5C61.0

ICD-11 MMS 2026-01

Glucose-galactose malabsorption

Description

Glucose-galactose malabsorption is characterised by diarrhoea and severe neonatal dehydration. Around 300 cases have been described to date. Moderate glucosuria has also been reported, but fructose absorption is normal. Glucose-galactose malabsorption is caused by a mutation in the SLC5A1 gene, encoding the glucose-sodium cotransporter, SGTL1. The mode of transmission is autosomal recessive. The fatal consequences of this syndrome can be avoided by following a glucose and galactose restricted diet.

Index terms

  • Glucose-galactose malabsorption
  • Congenital monosaccharide malabsorption
  • SGLT1 deficiency
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  • Congenital glucose-galactose malabsorption

Excludes

  • 5C51.42 – Glucose or galactose intolerance of newborn

Exclusions from above levels

  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
  • 5A81.1 – 5-alpha-reductase deficiency
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  • 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

  • 5C61.1 – Maltase-glucoamylase deficiency
  • 5C61.2 – Congenital sucrase-isomaltase deficiency
  • 5C61.3 – Alpha, alpha trehalase deficiency
  • 5C61.4 – Acquired monosaccharide malabsorption
  • 5C61.5 – Disorders of facilitated glucose transport
  • 5C61.6 – Lactose intolerance
  • 5C61.Y – Other specified disorders of carbohydrate absorption or transport
  • 5C61.Z – Disorders of carbohydrate absorption or transport, unspecified

Foundation entity

Glucose-galactose malabsorption – http://id.who.int/icd/entity/2108415931

Part of: 5C61: Disorders of carbohydrate absorption or transport › 05: Endocrine, nutritional or metabolic diseases