Jj. Baudon et al., SUCRASE-ISOMALTASE DEFICIENCY - CHANGING PATTERN OVER 2 DECADES, Journal of pediatric gastroenterology and nutrition, 22(3), 1996, pp. 284-288
Twenty-two cases of sucrase-isomaltase deficiency (SID) were observed
over a period of 20 years. Since 1977 delay of introduction of sucrose
and its decrease in infants' diets have modified the symptomatology.
In general, onset of diarrhea has not taken place immediately but 15 d
ays to 2 months after introduction of sucrose. Out of 12 cases with de
hydration, five occurred 3 to 7 months after the beginning of sucrose
diet. Hypotrophy was not constant (11 of 22 cases), thus diagnosis was
delayed in 17 of 22 cases. A yellow complexion due to rising carotene
levels in the blood is a striking feature. Because of falsely positiv
e sucrose load tests (four out of 14 nonSID infants) and failure of th
e hydrogene breath test (one out of five studied cases), disaccharidas
e determination remains the key to diagnosis. Despite the genetic diff
erence symptoms seem to depend on infant feeding practices.