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

被引:12
作者
Madani, Hamid [1 ]
Rahimi, Zohreh [2 ,3 ]
Manavi-Shad, Mohammad [4 ]
Mozafari, Hadi [2 ]
Akramipour, Reza [4 ]
Vaisi-Raygani, Asad [3 ]
Rezaei, Mansour [5 ]
Malek-Khosravi, Shohreh [5 ]
Shakiba, Ebrahim [3 ]
Parsian, Abbas [6 ]
机构
[1] Kermanshah Univ Med Sci, Sch Med, Dept Pathol, Kermanshah, Iran
[2] Kermanshah Univ Med Sci, Sch Med, Med Biol Res Ctr, Kermanshah, Iran
[3] Kermanshah Univ Med Sci, Sch Med, Dept Biochem, Kermanshah, Iran
[4] Kermanshah Univ Med Sci, Sch Med, Dept Pediat, Kermanshah, Iran
[5] Kermanshah Univ Med Sci, Rhazes Ctr Res Family Hlth & Sexual Med, Kermanshah, Iran
[6] NIAAA, Div Neurosci & Behav, NIH, Rockville, MD 20852 USA
关键词
beta-thalassemia; Mutation; Lipid; Lipoprotein; Western Iran; SICKLE-CELL-DISEASE; INTERMEDIA PATIENTS; HYPOCHOLESTEROLEMIA; CHOLESTEROL;
D O I
10.1007/s11033-010-0397-3
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
To determine the plasma lipid and lipoprotein profiles and their possible association with the type of beta-thalassemia mutation we studied 103 major beta-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 beta(0) type of mutation with those homozygous for a beta(+) 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 beta-thalassemia patients and the presence of more severe genotype in patients is correlated with more reduction in TG, TC, and LDL-C levels.
引用
收藏
页码:2573 / 2578
页数:6
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