Lipid metabolism disordersE78.9

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 26.12.2021

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Synonym(s)

Cholesterol Lowering; Dyslipidemia; Dyslipoproteinemias; Hyperchoelsterinemias; Hyperlipidemia; Hyperlipoproteinaemia; Hypocholesterolemia; Lipometabolic diseases; Lipometabolic disorders

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DefinitionThis section has been translated automatically.

Hyperlipoproteinaemia (HLP) or hyperlipidaemia is generally understood to be an increased concentration of cholesterol, triglycerides, lipoproteins with a shift in the relative proportion of the LDL or VLDL fraction in the blood. The lipoproteins of plasma consist of lipids (triglycerides, cholesterol, cholesterol esters and phospholipids) and apolipoproteins. Lipids are substances of plasma and cells that are soluble in hydrophobic organic solvents

According to Fredrickson 's classification, congenital lipometabolic disorders are divided into five classes that differ in clinical, laboratory and prognosis. It should be noted that the Fredrickson classification does not take into account lipoprotein a and does not consider the genetic causes underlying the hyperlipoproteinaemias.

The lipoproteins are classified according to density classes and functions as follows:

  • Chylomicrons: 3% cholesterol/90% triglycerides. Function: exogenous glycerides from the intestine to extrahepatic tissues and the liver
  • VDL (very low density lipoproteins): 15% cholesterol/65% triglycerides. Function: endogenous glycerides from the liver to extrahepatic tissues
  • LDL VDL (low density lipoproteins): 45% cholesterol/10% triglycerides. Function:Choelsterol from the liver to extrahepatic tissues
  • HDL (low density lipoproteins): 20% cholesterol/5% triglycerides. Function: Cholesterol from extrahepatic tissues.

ClassificationThis section has been translated automatically.

A distinction is made between primary and secondary hyperlipoproteinaemias. Primary hyperlipoproteinaemia is a disease of its own, usually of genetic origin, whereas secondary hyperlipoproteinaemia is a consequence of other underlying diseases. Most patients with hyperlipoproteinaemia show a combination of genetic predisposition and external factors (lifestyle, diseases or medication).

E78 Disorders of lipoprotein metabolism and other lipidaemias (classification according to ICD)

  • E78.0 Hereditary (familial) hypercholesterolemia
  • E78.1 Pure hypertriglyceridemia (familial hypertriglyceridemia)
    • E78.1 Endogenous hypertriglyceridemia
    • E78.1 Hyperlipidemia, Group B
    • E78.1 Hyperlipoproteinemia type IV according to Fredrickson
    • E78.1 Hyperlipoproteinaemia of the very-low-density-lipoprotein type (VLDL)
    • E78.1 Hyperprebetalipoproteinemia
  • E78.2 Mixed hyperlipoproteinaemia
    • E78.2 Hyperbetalipoproteinemia with prebetalipoproteinemia
    • E78.2 Hypercholesterolemia with endogenous hypertriglyceridemia
    • E78.2 Hyperlipidemia, Group C
    • E78.2 Hyperlipoproteinemia type IIb or III according to Fredrickson
    • E78.2 Lipoproteinemia with broad beta-band (floating-betalipoproteinemia)
    • E78.2 Tubo-eruptive xanthoma
    • E78.2 Xanthoma tuberosum
  • E78.3 Chylomicronemic syndrome (hyperchylomicronemia)
    • E78.3 Mixed hypertriglyceridemia
    • E78.3 Hyperlipidemia, Group D
    • E78.3 Hyperlipoproteinemia type I or V according to Fredrickson
  • E 78.4 Other hyperlipidemia
    • E 78.4 Lipoprotein (a)-Hyperlipoproteinemia
  • E78.5 Hyperlipidemia, not specified
  • E78.6 Hereditary HDL depressions (lipoprotein deficiency)
    • E78.6 A-Betalipoproteinemia
    • E78.6 High-density lipoprotein deficiency
    • E78.6 Hypoalphalipoproteinemia
    • E78.6 Hypobetalipoproteinemia (familial)
    • E78.6 Lecithin cholesterol acyltransferase deficiency
    • E78.6 Tangier disease

Classification according to etiological aspects (vaiiert n. Oette K 2016):

Hereditary (familial) hypercholesterolemia E78.0

Further lipid metabolism disorders

Familial combined (mixed) hyperlipoproteinemia (FKHL) E78.2

Occurrence/EpidemiologyThis section has been translated automatically.

Disorders of the lipoprotein metabolism are among the most frequently diagnosed diseases in Germany. In the algal group >40 years of age, >50% of the population in western industrialized countries have cholesterol levels >200mg/dl. Analogous values are available for the triglyceride values.

EtiopathogenesisThis section has been translated automatically.

Hyperlipoproteinemias and dyslipoproteinemias are only symptoms. Basically, they can be assigned to 3 clearly definable groups as well as mixed forms:

  • Reactive-physiological forms
  • Primary forms (hereditary forms)
  • Secondary forms (secondary to underlying diseases)
  • Mixed forms

Causes of the secondary forms:

  • Hypertriglyceridemias: diabetes mellitus; metabolic syndrome, obesity; high alcohol consumption, chronic liver and kidney diseases, medications (e.g. retinoids), etc.
  • Hypercholesterolaemia: e.g. nephrotic syndrome, hypothyroidism, diabetes mellitus, drugs (e.g. glucocorticoids, retinoids)
  • Mixed forms (combined hypertriglyceridemias/hypercholesterolemias): as in hypercholesterolemias

Differential diagnosisThis section has been translated automatically.

TablesThis section has been translated automatically.

Therapy recommendations of the European Atherosclerosis Society

Severity

LDL/cholesterol

Non-drug therapy

Drug therapy

Hypercholesterolemia

Light

LDL-C: 135-175 mg/dl

Dietary advice, intensive advice for other risks

for CHD or other risks

Total C: 200-250 mg/dl

Moderate

LDL-C: 175-215 mg/dl

Dietary advice with controls over 3 months mostly successful

for CHD or high risk after 3-6 months diet, CSE inhibitors, resins, fibrates

Total C: 250-300 mg/dl

Heavy

LDL-C: > 215 mg/dl

Diet sometimes successful

after 3-6 months diet, CSE inhibitors, resins

Total C: > 300 mg/dl

Hypertriglyceridemia

Moderate

TG: 200-400 mg/dl

Weight loss, alcohol withdrawal, diabetes control, discontinue lipid-enhancing medication (e.g. thiazide diuretics)

for low HDL and CHD or high risk: fibrates, nicotinic acid, fish oil

LDL-C: < 135 mg/dl

Heavy

TG: > 400 mg/dl

with TG increase short dietary phase, because with increasing values danger of pancreatitis

after 2-3 months diet without significant improvement: fibrates, nicotinic acid, fish oil

LDL-C: < 135 mg/dl

Diet/life habitsThis section has been translated automatically.

  • Cholesterol-reducing diet: total fat reduction, vegetable fats rich in linoleic acid are preferred, cholesterol restriction (1 egg yolk = 270 mg cholesterol!), fibre (binds fats in the intestine), fish with omega-3 fatty acids.
  • Triglycerin-reducing diet: total fat reduction, alcohol restriction, preference for vegetable fats rich in linoleic acid, low sugar, many small meals.

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Last updated on: 26.12.2021