Angeborene Glucose- und Galaktose-Malabsorption

Quantitative Radioautography of Sugar Transport in Intestinal Biopsies from Normal Humans and a Patient with Glucose-Galactose Malabsorption

“…7, 8). Slow entry capable of proceeding uphill suggests limited carrier function in this particular patient and is consistent with reports from two other patients that glucose absorption in vivo proceeded at about 10% of the normal rate (12,16). Furthermore, evaluation of glucose-galactose carriers by phlorizin binding (Table II) indicates that the carrier density in the patient's microvillar membrane was about 10% of normal, while, at least with respect to phlorizin affinity or 1/K, the function of the existing carriers was unchanged.…”

“…Although the precise defect is yet to be explained, studies of several patients with this disorder indicate that it is a specific inborn error of sugar transport. Patients exhibit a life-threatening impairment of glucosegalactose absorption, while absorption of fructose (12)(13)(14), amino acids (15,16), sodium (16), and apparently other metabolites is normal. Their pedigrees indicate that the disorder is inherited in an autosomal recessive manner (9,17).…”

Renal and intestinal hexose transport in familial glucose-galactose malabsorption

“…patients have been reported previously with intestinal malabsorption of the sterically similar monosaccharides D-glucose and D-galactose (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14). The familial nature of this disease was first determined by Lindquist and Meeuwisse in a study of one large Swedish family (2,14,15).…”

Glucose-galactose malabsorption

“…Some absorption, probably by passive diffusion, of glucose and galactose does take place, however. This was shown by Linneweh, Schaumloffel, Graul, and Bode (1966) who found, by determining the absorption rate of orally administered 14Clabelled glucose and galactose, that their patient absorbed 7 6% and 4. 5%, respectively, the corresponding values in normal controls being 95-5 % and 83 0%.…”