The clinical and biochemical characteristics of type III hyperlipoproteinemia are described in 64 patients (35 males and 29 females). Homozygosity for apolipoprotein E2, the presence of an abnormally cholesterol-rich very low density lipoprotein fraction (beta-VLDL) and an elevated ratio of very low density lipoprotein cholesterol to plasma triglycerides (> 0.3; normal ratio about 0.2) were the basis for the diagnosis. Mean serum cholesterol and triglyceride concentrations at the first visit in the clinic were 426 +/- 221 and 719 +/- 996 mg/dl, respectively. The mean age at diagnosis of the disorder was 49 years in males and 53 years in females. There was a high prevalence of obesity (72%), xanthomas (42%), and atherosclerosis (39%), especially peripheral vascular disease (31%). Early and correct diagnosis of this familial lipoprotein disorder seems necessary because of the prompt and beneficial response to therapeutic interventions.
The familial lipoprotein disorder type III hyperlipoproteinemia (HPL) carries a marked increase in the risk of accelerated and premature atherosclerosis, but there is considerable variation among affected individuals in susceptibility to cardiovascular disease (CVD). We studied the influence of independent risk factors for atherosclerosis in 67 patients with clinically overt type III HPL and homozygosity for apolipoprotein (apo) E2. Among the different risk factors (lipid and lipoprotein levels, age, sex, body mass index, smoking status, hypertension, and diabetes mellitus) there was only a statistically significant difference in age between 25 patients with atherosclerosis and 42 patients without atherosclerosis. Serum lipoprotein (a), [Lp, (a)], levels were 30.6% higher in the atherosclerosis group, but this was not statistically significant. We conclude that (in contrast to familial hypercholesterolemia) elevated Lp (a) concentrations may not be regarded as a component of the clinical syndrome of type III HPL.
Da die Erkrankung sehr selten ist und in frühzeitiger Operation dic einzige Möglichkeit besteht, den Kranken zu retten, erschêint die Mitteilung einer weiteren derartigen Beobachtung berechtigt. W. 1-1., l4jähriger Knabe Er leistete im Toribruch schwerste Arbeit und zog sich einen subkutanen Abszeß an de rechten Hand zu, der am 10. VII. inzidiert wurde, ohne daß danach die erhöhte. Temperatur abfiel. Es stellten sich allmählich Mattigkeit und zeitweise Benommenheit. urd Erbrechen ein. Temperatur abends vom 10.-17. VII. bis 39°. Wegen Verdachtes auf eine akute Erkrankung des Zentralnervensystems wurde ich von dem behandelnden Arzt zugezogen. Zur Lumbalpunktion sollte ich das erforderliche Instrumentarium mitnehmen.
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