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

Abstract

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.

• Authors: F M Méndez Aparicio
• Journal: Anales espanoles de pediatria (An Esp Pediatr) Vol. 13 Issue 8 Pg. 720-5 (Aug 1980) ISSN: 0302-4342 [Print] Spain
• Vernacular Title: Necrosis isquémica de las extremidades en una deficiencia congénita de sacarasa isomaltasa.
• PMID: 7436153 (Publication Type: Case Reports, English Abstract, Journal Article)

Chemical References

• Multienzyme Complexes
• Sucrose
• Sucrase-Isomaltase Complex

Topics

• Carbohydrate Metabolism, Inborn Errors (complications)
• Diarrhea, Infantile (chemically induced, complications)
• Disseminated Intravascular Coagulation (etiology)
• Fingers (pathology)
• Gangrene (etiology)
• Humans
• Infant
• Infant Food (adverse effects)
• Male
• Multienzyme Complexes (deficiency)
• Shock (etiology)
• Sucrase-Isomaltase Complex (deficiency)
• Sucrose (metabolism)
• Toes (pathology)