Disaccharides and cystic fibrosis of the pancreas.

Disaccharide intolerance has been described in cystic fibrosis of the pancreas (Cozetto, 1963; Nordio et al., 1966; Sunshine and Kretchmer, 1964). However, the relation between clinical signs and symptoms, and biochemical investigations is not clear. For instance, Cozetto (1963) reported isolated depressed lactase activity in an otherwise normal mucosa, but gave no clear clinical evidence in support of lactose intolerance. Two patients of Nordio et al. (1966) with cystic fibrosis of the pancreas showed evidence of disaccharidase deficiency in jejunal biopsies, and flat glycaemic responses to lactose, but disaccharide loads did not produce diarrhoea. In this paper, an attempt has been made to correlate biochemical studies with clinical evidence of disaccharide intolerance. The term 'disaccharide intolerance' will be taken to mean, as suggested by Townley (1966), that there is 'the development of symptoms after ingestion of disaccharide and this is a clinically observed phenomenon'. Seventeen children with cystic fibrosis of the pancreas have been investigated to determine whether intolerance to disaccharides from ordinary sources in the diet contributes to the gastrointestinal symptoms in this disease.