5C54.Z

ICD-11 MMS 2026-01

Inborn errors of glycosylation or protein modification, unspecified

Exclusions from above levels

  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 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

  • 5C54.0 – Disorders of protein N-glycosylation
  • 5C54.1 – Disorders of protein O-glycosylation
  • 5C54.2 – Disorders of multiple glycosylation or other pathways
  • 5C54.Y – Other specified inborn errors of glycosylation or other specified protein modification

Foundation entity

Inborn errors of glycosylation or other specified protein modification – http://id.who.int/icd/entity/85138870

Part of: 5C54: Inborn errors of glycosylation or other specified protein modification › 05: Endocrine, nutritional or metabolic diseases