Chylomicronemic syndrome E78.3
Synonym(s)
Bürger-Grütz Syndrome; Familial hyperchylomicronemia; Fat-induced hypertriglyceridemia; Hyperchylomicronemia familial; hyperlipemic idiopathic; Hyperlipoproteinemia type I; Hypertriglyceridemia Fat induced; Idiopathic hyperlipemic xanthomatosis; Idiopathic hyperlipidemic xanthomatosis; Xanthomatosis idiopathic hyperlipidemic
Definition
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Familial hyperchylomicronemia with development of multiple eruptive xanthomas, retinal lipaemia, recurrent upper abdominal colics.
Etiopathogenesis
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Autosomal recessive mutations of the lipoprotein lipase gene (LIPD gene; gene locus 8p22) with consecutive partial deficiency of lipoprotein lipase.
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Manifestation
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About 10 years old.
Localization
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Mainly glutaeal region, thighs, back, chest, arms, face.
Laboratory
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Increase of triglycerides and chylomicrons in serum.
Therapy
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Low fat, low cholesterol and high fibre diet; cooperation with the internist.
Progression/forecast
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Favourable if the appropriate diet is followed, otherwise cardiovascular complications.
Recommended articles
Incoming links (5)
Bürger-grütz syndrome; Hyperlipoproteinemia type i; Hypertriglyceridemia, fat induced; Xanthomatosis, idiopathic hyperlipidemic; Xanthome eruptive;Outgoing links (1)
Xanthome eruptive;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
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Prof. Dr. med. Peter Altmeyer