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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Recent developments in Hirschsprung's-associated enterocolitis
Elizabeth M Pontarelli1, Henri R Ford, Christopher P Gayer
1Children's Hospital Los Angeles, 4650 Sunset Blvd MS#100, Los Angeles, CA 90027, USA.
Hirschsprung's-associated enterocolitis (HAEC) continues to be a significant source of morbidity for patients with Hirschsprung's disease (HD). New clinical and histologic classification systems for HAEC will improve consistency between reports and increase the ability to compare outcomes. A complete understanding of disease pathogenesis is lacking, but evidence suggests that the intestinal microbiota may play a role in the development of HD and HAEC. The benefits of adjunctive therapies, such as anal dilations and botulinum toxin to reduce the incidence of HAEC following corrective endorectal pull-through, remain controversial. Finally, new clinical data have identified an association between HAEC and inflammatory bowel disease and will likely lead to further genetic studies to elucidate the connection between these two disease processes.
Hirschsprung's-associated enterocolitis (HAEC) continues to be a significant source of morbidity for patients with Hirschsprung's disease (HD). New clinical and histologic classification systems for HAEC will improve consistency between reports and increase the ability to compare outcomes. A complete understanding of disease pathogenesis is lacking, but evidence suggests that the intestinal microbiota may play a role in the development of HD and HAEC. The benefits of adjunctive therapies, such as anal dilations and botulinum toxin to reduce the incidence of HAEC following corrective endorectal pull-through, remain controversial. Finally, new clinical data have identified an association between HAEC and inflammatory bowel disease and will likely lead to further genetic studies to elucidate the connection between these two disease processes.
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