Last update Oct. 4, 2020

Congenital lactose intolerance

Very High Risk

Very unsafe. Contraindicated. Use of an alternative or cessation of breastfeeding. Read the Comment.

A very rare and extremely serious disease, via autosomal recessive inheritance (Vandenplas 2015, Diekmann 2015, Torniainen 2009, Järvelä 1998, Levin 1970).

The congenital absence of intestinal lactase prevents the hydrolyzing of lactose in breastmilk, resulting in very serious diarrhea from the first days of life (Torniainen 2009, Savilahti 1973, Levin 1970).

Although the treatment consists of a lactose-free diet and, therefore, it is not possible to breastfeed, there is a reported case of feeding an infant suffering from congenital lactose intolerance with hydrolyzed bank milk with added lactase: 900 units of lactase activity in 200 ml of milk (Similä 1982).

Breastfeeding is not contraindicated in temporary intolerance secondary to gastroenteritis (Vandenplas 2015).

Suggestions made at e-lactancia are done by APILAM team of health professionals, and are based on updated scientific publications. It is not intended to replace the relationship you have with your doctor but to compound it. The pharmaceutical industry contraindicates breastfeeding, mistakenly and without scientific reasons, in most of the drug data sheets.

Jose Maria Paricio, Founder & President of APILAM/e-Lactancia

Your contribution is essential for this service to continue to exist. We need the generosity of people like you who believe in the benefits of breastfeeding.

Thank you for helping to protect and promote breastfeeding.

José María Paricio, founder of e-lactancia.

Other names

Congenital lactose intolerance is also known as


Congenital lactose intolerance belongs to this group or family:


  1. Vandenplas Y. Lactose intolerance. Asia Pac J Clin Nutr. 2015;24 Suppl 1:S9-13. Abstract
  2. Diekmann L, Pfeiffer K, Naim HY. Congenital lactose intolerance is triggered by severe mutations on both alleles of the lactase gene. BMC Gastroenterol. 2015 Mar 21;15:36. Abstract
  3. Torniainen S, Savilahti E, Järvelä I. [Congenital lactase deficiency--a more common disease than previously thought?]. Duodecim. 2009;125(7):766-70. Finnish. Abstract
  4. Järvelä I, Enattah NS, Kokkonen J, Varilo T, Savilahti E, Peltonen L. Assignment of the locus for congenital lactase deficiency to 2q21, in the vicinity of but separate from the lactase-phlorizin hydrolase gene. Am J Hum Genet. 1998 Oct;63(4):1078-85. Abstract
  5. Savilahti E, Launiala K, Kuitunen P. Congenital lactase deficiency. A clinical study on 16 patients. Arch Dis Child. 1983 Apr;58(4):246-52. Abstract
  6. Similä S, Kokkonen J, Kouvalainen K. Use of lactose-hydrolyzed human milk in congenital lactase deficiency. J Pediatr. 1982 Oct;101(4):584-5. No abstract available. Abstract
  7. Levin B, Abraham JM, Burgess EA, Wallis PG. Congenital lactose malabsorption. Arch Dis Child. 1970 Apr;45(240):173-7. No abstract available. Abstract

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