Plasma lipids and lipoproteins in children and young adults with major β-thalassemia from western Iran: influence of genotype

• DOI: 10.1007/s11033-010-0397-3
• Publication Date: 2010-11-17T07:10:40Z
• Authors: Hamid Madani; Zohreh Rahimi; Mohammad Manavi-Shad; Hadi Mozafari; Reza Akramipour; Asad Vaisi-Raygani; Mansour Rezaei; Shohreh Malek-Kho...; Ebrahim Shakiba; Abbas Parsian
• Keywords: 0303 health sciences; Adolescent; Lipoproteins; beta-Thalassemia; Iran; Polymerase Chain Reaction; Statistics, Nonparametric; 3. Good health; Young Adult; 03 medical and health sciences; Cholesterol; Mutation; Humans; Child; Polymorphism, Restriction Fragment Length; Triglycerides

To determine the plasma lipid and lipoprotein profiles and their possible association with the type of β-thalassemia mutation we studied 103 major β-thalassemia patients including 71 children and 32 young adults compared to 102 healthy subjects consisted of 90 children and 12 young healthy adults. The plasma lipid and lipoprotein levels were measured by conventional methods. Considering all of the patients the levels of total cholesterol (TC), LDL-cholesterol (LDL-C), and HDL-cholesterol (HDL-C) were significantly lower compared to controls. However, the level of TG was significantly higher in cases than controls. Comparing thalassemic patients homozygous for a β0 type of mutation with those homozygous for a β+ type of mutation (IVSI.110 G:A) indicated that the levels of LDL-C, TC were significantly increased and TG concentration tended to be higher in the latter patients. In conclusion, our study indicates that hemolytic stress results in hypocholesterolemia in major β-thalassemia patients and the presence of more severe genotype in patients is correlated with more reduction in TG, TC, and LDL-C levels.