Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells

Autor(es)

Terra, Simone Antunes ^[1] ; Goncalves, Anderson Cesar ^[2] ; Lourencao, Pedro Luiz Toledo de Arruda ^[2] ; Rodrigues, Maria Aparecida Marchesan ^[1]

Afiliação do(s) autor(es)

^[1] Sao Paulo State Univ UNESP, Dept Pathol, Fac Med, BR-18618687 Botucatu, SP - Brazil

^[2] Sao Paulo State Univ UNESP, Botucatu Med Sch, Dept Surg & Orthoped, Ave Prof Montenegro, BR-18618970 Botucatu, SP - Brazil

• Tipo de documento: Artigo de Revisão
• Fonte: WORLD JOURNAL OF GASTROENTEROLOGY; v. 27, n. 44 NOV 28 2021.
• Citações Web of Science: 0

Resumo

Intestinal neuronal dysplasia type B (IND-B) is a controversial condition among gastrointestinal neuromuscular disorders. Constipation is its most common clinical manifestation in patients. Despite intense scientific research, there are still knowledge gaps regarding the diagnostic criteria for IND-B in the histopathological analysis of rectal biopsies. The guidelines published in the past three decades have directed diagnostic criteria for quantifying the number of ganglion cells in the nervous plexus of the enteric nervous system. However, it is very complex to distinguish numerically what is pathological from what is normal, mainly because of the difficulty in determining a reliable control group composed of healthy children without intestinal symptoms. Thus, a series of immunohistochemical markers have been proposed to assist in the histopathological analysis of the enteric nervous system. Several of these markers facilitate the identification of other structures of the enteric nervous system, in addition to ganglion cells. These structures may be related to the etiopathogenesis of IND-B and represent new possibilities for the histopathological diagnosis of this disease, providing a view beyond the number of ganglion cells. This review critically discusses the aspects related to the disease definitions and diagnostic criteria of this organic cause of constipation. (AU)

• Processo FAPESP: 17/26205-9 - Displasia Neuronal Intestinal na Infância: Critérios diagnósticos e Marcadores Imuno-histoquímicos
• Beneficiário: Maria Aparecida Marchesan Rodrigues Kobayasi
• Modalidade de apoio: Auxílio à Pesquisa - Regular