2):315-21.
el-Nawawy A, Kassem AS, Eissa M, et al. Gall bladder sludge and stones in multitransfused Egyptian thalassaemic patients. East Mediterr Health J. 2001; 7(4-5):635-41.
Tankanitlert J, Morales NP, Fucharoen P, et al. Association between promoter and coding region mutations of UDP-glucuronosyltransferase 1A1 and beta-thalassemia/Hb E with cholelithiasis. Eur J Haematol. 2008;80(4):351-5.
AlFadhli S, Al-Jafer H, Hadi M, et al. The effect of UGT1A1 promoter polymorphism in the development of hyperbilirubinemia and cholelithiasis in hemoglobinopathy patients. PLoS One. 2013; 8(10):e77681
Au WY, Cheung WC, Chan GC, et al. Risk factors for hyperbilirubinemia and gallstones in Chinese patients with b thalassemia syndrome. Haematologica. 2003;88(2):220-2.
Panpimanmas S, Manmee C. Risk factors for gallstone disease in a Thai population. J Epidemiol. 2009; 19(3):116-21
Novacek G. Gender and gallstone disease. Wien Med Wochenschr. 2006; 156(19-20):527-33.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.