Journal of Pediatric Gastroenterology & Nutrition:
Letter to the Editor
To the Editor: I read with interest the article by Wood et al. (1) concerning necrotising enteritis in a 2-year-old boy who was subsequently diagnosed with cystic fibrosis.
The data from this case report were extrapolated to support the theory that deficient pancreatic proteolytic activity predisposes to tissue damage from the β toxin of Clostridium perfringens type C. This is a fascinating theory that has been proposed to explain the finding of pigbel in Papuan New Guinea highlanders, particularly children (2), prior to the development of a successful vaccine (3). Pancreatic proteolytic activity is thought to be depressed in highlanders secondary to marginal protein intake and a diet rich in protease inhibitors. It is unusual for highlanders to be frankly malnourished, but they rely more on the sweet potato as a dietary staple than the coastal people who rarely, if ever, suffer from pig-bel, even though they consume quantities of pork that are often prepared in unsanitary conditions. Sweet potato contains various proteolytic inhibitors that may prevent the destruction of the β toxin (2).
I am not sure whether the data presented support this theory. No details were given of the preceding dietary intake; gram-negative as opposed to gram-positive organisms were seen in the gut lumen; and serum was not assessed for the presence of β toxin. Alternative explanations are just as plausible. A similar picture is seen in neutropenic enterocolitis with Clostridium septicum and other clostridial species (4,5). In Western communities, this entity is probably more common than pig-bel.
The child had an elevated white count, but no assessment was made of neutrophil function, which can be depressed in malnourished states (6) and probably should be evaluated postrecovery. This child had a height/weight problem that was presumably secondary to pancreatogenous malabsorption. Deficiency of antioxidant fat-soluble vitamin E and vitamin A (7) may have further limited the child's defenses against tissue damage. The hypothesis that the pancreas acts as a policeman to mediate the undesirable toxin-mediated effects of pathogens is appealing but needs further support.
University of Adelaide
Department of Pediatrics
Women's and Children's Hospital
North Adelaide, South Australia
1. Wood CM, Spicer RD, Beddis IR, Puntis JWL. Pancreatic exocrine failure in cystic fibrosis presenting as necrotising enteritis. J Pediatr Gastroenterol Nutr
2. Lawrence G, Walker PD. Pathogenesis of enteritis necroticans in Papua New Guinea. Lancet
3. Lawrence A, Shann F, Freestone DS, Walker PD. Prevention of necrotising enteritis in Papua New Guinea by active immunisation. Lancet
4. King A, Rampling A, Wight DGF, Warren RE. Neutropenic enterocolitis due to Clostridium septicum
infection. J Clin Pathol
5. Newbold KM, Lord MG, Baglin TP. Role of clostridial organisms in neutropenic enterocolitis. J Clin Pathol
6. Arbo A, Santos JI. Diarrheal diseases in the immunocompromised host. Pediatr Infect Dis J
7. Underwood BA, Denning CR. Blood and liver concentrations of vitamin A and E in children with cystic fibrosis of the pancreas. Pediatr Res