3A91

ICD-11 MMS 2026-01

Congenital methaemoglobinaemia

Description

A disease caused by determinants in the antenatal period leading to lack of the enzyme cytochrome b5 reductase. This disease is characterised by elevated levels of methemoglobin within the blood leading to haemoglobin ineffectively releasing oxygen to body tissues. This disease may present with shortness of breath, cyanosis, headache, fatigue, exercise intolerance, dizziness and loss of consciousness. Confirmation is by identification of mutation by genetic testing.

Index terms

  • Congenital methaemoglobinaemia

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

  • 3A50 – Thalassaemias
  • 3A51 – Sickle cell disorders or other haemoglobinopathies
  • 3A70 – Aplastic anaemia
  • 3A71 – Anaemia due to chronic disease
  • 3A72 – Sideroblastic anaemia
  • 3A73 – Congenital dyserythropoietic anaemia
  • 3A90 – Anaemia due to acute disease
  • 3A92 – Hereditary methaemoglobinaemia
  • 3A93 – Acquired methaemoglobinaemia
  • 3A94 – Acute posthaemorrhagic anaemia
  • 3A9Y – Other specified anaemias or erythrocyte disorders
  • 3A9Z – Anaemias or other erythrocyte disorders, unspecified

Foundation entity

Congenital methaemoglobinaemia – http://id.who.int/icd/entity/1981095916

Part of: 03: Diseases of the blood or blood-forming organs