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5C61.61 Congenital lactase deficiency

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

synonyms

• Congenital lactase deficiency
• hereditary alactasia
• congenital lactose intolerance
• congenital alactasia
• congenital alactasia syndrome
• congenital lactose malabsorption
• disaccharide intolerance type 2
• CLD - [congenital lactase deficiency]
• disaccharide intolerance 2
• lactose intolerance of newborn
• hereditary lactase deficiency

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