The evaluation of meconium disease by distribution of cathepsin D in intestinal ganglion cells

Meconium disease (MD) results in intestinal obstruction in the neonate wher e tenacious meconium is found in the distal ileum and proximal colon. The o bstructive symptoms improve at several days of age after some of the meconi um is passed. We observed premature infants with MD who underwent ileostomy for intestinal obstruction due to tenacious meconium. Afterward, meconium was passed well and the clinical symptoms improved. After closing the ileos tomy, growth and defecation became normal. The MD in our cases was document ed by histologic changes in the maturation of ganglion cells observed at th e time of ileostomy creation and closure. For an objective evaluation of th e maturation of intestinal ganglion cells (IGC), we attempted to distinguis h immature from mature cells by the expression of cathepsin D. We examined the distribution of cathepsin D in IGC in patients with MD to test the hypo thesis that ganglion-cell immaturity might be related to MD. In ganglion ce lls at the time of ileostomy, cathepsin D was detected in the perinuclear c ytoplasm (immature staining pattern), while at the time of ileostomy closur e it was detected in intense granules throughout the cytoplasm (mature stai ning pattern). We propose that it would be possible to evaluate the maturat ion of IGC by the intracellular distribution of cathepsin D in MD and sugge st that immaturity of IGC might be the cause of MD.