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Clinical evolution of patients with neuronal intestinal dysplasia: which Treatament is better?

Grant number: 14/03710-1
Support type:Scholarships in Brazil - Scientific Initiation
Effective date (Start): July 01, 2014
Effective date (End): June 30, 2016
Field of knowledge:Health Sciences - Medicine
Principal Investigator:Pedro Luiz Toledo de Arruda Lourencao
Grantee:Laura Luiza Minelli Rosa
Home Institution: Faculdade de Medicina (FMB). Universidade Estadual Paulista (UNESP). Campus de Botucatu. Botucatu , SP, Brazil

Abstract

Introduction: Intestinal Neuronal Dysplasia (IND) is a gastrointestinal neuromuscular disease, characterized by complex changes in the enteric nervous system. Its diagnosis depends on the histopathological analysis of rectal biopsies. It is defined by at least 15% of submucosal giant ganglia (with more than 8 ganglion cells each), in 30 histological sections to be examined. The pathogenesis of NID is still unclear. Several hypotheses are discussed, but none of them is widely accepted. It is hypothesized that NID can occur from a primary alteration, during embryological development of neural crest tissues, or from a secondary alteration, due to obstructive or inflammatory bowel diseases. The two therapeutic modalities for NID are the conservative medical treatment and the surgical treatment. However, their results are quite discordant and follow-up studies are rare and with a limited number of patients, which precludes conclusions regarding the best treatment modalities. In this context, long-term follow up series are necessary to investigate what is the best therapeutic modality for NID. Objective: To evaluate the clinical outcome of patients with Intestinal Neuronal Dysplasia, considering the two therapeutic modalities applied. Methods: This is a longitudinal, observational, retrospective and comparative follow-up study. Firstly, we will review the records of 63 patients aged 0-15 years with NID, attended at Botucatu Medical School's Hospital, in the period of 1998 to 2010. In the second phase of the study, prospectively, patients will be invited to a semi-structured interview in which questionnaires will be applied to investigate their current clinical status, focusing on bowel habit, fecal incontinence, stool consistency and overall quality of life. All patients will be informed of the purpose of the survey and will sign an informed consent term. The project was approved by the Ethics Committee of the Botucatu Medical School UNESP (Protocol number 211 156).