Primary glucose-galactose malabsorption

Abstract

Primary glucose- galactose malabsorption is a rare hereditary disorder, most probably due to a defect in the active transport of both monosaccharides in the small intestinal mucosa. Severe watery diarrhea, dehydration and acidosis appearing soon after birth are the most prominent signs. Withdrawal of both glucose and galactose from the diet is the only adequate therapy. The authors describe the clinical course during the first 2 years of life in a girl with this disorder.