Intestinal Sucrase Deficiency Presenting as Sucrose Intolerance in Adult Life

Abstract

The onset in adult life of intolerance to sucrose and an impaired starch tolerance, coincident with an acute diarrhoeal episode, is described in a 29-year-old man with a two-year history of diarrhoea. Enzyme assays of jejunal mucosa showed a virtual absence of sucrase, with very low palatinase and reduced maltase activities, and it is suggested that this is a case of hereditary sucrose intolerance, unmasked by an acute attack of gastro-enteritis. Response to a sucrose-free and restricted starch diet has been satisfactory. We are grateful to Dr. J. Dow, St. George's Hospital, Tooting, and Dr. C. C. Booth, Hammersmith Hospital, for permission to publish this case. We wish to thank Dr. E. Ann Burgess for the mucosal enzyme studies ; Dr. E. O. Riecken and Professor A. G. E. Pearse for the histochemical studies ; Mr. J. Spencer for urine chromatography ; Dr. W. Payne for the faecal lactic acid determinations ; and the Staff of the Metabolic Unit, Hammersmith Hospital for making this study possible. One of us (G. N.) is indebted to Smith Klein and French Ltd. for a grant. © 1965, British Medical Journal Publishing Group. All rights reserved.