Familial combined hyperlipidemia. Part II. Clinical picture
Vaverková, H.; Weinbergová, O.; Horcicka, V.; Kubasta, M.; Vrublovský, P.
Acta Universitatis Palackianae Olomucensis Facultatis Medicae 114: 243-259
1986
ISSN/ISBN: 0301-2514 PMID: 2946184 Document Number: 271317
An analysis of the clinical picture of members of 32 families who fulfilled the criteria of familial combined hyperlipidemia was carried out. There were studied 32 propositi of these families (mean age 50.9 .+-. 8.3 years; 27 men and 5 women), 73 of their living siblings (31 men and 42 women, mean age 52.8 .+-. 8.6 yrs.) and 58 children of propositi whose age was more than 15 (28 men and 30 women, mean age 26.2 .+-. 6.2 yrs.). In these families, the most frequent clinical picture was an ischemic heart disease which we found in the generation of the propositi (propositi and their siblings) in 50% of hyperlipidemic persons. It appeared significantly more frequently in males (60.8%) than in females (37.5%), the mean clinical manifestation occurred, however, in both sexes at the same age - 50 years. The cumulative probability of the occurrence of the clinical manifestation of IHD in hyperlipidemic (HL) and normolipidemic members (NL) of the families in view of the individual decades was as follows: (1) men: 0-29 years: HL - 0%/NL - 0%, 30-39 yrs.: HL - 1.9%NL -0%, 40-49 yrs.: HL - 27.3%/NL - 0%, 50-59 yrs.: HL - 74.9%/NL - 12.5%, 60-69 yrs.: HL -87.4%/NL - 37.5%; (2) women: 0-29 years: HL - 0%/NL - 0%, 30-39 yrs.: HL - 9.6%/NL - 0%, 40-49 yrs.: HL - 21.7%/NL - 0%, 50-59 yrs.: HL - 42.8%/NL - 0%, 60-69 yrs.: HL -71.4%/NL - 0%. IHD occurred with the same frequency in all the lipoprotein types (IIa, IIb, IV and V), in patients with hypertriglyceridemia even some years earlier than in persons with isolated hyperbetalipoproteinemia. The hypertriglyceridemic members of these families of more than 30 years of age (with the lipoprotein pictures IIb, IV and V) showed a significantly more frequent occurrence of other risk factors of atherosclerosis, namely arterial hypertension, overweight and diabetes mellitus of type II than patients with isolated hyperbetalipoproteinemia with type IIa who in this respect did not differ significantly from the normolipidemic relatives. In hypertriglyceridemic members of families there was also a more frequent occurrence of hyperuricemia, clinically manifest gout and cholecystolithiasis than in type IIa. During the examination of these families, hyperlipoproteinemia was found present in half of the children of the propositi who were of a relatively young age at which it can be assumed that by an early introduction of primary multifactorial prevention of IHD a retardation of the process of atherogenesis can be achieved. Consequently, the screening of all the members of families of propositi with primary hyperlipoproteinemia should become a necessary requirement.