ICD-11 classes
13 Diseases of the digestive system
Diseases of small intestine
DA90 — Nonstructural developmental anomalies of small intestine
DA90.1 — Congenital intestinal transport defect
5C61.0 — Glucose-galactose malabsorption
ICD-11 5C61.0 — Glucose-galactose malabsorption
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.
The diagnosis includes nothing.
It excludes 1 item.
- Glucose or galactose intolerance of newborn (5C51.42)
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: