5C51.0

ICD-11 MMS 2026-01

Disorders of the pentose phosphate pathway

Index terms

  • Disorders of the pentose phosphate pathway
  • Essential pentosuria
  • L-xylulose reductase deficiency
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  • L-xylulosuria
  • Xylitol dehydrogenase deficiency
  • Ribose-5-phosphate isomerase deficiency
  • Transaldolase deficiency

Exclusions from above levels

  • 5A41 – hypoglycaemia NOS
  • 5A42 – Increased secretion of glucagon
  • 5C56.3 – Mucopolysaccharidosis
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  • http://id.who.int/icd/entity/465177735 – Diabetes mellitus
  • 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
  • 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

Foundation children elsewhere

  • 3A10.00 – Haemolytic anaemia due to glucose-6-phosphate dehydrogenase deficiency

Sibling codes

  • 5C51.1 – Disorders of glycerol metabolism
  • 5C51.2 – Disorders of glyoxylate metabolism
  • 5C51.3 – Glycogen storage disease
  • 5C51.4 – Disorders of galactose metabolism
  • 5C51.5 – Disorders of fructose metabolism
  • 5C51.Y – Other specified inborn errors of carbohydrate metabolism
  • 5C51.Z – Inborn errors of carbohydrate metabolism, unspecified

Foundation entity

Disorders of the pentose phosphate pathway – http://id.who.int/icd/entity/2067324607

Part of: 5C51: Inborn errors of carbohydrate metabolism › 05: Endocrine, nutritional or metabolic diseases