PEDIATRICS Vol. 36 No. 2 August 1965, pp. 231-235
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THE HANDLING OF SOYA ALPHA-GALACTOSIDES BY A NORMAL AND A GALACTOSEMIC CHILD

Richard Gitzelmann M.D.1 and Salvatore Auricchio M.D.1

1 Laboratory for Metabolic Research, University Children's Hospital, Zürich, Switzerland

1. No alpha-galactosidase activity in homogenates of human small intestinal mucosa was demonstrated either with stachyose, raffinose, or melibiose.

2. In a healthy child, oral raffinose and melibiose loads failed to produce changes in blood glucose, blood galactose, or red cell galactose-1-phosphate. Trace amounts of either saccharide were excreted in the urine. Following the raffinose load, diarrhea occurred and raffinose and melibiose as well as their hydrolysis products were found in the feces.

3. In a galactosemic patient, red cell galactose-1-phosphate rose as expected after ingestion of 2 gm of galactose. No such rise was seen after administration of equivalent and of double equivalent amounts of stachyose. Prolonged dietary supplementation with raffinose did not significantly alter non-fasting erythrocyte galactose-1-phosphate.

4. It is concluded that soybean formulas are generally safe for galactosemic infants; however, caution is advised for patients suffering from diarrhea.

Submitted on October 20, 1964
Accepted on February 15, 1965