Catalog

Congenital lactase deficiency

5C61.61

Link to WHO Definition

Congenital lactase deficiency – http://id.who.int/icd/entity/2109252471

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.

Parents