Increased concentrations of HbAlab in hereditary fructose intolerance and galactosemia.
In patients with diabetes mellitus nonenzymatic glycosylation of hemo-globin is a result of increased blood glucose concentrations. In analogy glycosylated hemo-globin fractions were determined in 23 patients with hereditary fructose intolerance (HFI) and 8 patients with galactosemia (G) by means of hemoglobin chromatography on a column packed with Bio-Rex 70 resin. The concentrations were compared to those of 14 control patients and 43 patients with type 1 diabetes mellitus. Compared to controls, in HFI- and G-patients HbAlab was significantly increased. In contrast diabetic patients presented with a marked and significant increase of the HbAlc fraction. When purified hemoglobin was incubated with different monosaccharides respectively monosaccharide phosphates, an increase of HbAlab resulted mainly after galactose and fructose-1-phosphate. The determination of HbAlab in patients with HFI and G is considered a possible means of metabolic control.
D002239:Carbohydrate Metabolism, Inborn Errors / Q000235:genetics*; D002648:Child; D003922:Diabetes Mellitus, Type 1 / Q000235:genetics; D005633:Fructose Intolerance / Q000097:blood / Q000235:genetics*; D005693:Galactosemias / Q000097:blood / Q000235:genetics*; D006442:Glycated Hemoglobin / Q000235:genetics*; D006801:Humans
D006442:Glycated Hemoglobin