Lipoprotein (a) hyperlipoproteinemiaE78.4

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

lipoprotein(a) hyperlipipoproteinemia; lipoprotein(a) hyperlipoproteinemia; Lp(a) increase; Remnant hyperlipidemia

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DefinitionThis section has been translated automatically.

Very rare, autosomal recessive inherited lipid metabolism disorder. An abnormal apolipoprotein E leads to incomplete degradation of chylomicron remnants and IDL (intermediate dense lipoproteins), so that their plasma concentrations increase. The consequences are a massively increased risk of arteriosclerosis.

EtiopathogenesisThis section has been translated automatically.

In addition to LDL, Lp(a) contains ApoB-100, a plasminogen-like Apo(a), which competes with the plasminogen for the binding sites on the exothelial cells (antiplasminogenic effect). High Lp(a) may inhibit local fibrinolysis in the endothelial region of the vessels. Lp(a) concentrations >30mg/dl are considered an independent atherosclerosis risk factor.

Clinical featuresThis section has been translated automatically.

Xanthelasma, tuberous xanthomas and tendinous xanthomas, eruptive xanthomas, yellow hand lines due to lipoid deposition(Xanthoma palmare striatum). Coronary sclerosis, peripheral, arterial circulatory disturbance.

LaboratoryThis section has been translated automatically.

The serum is milky cloudy or clear, cholesterol and triglycerides are elevated. Lowering of the glucose tolerance threshold.

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer