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schopné viazať sa na receptor LDL. Dôsledkom toho je zvýšenie koncentrácie cholesterolu v plazme<br />
a predčasná manifestácia atero<strong>sk</strong>lerózy. Vý<strong>sk</strong>yt ochorenia v populácii sa odhaduje na 1:500<br />
(po<strong>do</strong>bne ako familárna hypercholesterolémia).<br />
Klin. sa prejavuje príznakmi po<strong>do</strong>bnými familiárnej hypercholesterolémii: šľachová xantomatóza,<br />
gerontoxon a xanthelasma palpebrae, arcus senilis corneae, predčasný vývoj atero<strong>sk</strong>lerózy a<br />
ischemickej choroby srdca, zvýšené hodnoty cholesterolu v sére, (najniţšie priemerné hodnoty sú<br />
7,0 mmol/l).<br />
Na dg. a dfdg. FDA B-100 slúţia tieto metódy: 1. sle<strong>do</strong>vanie väzby častíc LDL na receptory LDL<br />
kultivovaných ľud<strong>sk</strong>ých fibroblastov; 2. vyuţitie monoklonových protilátok MB 47; 3. metódy<br />
molekulovej biológie.<br />
FDA B-100 opísali a patogenézu objasnili prvýkrát v The Gladstone Foundation Laboratories for<br />
Cardiovascular Disease v San Franciscu. R. 1985 Vega a Grundy opísali pacienta, kt. mal poruchu<br />
klírensu LDL z plazmy.<br />
<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong>-<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><br />
Zjednodušená 3-typová klasifikácia EAS<br />
<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><br />
EAS WHO Zvýšené lipopropteíny<br />
<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong>-<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><br />
Hypercholesterolémia IIA LDL al. HDL<br />
Kombinovaná dyslipoproteinémia IIB, III VLDL + LDL, pri type III remnantné častice<br />
Hypertriacylglycerolémia IV, V VLDL, chylomikróny<br />
<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><br />
Na th. účely v klin. praxi zaviedla Európ<strong>sk</strong>a spoločnosť atero<strong>sk</strong>lerózy r. 1992 zjednodušenú<br />
fenotypovú klasifikáciu d. (→dyslipoproteinémie).<br />
Sek. d. sprevádza rôzne ochorenia, najčastejšie diabetes mellitus, nefrotický sy., hepatopatie,<br />
pankreatopatie a i., príp. ju vyvolávajú lieky (napr. kortikoidy, neselektívne -blokátory a i.).<br />
<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong>-<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><br />
Frekvencia vý<strong>sk</strong>ytu primárnych hyperlipoproteinémií (%)<br />
<strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><strong>–</strong><br />
Typ Beţná populácia Infarkt myokardu<br />
Familiárna hypercholesterolémia 0,2 3 <strong>–</strong> 6<br />
Kombinovaná familiárna hyperlipidémia 0,5 10 <strong>–</strong> 20<br />
Familiárna hypertriacylglycerolémia 0,3 5<br />
Familiárna dysbetalipoproteinémia (typ III) 0,1 1<br />
Hyperlipidémia typ V 0,02 < 1<br />
Sekundárne hyperlipoproteinémie sa vy<strong>sk</strong>ytujú pri týchto stavoch:<br />
Sekundárna hypercholesterolémia: mentálna anorexia, hypotyreóza, nefrotický sy., biliárna<br />
hepato-patia, th. glukokortikoidmi, Cushingov sy., mentálna anorexia, gravidita, porfýria a myelóm.<br />
Sekundárna hypertriacylglycerolémia: diabetes mellitus, urémia, alkoholizmus, obezita, th.<br />
neselek-tívnymi b-blokátormi, systémový lupus erythematosus, dysgamaglobulinémia, glykogenóza<br />
typu I, lipodystrofia<br />
Sekundárna kombinovaná hyperlipidémia: hypertyreóza, nefrotický sy., akromegália<br />
Sekundárna hyperlipoproteinémia typ V: diabetes mellitus typ I, pankreatití<strong>do</strong>u, chron. obličkovej<br />
ne<strong>do</strong>statočnoti a alkoholizme.<br />
Deficit lipoproteínovej lipázy: Bournevilleov sy., Fracoisov sy., Niemannova-Pickova choroba,<br />
Gaucherova choroba, Han<strong>do</strong>va-Schüllerova-Christianova choroba, histiocytóza X, pseu<strong>do</strong>dna. S
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