5C80.00

ICD-11 MMS 2026-01

Primary hypercholesterolaemia

Description

This is a genetic disorder characterised by high cholesterol levels, specifically very high levels of low-density lipoprotein (LDL, "bad cholesterol"), in the blood and early cardiovascular disease.

Index terms

  • Primary hypercholesterolaemia
  • LDL - [low density lipoprotein} hyperlipoproteinemia
  • cholesterolaemia
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  • essential cholesterolaemia
  • essential hypercholesterolaemia
  • group A hyperlipemia
  • group A hyperlipidaemia
  • increased low density lipoprotein
  • low-density-lipoid-type hyperlipoproteinemia
  • low-density-lipoprotein-type
  • low-density-lipoprotein-type hyperlipoproteinemia
  • pure hypercholesterinaemia
  • pure hypercholesterolaemia
  • Familial hypercholesterolaemia
  • Fredrickson 2A
  • Fredrickson type 2A hyperlipoproteinemia
  • Hypercholesterolaemia due to LDL receptor deficiency
  • essential familial hypercholesterolaemia
  • familial cholesterolaemia
  • familial hyperbetalipoproteinaemia
  • familial hypercholesterinaemia
  • hereditary cholesterolaemia
  • hereditary hypercholesterinaemia
  • hereditary hypercholesterolaemia
  • low density lipoprotein catabolic defect
  • low density lipoprotein receptor disorder
  • Familial hypercholesterolaemia - heterozygous
  • Familial hypercholesterolaemia - homozygous
  • Familial hypercholesterolemic xanthomatosis
  • Familial defective apolipoprotein B-100
  • Autosomal recessive hypercholesterolaemia
  • Autosomal dominant hypercholesterolaemia
  • Polygenic hypercholesterolaemia
  • Hyperbetalipoproteinaemia

Exclusions from above levels

  • 5C56.0 – Sphingolipidosis
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
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  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C80.01 – Secondary hypercholesterolaemia
  • 5C80.0Z – Hypercholesterolaemia, unspecified

Foundation entity

Primary hypercholesterolaemia – http://id.who.int/icd/entity/163750325

Part of: 5C80.0: Hypercholesterolaemia › 5C80: Hyperlipoproteinaemia › 05: Endocrine, nutritional or metabolic diseases