Resumen

JIMENEZ MONTERO, José G; BOLANOS ARRIETA, Mario  y  VARGAS PICADO, Marco A. Características clínicas y perfil de lípidos, lipoproteínas y lipoproteína (a) en pacientes con dislipoproteinemias primarias. Rev. costarric. cardiol [online]. 2000, vol.2, n.2, pp.15-23. ISSN 1409-4142.

Dyslipoproteinemias are associated with a number of medical complications such as cardiovascular diseases and acute pancreatitis In patients with cardiovascular disease atherogenic lipoproteins LDL, VLDL, Lp (a) are usually elevated and cholesterol HDL reduced. This study presents pertinent clinical characteristics, lipid profile and the concentration of lipoprotein (a) in patients with primary dyslipoproteinemias and in normo lipidemic relatives. Twenty patients with familial heterozygous hypercholesterolemia (Males 6, mean age 46 years; females 14, mean age 50 years); 18 patients with familial combined hyperlipidemia (males 11, mean age 35 years; females 7, mean age 49 years) and 9 patients with type III dyslipidemia (males 7, mean age 28 years; females 2, 60 and 30 years) were studied. Eleven patients had tendinous xanthomas (6 with familiar hypercholesterolemia and 4 with type III dyslipidemia), four patients had premature coronary heart disease. Cholesterol, triglyceride, and LDL cholesterol levels were elevated in all patients; the cholesterol/HDL ratio was higher in patients with familiar hypercholesterolemia and with type III dyslipidemia than those with familial combined hyperlipidemia. Mean plasma lipoproteín (a) was similar in all the study groups. It was found that aproximately 25 per cent of the hyperlipidemic patients had Lp (a) concentrations higher than 20 mg/dl and were also similar in normo and hyperlipidemic subjects. This study showed an atherogenic lipid profile in familial hypercholesterolemic and type III dyslipidemic patients. Lp (a) concentration seems to be independent of the functional status of the LDL receptor due to the fact that it's concentrations were similar in all types for dyslipoproteinemias.

Palabras clave : Lipid profile; lipoproteins; lipoprotein (a); primary dislipoproteinemias.