engleski

Prognoza poremećaja srodnog Hirschprungovoj bolesti: vrijednost omjera ganglija i živčanih vlakana

Hirschsprung's disease allied disorder (HAD) covers a large variety of different pathological findings of abnormal myenteric nervous plexus manifesting as the congenital motor dysfunction of the intestine. The difference varies from complete absence of ganglia (M. Hirschprung), hypoganglionosis to the normal quantity and distribution of ganglia. The state of hyperganglionosis is considered as the colonic neuronal dysplasia and also is in the group of HAD. The bowel shows the relative lack of ganglia cells together with increased and abnormal proliferation of mucosal nerve fibres. We have followed 15 patients treated and compared the clinical outcome with the histopathological findings of ganglia and nerve fibres. Objective: To estimate a possible connection between ganglia-nerve fibres (G/NF) ratio and the clinical outcome in HAD predicting a possible good prognosis of patients even before treatment. Methods: Full thickness of the bowel-wall specimens were obtained from 15 patients with HAD. Sections were treated with antibodies against neurofilament protein (NF), marking ganglia and S-100, marking nerve fibres. Median value of ganglia was estimated as a result of the number of ganglia in three hot-spots in high-power field magnification (X200).The same method was used for estimating a median value of nerve-fibers. Results: One out of 15 patients suggested Hirschprung disease while we found 14 of them more likely to be diagnosed with HAD. 5 patients underwent surgery were found to have low G/NF ratio while 9 out of 10 patients without underwent surgery had higher G/NF ratio. Conclusion: We propose that the ganglia-nerve-fiber (G/NF) ratio can be used to predict the clinical outcome and long term prognosis as well as the need for invasive proceedures in patients diagnosed with HAD. High G/NF ratio could be a predictor of good clinical outcome and decreased need for surgery, while lower G/NF ratio shows poorer prognosis and increases the need for surgical treatment. The number of patients is small for the reliable statistical analysis.

Poremećaj srodan Hirschprungovoj bolesti ; prognoza ; ganglij ; živac

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