Lactase, the enzyme that breaks down lactose, splits the lactose into two monosaccharides or single sugars: glucose and galactose. Youve read {{metering-count}} of {{metering-total}} articles this month. In adult onset lastose intolerance, lactase enzyme production starts to decrease as a person matures. Learn more about the Merck Manuals and our commitment to Global Medical Knowledge. Disaccharidase deficiency | Oxford Textbook of Medicine | Oxford Academic Inherited (congenital) enzyme deficiency, which is a rare condition that is present from birth Usually this disorder occurs in babies and children tend to outgrow it as they age. 541-6. Except in the case of a lactase deficiency, which improves dramatically when lactose is removed from the diet, it is often difficult to separate specific disaccharide intolerances from each other. The lactose composition of her milk will remain constant, regardless of whether she consumes milk and dairy products. Elimination. Most legumes and beans are starchy, so this content should be taken into consideration. In general, most individuals with CSID can eat plain sources of protein, including beef, pork, lamb, fish, turkey, chicken, and eggs. Clinical Characteristics of Disaccharidase Deficiencies Amon 1967 Sep;53(3):371-374. It is absolutely amazing, he is a different kid! Undigested disaccharides cause an osmotic load that attracts water and electrolytes into the bowel, causing watery diarrhea. You should also avoid processed meats such as bacon, sausage, luncheon meat, deli meat, liverwurst, and pt since many of these food items are cured with sucrose or have starch fillers. 2012; 55(2):S28-S30. 0000010088 00000 n If the infant is allergic to cows milk proteins an extensively hydrolyzed sucrose-free casein formula such as Enfamil Nutramigen (Mead Johnson) and Enfamil Pregestimil (Mead-Johnson) is recommended.. A convenient way to manage disaccharide intolerances is to conduct the diet in three phases:3. References <<06E783B5A772D64DBD765FF42D03F1F7>]/Prev 398468/XRefStm 1706>> If the deficiency is due to a temporary deficiency, dietary exclusion of all the disaccharides, followed by reintroducing each one individually in increasing doses, may be feasible. Note that the chart below shows ranges for abnormal laboratory values in the United States only. (Genetically programmed down-regulation of the lactase gene is detectable in children from the second year of life, although the onset and extent are somewhat variable. 1972 Jun;60(6):478-82. However, 80 to 85% of whites of Northwest European descent produce lactase throughout life and are thus able to digest milk and milk products.