48 ENZYME REPLACEMENT THERAPY WITH YEAST SACCHAROMYCES CEREVISIAE IN CONGENITAL SUCRASE-ISOMALTASE-DEFICIENCY (SID)

Abstract

In an 11 year old girl with SID, treatment with fresh bakers yeast resulted in a dramatic clinical improvement. Therefore we have started a study on saccharolytic enzymes in yeast in vivo and in vitro. In vivo H2 breath tests ( HBT ) were performed in 8 patients with SID upon sucrose loading ( 2 g/kg, X = 41 g ) with and without the addition of 0.3 g lyophilized Saccharomyces cerevisiae ( LSC ). H2 values were corrected for atmospheric contamination and fasting levels. In addition to clinical improvements half of the patients showed a normalization of the HBTs and the remainder a clear decrease of the H2 areas under the curves by administration of yeast ( average H2 reduction by 70 % ). In a control group of 5 patients with the adult form of lactase deficiency neither clinical symptoms nor HBT after the lactose loading were changed by the yeast therapy. - The sucrase activity in LSC as well as in fresh bakers yeast was very high ( 6.2 and 24 mmol/min/g protein respectively ) but the isomaltase and lactase activities were relatively low ( isomaltase 0.91 ± 0.01 and 0.28 ± 0.03 resp., lactase 0.06 ± 0.005 and 0.2 ± 0.03 resp. ). Dried brewers yeast showed only a very low sucrase activity ( 0.14 mmol/min/g protein ). - In contrast to the fecal flora, yeast did not release any hydrogen from carbohydrates under anaerobic conditions in vitro. - In conclusion, patients with SID and difficulties in keeping a sucrose free diet will benefit from a small amount of viable yeast cells after a sucrose rich meal. -Supported by Deutsche Forschungsgemeinschaft.