Enteritis necroticans (Pigbel) in a diabetic child.

Background: Enteritis necroticans (pigbel), an often fatal illness characte rized by hemorrhagic, inflammatory, or ischemic necrosis of the jejunum, oc curs in developing countries but is rare in developed countries, where its occurrence is confined to adults with chronic illnesses. The causative orga nism of enteritis necroticans is Clostridium perfringens type C, an anaerob ic gram-positive bacillus. Methods: In December 1998, enteritis necroticans developed in a 12-year-old boy with poorly controlled diabetes mellitus after he consumed pig intesti nes (chitterlings). He presented with hematemesis, abdominal distention, an d severe diabetic ketoacidosis with hypotension. At laparotomy, extensive j ejunal necrosis required bowel resection, jejunostomy, and ileostomy. Sampl es were obtained for histopathological examination. Polymerase-chain-reacti on (PCR) assay was performed on paraffin-embedded bowel tissue with primers specific for the cpa and cpb genes, which code for the alpha and beta toxi ns produced by C. perfringens. Results: Histologic examination of resected bowel tissue showed extensive m ucosal necrosis, the formation of pseudomembrane, pneumatosis, and areas of epithelial regeneration that alternated with necrotic segments - findings consistent with a diagnosis of enteritis necroticans. Gram's staining showe d large gram-positive bacilli whose features were consistent with those of clostridium species. Through PCR amplification, we detected products of the cpa and cpb genes, which indicated the presence of C. perfringens type C. Assay of ileal tissue obtained during surgery to restore the continuity of the patient's bowel was negative for C. perfringens. Conclusions: The preparation or consumption of chitterlings by diabetic pat ients and other chronically ill persons can result in potentially life-thre atening infectious complications. (N Engl J Med 2000;342:1250-3.) (C) 2000, Massachusetts Medical Society.