The application of immunohistochemistry in the diagnosis of Hirschsprung's disease and allied diseases

Objective To study the use of immunohistochemistry in the diagnosis of Hirschsprung's disease (HSCD) and allied diseases.Methods Specimens were collected from resected colons of 6 HSCD,2 intestinal neuronal dysplasia (IND),14 HSCD + IND,4 hypoganglionosis patients,4 control cases and 5 cases of mucosal biopsies,from Shanghai Children's Medical Center.The expressions of protein gene product 9.5 (PGP 9.5),cathepsin D and calretinin (CR) were measured using irnmunohistochimostry (IHC).Results Ganglion cells and nerve fibers of submucosa and muscle layers were positively stained for PGP 9.5 in normal intestines.A lot of positive staining of fine nerve fibers was seen.Only ganglion cells were positively stained for cathepsin D.Staining of mature ganglion cells were much stronger than the immature cells.The nerve fibers showed no expression.The ganglion cells and the nearby nerve fibers were positively stained for CR,with moderate fine nerve fibers in the normal mucosa and submucosa.There were differential expressions of PGP 9.5,cathepsin D and CR among HSCD,IND and hypoganglionosis patients.Conclusions This study shows that CR IHC may be a useful technique in the pathological diagnosis of suction rectal biopsies for HSCD.PGP 9.5 and CR IHC stain are better than cathepsin D in recognizing imtmature ganglion cells.PGP 9.5,cathepsin D and CR IHC stain are useful adjunct techniques for HSCD,INDand hypoganglionsis. Key words: Hirschspurng disease;  Immunohistochemistry;  Pathology