TY - CHAP M1 - Book, Section TI - Disorders of Fructose Metabolism A1 - Roth, Karl S. A1 - Berry, Gerard T. A2 - Sarafoglou, Kyriakie A2 - Hoffmann, Georg F. A2 - Roth, Karl S. PY - 2017 T2 - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e AB - Fructose is a quantitatively important source of carbohydrates, especially in the Western diet. It is a component of table sugar (sucrose, Glc-Fru disaccharide) and is contained in large amounts in honey, fruits, and various vegetables. Fructose, sucrose, and sorbitol (metabolized mainly via fructose) are frequent food additives. A special concern is sucrose-containing infant formulas which may elicit a potentially fatal response in an affected newborn. Fructose also constitutes the main carbohydrate in seminal fluid. The average daily dietary intake by Americans was estimated to be 100 g.1,2 In the last century fructose and sorbitol were used as carbohydrates in parenteral nutrition, for example, equimolar solutions of glucose and fructose prepared by hydrolysis of sucrose as “invert sugar.” IV solutions containing fructose or sorbitol can be toxic to patients with fructose intolerance and should no longer be used. Oral fructose is absorbed in the small intestine and mainly metabolized in the liver. Significant fructose metabolism also takes place in the kidney and the small intestine, explaining the organ manifestations of genetic diseases of fructose metabolism (see below). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1140315020 ER -