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

• Grant number: 14/04271-1
• Support Opportunities: Regular Research Grants
• Start date: March 01, 2015
• End date: February 28, 2017
• Field of knowledge: Health Sciences - Medicine - Surgery
• Principal Investigator: Pedro Luiz Toledo de Arruda Lourencao
• Grantee: Pedro Luiz Toledo de Arruda Lourencao
• Host Institution: Faculdade de Medicina (FMB). Universidade Estadual Paulista (UNESP). Campus de Botucatu. Botucatu , SP, Brazil
• Associated researchers: Maria Aparecida Marchesan Rodrigues Kobayasi

Abstract

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). (AU)

Scientific publications

(References retrieved automatically from Web of Science and SciELO through information on FAPESP grants and their corresponding numbers as mentioned in the publications by the authors)

JOZALA, DEBORA RODRIGUES; DE FARIA OTIVEIRA, ISABELLE STEFAN; PAIVA ORTOLAN, ERIKA VERUSKA; DE OLIVEIRA JUNIOR, WILSON ELIAS; COMES, GIOVANA TUCCILLE; GRANADO CASSETTARI, VANESSA MELLO; SELF, MARIETTA MARIE; TOLEDO DE ARRUDA LOURENCAO, PEDRO LUIZ. Tradução para o português (Brasil), adaptação transcultural e avaliação da reprodutibilidade da Escala de Bristol para Consistência de Fezes modificada para crianças. Jornal de Pediatria, v. 95, n. 3, p. 321-327, MAY-JUN 2019. (14/04271-1, 15/03649-3)

TOLEDO DE ARRUDA LOURENCAO, PEDRO LUIZ; PAIVA ORTOLAN, ERIKA VERUSKA; MINELLI ROSA, LAURA LUIZA; ANGELINI, MARCOS CURCIO; GRANADO CASSETTARI, VANESSA MELLO; TERRA, SIMONE ANTUNES; MARCHESAN RODRIGUES, MARIA APARECIDA. What should be the treatment for intestinal neuronal dysplasia type B? A comparative long-term follow-up study. JOURNAL OF PEDIATRIC SURGERY, v. 56, n. 9, p. 1611-1617, SEP 2021. (14/04271-1)

ANGELINI, MARCOS C.; SILVA, ALANA MAIA E.; FELIX, TAINARA F.; LAPA, RAINER M. L.; TERRA, SIMONE A.; RODRIGUES, MARIA A. M.; ORTOLAN, ERIKA V. P.; REIS, PATRICIA P.; LOURENCAO, PEDRO L. T. A.. Identification of potential molecular pathogenesis mechanisms modulated by microRNAs in patients with Intestinal Neuronal Dysplasia type B. SCIENTIFIC REPORTS, v. 9, NOV 27 2019. (14/04271-1, 15/03664-2, 14/00367-4)