[Peripheral gangrene in a congenital sucrase isomaltase deficiency (author's transl)].

A case of ischaemic necrosis of extremities due to haemodynamic shock with disseminated intravascular coagulation (DIC) is reported. The onset of haemodynamic shock and the appearance of vascular disorder were produced at once. Both processes, haemodynamic shock and DIC were originated by the administration of food containing sucrose and starch to an infant whose posterior study confirms to suffert a congenital sucrase isomaltase deficiency. Sucrase isomaltase deficiency was documented by oral sucrose loading tests and by enzymatic assay of small intestinal mucose biopsy.