Glycogen storage disease type I. Results of treatment with frequent daytime feeding, combined with nocturnal intragastric feeding and with administration of an alpha-glucosidase inhibitor.
Seven patients with glycogen disease type I have been treated with nocturnal intragastric feeding combined with frequent daytime feeding. Follow-up shows a striking improvement in their clinical condition including growth rate. Determination of biochemical parameters reveals a significant increase in mean blood glucose levels and a significant decrease of lactate, pyruvate, alanine, uric acid, triglycerides, and SGOT in blood. Additional administration of an alpha-glucosidase inhibitor in four patients caused a significant increase in blood lactate despite unchanged blood glucose levels.
D020909:Acarbose; D000409:Alanine / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D001786:Blood Glucose / Q000032:analysis; D002648:Child; D004750:Enteral Nutrition; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000188:drug therapy / Q000628:therapy*; D006801:Humans; D007773:Lactates / Q000097:blood; D008297:Male; D009844:Oligosaccharides / Q000627:therapeutic use*; D011773:Pyruvates / Q000097:blood; D013997:Time Factors; D014280:Triglycerides / Q000097:blood; D014312:Trisaccharides / Q000627:therapeutic use*; D014527:Uric Acid / Q000097:blood
D001786:Blood Glucose; D007773:Lactates; D009844:Oligosaccharides; D011773:Pyruvates; D014280:Triglycerides; D014312:Trisaccharides; D014527:Uric Acid; D001219:Aspartate Aminotransferases; D000409:Alanine; D020909:Acarbose