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Table 8 Pastor et al. (2009) HAEC score items [76]

From: ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease

History

 Diarrhea with explosive stool

2

 Diarrhea with foul-smelling stool

2

 Diarrhea with bloody stool

1

 Previous history of enterocolitis

1

Physical examination

 

 Explosive discharge of gas and stool on rectal examination

2

 Distended abdomen

2

 Decreased peripheral perfusion

1

 Lethargy

1

 Fever

1

Radiologic examination

 Multiple air fluid levels

1

 Dilated loops of bowel

1

 Sawtooth appearance with irregular mucosal lining

1

 Cut off sign in rectosigmoid region with absence of distal air

1

 Pneumatosis

1

Laboratory

 

 Leukocytosis

1

 Shift to left

1

  1. Note: Using a cut-off of > 4 points to identify suspected HAEC is more sensitive and may have better clinical applicability than Pastor and co-workers' original cut-off of > 10 points [75]
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172