Aterosklerosis Prematur dan Dislipidemia pada Anak Penderita Thalassemia Beta Mayor

Abstract

Aterosklerosis adalah penyakit progresif lambat yang ditandai oleh penebalan bertahap tunika intima. Faktor pemicu aterosklerosis prematur adalah hemolisis kronis, peningkatan cadangan kadar zat besi serum, dan dislipidemia. Aterosklerosis prematur adalah salah satu komplikasi kardiovaskular penting pada penderita thalassemia beta mayor. Pada penderita thalassemia beta, kadar kolesterol total, HDL, dan LDL lebih rendah; sebaliknya kadar trigliserida lebih tinggi dibandingkan individu normal. Dislipidemia pada thalassemia beta disebabkan berbagai faktor, antara lain dilusi plasma akibat anemia, meningkatnya aktivitas eritropoeisis, gangguan fungsi hati karena penimbunan zat besi, gangguan hormonal, dan menurunnya aktivitas lipolisis di luar hati.

Atherosclerosis is a slow progressive disease characterized by gradual intimal thickening. The predisposing factors for premature atherosclerosis are chronic hemolysis, increased serum iron deposit, and dyslipidemia. Premature atherosclerosis has emerged as important cardiovascular complication in beta-thalassemia major. Beta-thalassemia is associated with lower total cholesterol, LDL, and HDL, and higher triglyceride level. Dyslipidemia in beta-thalassemia is caused by several factors, such as plasma dilution due to anemia, increased erythropoietic activity, liver injury due to iron overload, hormonal disturbance, and decreased extra hepatic lipolysis activity.