Clostridial necrotizing enteritis

Clostridial necrotizing enteritis (CNE) -- also called enteritis necroticans and pigbel -- is an often fatal type of food poisoning caused by a β-toxin of Clostridium perfringens, Type C. It occurs in some developing countries, but was also documented in Germany following World War II. The toxin is normally inactivated by certain proteolytic enzymes and by normal cooking, but when these protections are impeded, the disease emerges.

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Etiology

All the factors collectively causing CNE are generally only present in the hinterlands of New Guinea and parts of Africa, Latin America, and Asia. These factors include protein deprivation (causing inadequate synthesis of trypsin protease (an enzyme), to which the toxin is very sensitive), poor food hygiene, episodic meat feasting, staple diets containing trypsin inhibitors (sweet potatoes), and infection by Ascaris parasites which secrete a trypsin inhibitor. In New Guinea (origin of the term “pigbel”), the disease is usually spread through contaminated meat (especially pork) and perhaps by peanuts. (CNE was also diagnosed in post WWII Germany, where it was known as Darmbrand or "fire bowels").

Clinical aspects

CNE is a necrotizing inflammation the small bowel (especially the jejunum but also the ileum). Clinical results may vary from mild diarrhea to a life-threatening sequence of severe abdominal pain, vomiting, bloody stool, ulceration of the small intestine with leakage (perforation) into the peritoneal cavity and possible death within a single day due to peritonitis. Treatment involves suppressing the toxin-producing organisms with antibiotics such as penicillin G or metronidazole. About half of seriously ill patients require surgery for perforation, persistent intestinal obstruction, or failure to respond to the antibiotics. An investigational toxoid vaccine has been used successfully in some developing countries but is not available outside of research.

Other Clostridial toxemias

• Leukemia patients, cancer chemotherapy recipients and others suffering from suppressed white blood cells (neutropenia) can be afflicted by a similar syndrome, neutropenic enterocolitis, in which the cecum is targeted by Clostridium septicum in much the same way.

• In neonatal intensive care units, the syndrome of neonatal necrotizing enterocolitis may be caused in a similar way by C. perfringens, C. butyricum, and C. difficile, but this has not been proved.

References

Cooke RA. "Pig Bel." Perspectives in Pediatric Pathology. 1979;5:137-52. PMID: 575409

Murrell TG, Roth L, Egerton J, Samels J, Walker PD. "Pig-bel: enteritis necroticans. A study in diagnosis and management." Lancet. 1966 Jan 29;1(7431):217-22. PMID: 4159182

Murrell TG, Egerton JR, Rampling A, Samels J, Walker PD. "The ecology and epidemiology of the pig-bel syndrome in man in New Guinea." Journal of Hygiene, London. 1966 Sep;64(3):375-96. PMID: 4288244

Nuland, Sherwin B., “The Beast in the Belly”, Discover, Vol. 16 No. 02 (February 1995).