engleski

Prognosis of the Hirschsprung's disease allied disorder or neuronal dysplasia: the value of ganglia/nerve-fibers ratio

Introduction: The Hirschsprung's disease allied disorder (HAD) pathohistological findings of abnormal myenteric nervous pexus vary depending of the quantity of ganglia, therefore, the complete absence of ganglia (the real Hirschsprung), hypoganglionosis, and hyperganglionosis which is considered as the colonic neuronal dysplasia. The lack of ganglia cells and increased and abnormaln proliferation of mucosal nerve fibers follow the congenital dismotility problems of the gastrointestinal tract (GIT). Aims & Methods: The aim of our study was to determine a possible connection between ganglia/nerve-fibers ratio and the clinical outcome in Hirschsprung's disease allied disorders (HAD) predicting possible recovery of patients even before treatment. We have followed the recovery of 15 patients treated and compared the clinical outcome with the histopathological findings of ganglia and nerve fibers. Sections of the full-thickness bowel specimens, obtained from 15 patients with HAD, were treated with antibodies for neurofilament protein (NF), marking ganglia and for S-100, marking nerve fibers. Results: Five patients undergoing surgery were found to have low ganglia/nerve-fibers ratio while 9 out of 10 patients not showing the need for surgery had higher ganglia/nerve-fibers ratio. Conclusion: We propose that the ganglia/nerve-fibers ratio can be used to predict the clinical outcome and long term prognosis as well as the need for invasive procedures in patients diagnosed with HAD.

Hirschsprung's disease allied disorder (HAD) ; prognosis ; ganglia ; nerve

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