A patient with Hirschsprung's disease may develop an enterocolitis. This can be a source of significant morbidity and mortality.
The patient may be known to have Hirschsprung's disease or the patient may first present with the enterocolitis.
Clinical findings:
(1) fever with temperature > 38°C (100.4°F)
(2) diarrhea
(3) abdominal distention
(4) crampy abdominal pain
(5) bloody stools
(6) prostration
(7) lethargy
A patient with entercolitis should have a complete workup to identify an underlying pathogen such as Clostridium difficile, Cryptosporidium or enterocyte adherent E. coli..
Risk factors for enterocolitis:
(1) trisomy 21
(2) diagnosis of Hirschsprung's disease after 1 week of age
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