ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Disorders of metabolite absorption or transport
5C61 — Disorders of carbohydrate absorption or transport
5C61.6 — Lactose intolerance
5C61.61 — Congenital lactase deficiency
ICD-11 5C61.61 — Congenital lactase deficiency
This is a congenital deficiency of lactase (EC 3.2.1.108), inherited as an autosomal recessive trait, presenting in infancy and manifested by profuse watery diarrhoea in response to dietary milk, due to inability to digest lactose, a sugar found in milk and to a lesser extent milk-derived dairy products. The condition may lead to marasmus and death if lactose is not eliminated from the diet.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: