The most common of the hereditary disaccharidase deficiencies is the sucraseisomaltase defect.defective structural element with two separate active side chains for two
different substrates.Refusal of foods with
a high sugar content, abdominal distension, colicky pain and even malnutrition
may occur but, on the whole, symptoms are less pronounced than in lactase
deficiency.Sucrase and isomaltase, the two disaccharidases
involved in the hydrolysis of the two sugars, are maltases 3 and 4 in Semenza's
designation.The stools are frothy,
of low pH, and contain increased quantities of lactic acid.Isomaltose has a 1-6 a
bond between two molecules of glucose and forms the branching link in
glycogen and amylopectin.