ICD-11 classes
13 Diseases of the digestive system
Diseases of small intestine
DA96 — Intestinal malabsorption or protein-losing enteropathy
DA96.0 — Intestinal malabsorption
DA96.02 — Malabsorption or intolerance of specific nutrients
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:

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