Hyperlipidemia due to hepatic triacylglycerol lipase deficiency

Orpha code: 140905OMIM code: 614025

Definition

A rare hyperalphalipoproteinemia characterized by elevated plasma cholesterol and triglyceride (TG) levels with a marked TG enrichment of low- and high-density lipoproteins (HDL), presence of circulating beta-very low density lipoproteins and elevated HDL cholesterol levels, in the presence of a very low, or undetectable, postheparin plasma hepatic lipase activity. Premature atherosclerosis and/or coronary heart disease may be associated.

Disease data
Classification

Disease

Synonyms
Hyperlipidemia due to HL deficiency
Hyperlipidemia due to HTGL deficiency
Hyperlipidemia due to hepatic lipase deficiency
Hyperlipidemia due to hepatic triglyceride lipase deficiency
ORPHA code
140905
OMIM code
614025
ICD10 code
E78.4
ICD11 code
-

No additional description.

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