Glucose-Galactose Malabsorption

Key Features

• Rare disorder in which the sodium-glucose transport protein is defective

• Transport of glucose in the intestinal epithelium and renal tubule is impaired

• Acquired, transient form occurs mainly in infants younger than 6 months, usually following acute viral or bacterial enteritis

Clinical Findings

• Diarrhea begins with the first feedings, accompanied by reducing sugar in the stool and acidosis

Diagnosis

• Small bowel histology is normal

• Glycosuria and aminoaciduria may occur

• Glucose tolerance test is abnormal

• Fructose is well tolerated

Treatment

• In congenital disease,

  • Exclusion of glucose and galactose from the diet is mandatory

  • A carbohydrate-free base formula is used with added fructose

• Prognosis is good if diagnosed early.

• Tolerance for glucose and galactose improves with age