5C53.01

ICD-11 MMS 2026-01

Lactate dehydrogenase deficiency

Description

This refers to a deficiency in the enzyme present in a wide variety of organisms, including plants and animals. This exists in four distinct enzyme classes. Two of them are cytochrome c-dependent enzymes, each acting on either D-lactate (EC 1.1.2.4) or L-lactate (EC 1.1.2.3). The other two are NAD(P)-dependent enzymes, each acting on either D-lactate (EC 1.1.1.28) or L-lactate (EC 1.1.1.27). This article is about the NAD(P)-dependent L-lactate dehydrogenase.

Index terms

  • Lactate dehydrogenase deficiency

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

  • 5C53.00 – Pyruvate kinase deficiency
  • 5C53.02 – Pyruvate dehydrogenase complex deficiency
  • 5C53.03 – Pyruvate carboxylase deficiency
  • 5C53.0Y – Other specified disorders of pyruvate metabolism
  • 5C53.0Z – Disorders of pyruvate metabolism, unspecified

Foundation entity

Lactate dehydrogenase deficiency – http://id.who.int/icd/entity/1391506239

Part of: 5C53.0: Disorders of pyruvate metabolism › 5C53: Inborn errors of energy metabolism › 05: Endocrine, nutritional or metabolic diseases