3A10.3

ICD-11 MMS 2026-01

Familial pseudohyperkalaemia

Description

A disease caused by a genetically inherited mutation. This disease is characterised by a temperature-dependent defect in red cell membrane permeability to potassium that leads to high in vitro potassium levels in samples stored below 37°C leading to elevated potassium levels in the blood that does not reflect the true potassium level. Confirmation is by identification of genetic mutation through genetic testing.

Index terms

  • Familial pseudohyperkalaemia
  • Hereditary pseudohyperkalaemia
  • Familial pseudohyperkalaemia type 1
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  • Familial pseudohyperkalaemia type 2
  • Familial pseudohyperkalaemia, Cardiff type

Exclusions from above levels

  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth or the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3A10.0 – Haemolytic anaemias due to hexose monophosphate shunt or glutathione metabolism anomalies
  • 3A10.1 – Haemolytic anaemia due to adenosine deaminase excess
  • 3A10.2 – Hereditary elliptocytosis
  • 3A10.Y – Other specified hereditary haemolytic anaemia
  • 3A10.Z – Hereditary haemolytic anaemia, unspecified

Foundation entity

Familial pseudohyperkalaemia – http://id.who.int/icd/entity/1653996588

Part of: 3A10: Hereditary haemolytic anaemia › 03: Diseases of the blood or blood-forming organs