Relationship of decreased hepatic lipase activity and lipoprotein abnormalities to essential fatty acid deficiency in cystic fibrosis patients.
Polyunsaturated fatty acids are known to affect plasma lipids and lipoproteins but there is no information on the effect of essential fatty acid (EFA) deficiency on lipoprotein composition. The purpose of this study was to characterize lipoproteins from 17 cystic fibrosis (CF) patients in relationship to their EFA status (eicosatrienoic/arachidonic acid ratio) and compare them with those of 10 healthy siblings (SIB) and of 10 unrelated controls. In 7 EFA-deficient (EFAD) and 10 EFA-sufficient (EFAS) patients, hypocholesterolemia was associated with a decrease of HDL-cholesterol and of LDL-cholesterol which was more marked in the EFAD group. Similarly, although triglyceride enrichment of VLDL, LDL, HDL2, and HDL3 with a concomitant reduction of cholesteryl esters from all particles except HDL2 was observed in both CF groups, it was more sizable in the EFAD patients. These changes led to an increase in the particle size of VLDL, LDL, and HDL2 whereas the distribution of HDL3 was skewed to smaller particles. Alterations in the apoprotein composition of particles were greater in EFAD than in EFAS. A decrease of total postheparin lipolytic activity was observed in the two groups of CF patients as well as in siblings. It was entirely accounted for by hepatic lipase (mumol FFA/ml per h) which was more severely diminished in EFAD (2.8 +/- 0.6) than in EFAS (4.4 +/- 0.7) and SIB (5.1 +/- 0.5). Although the two groups of CF children differed in terms of growth, severity of malabsorption, and vitamin E status, these data suggest that disturbance of lipoprotein concentration, composition, size, and metabolism (hepatic lipase) may be in part related to EFA deficiency. Further studies are necessary to explore the effect of EFA deficiency on hepatic lipase activity.
Centre de Recherche Pédiatrique, Hôpital Ste-Justine, Montréal, Québec, Canada.
E
E
Lévy
G
G
Lepage
M
M
Bendayan
N
N
Ronco
L
L
Thibault
N
N
Galéano
L
L
Smith
C C
CC
Roy
D000293:Adolescent; D001053:Apolipoproteins / Q000032:analysis; D002648:Child; D003550:Cystic Fibrosis / Q000201:enzymology* / Q000378:metabolism; D004591:Electrophoresis, Polyacrylamide Gel; D005228:Fatty Acids, Essential / Q000172:deficiency*; D005260:Female; D006801:Humans; D008055:Lipids / Q000032:analysis; D008066:Lipolysis; D008071:Lipoprotein Lipase / Q000097:blood*; D008074:Lipoproteins / Q000097:blood* / Q000648:ultrastructure; D008099:Liver / Q000032:analysis / Q000201:enzymology*; D008111:Liver Function Tests; D008297:Male
D001053:Apolipoproteins; D005228:Fatty Acids, Essential; D008055:Lipids; D008074:Lipoproteins; D008071:Lipoprotein Lipase