title: 3-D technology used to accurately understand equine ileocolonic aganglionosis.
abstract: Ileocolonic aganglionosis (ICA) is the congenital and hereditary absence of neurons that constitute the enteric nervous system and has been described in various species including humans - Hirschsprung's disease - and horses - overo lethal white syndrome (OLWS). Hirschsprung's disease affects circa 1 in 5,000 live births. At best, this disease means an inability to absorb nutrients from food (humans). At worse, in horses, it always means death. Despite our general understanding of the functional mechanisms underlying ICA, there is a paucity of reliable quantitative information about the structure of myenteric and submucosal neurons in healthy horses and there are no studies on horses with ICA. In light of these uncertainties, we have used design-based stereology to describe the 3-D structure - total number and true size - of myenteric and submucosal neurons in the ileum of ICA horses. Our study has shown that ICA affects all submucosal neurons and 99% of myenteric neurons. The remaining myenteric neurons (0.56%) atrophy immensely, i.e. 63.8%. We believe this study forms the basis for further research, assessing which subpopulation of myenteric neurons are affected by ileocolonic aganglionosis, and we would like to propose a new nomenclature to distinguish between a complete absence of neurons - aganglionosis - and a weaker form of the disease which we suggest naming 'hypoganglionosis'. Our results are a step forward in understanding this disease structurally.
authors.affiliation: Laboratory of Experimental Neurogastroenterology, Universidade Paranaense, Umuarama, Brazil.
authors.forename: Eliane
authors.initials: E
authors.lastname: Muniz
authors.forename: Aliny A B
authors.initials: AA
authors.lastname: Lobo Ladd
authors.forename: Fernando V
authors.initials: FV
authors.lastname: Lobo Ladd
authors.forename: Andrea A P
authors.initials: AA
authors.lastname: da Silva
authors.forename: Fernanda V
authors.initials: FV
authors.lastname: Kmit
authors.forename: Alexandre S
authors.initials: AS
authors.lastname: Borges
authors.forename: Raffaella
authors.initials: R
authors.lastname: Teixeira
authors.forename: Lígia S L S
authors.initials: LS
authors.lastname: da Mota
authors.forename: Carla B
authors.initials: CB
authors.lastname: Belli
authors.forename: André L V
authors.initials: AL
authors.lastname: de Zoppa
authors.forename: Luis C L C
authors.initials: LC
authors.lastname: da Silva
authors.forename: Mariana P
authors.initials: MP
authors.lastname: de Melo
authors.forename: Antonio A
authors.initials: AA
authors.lastname: Coppi
mesh_terms: D000818:Animals; D002452:Cell Count; D005724:Ganglia / Q000473:pathology; D006627:Hirschsprung Disease / Q000175:diagnosis / Q000235:genetics / Q000662:veterinary*; D006734:Horse Diseases / Q000175:diagnosis* / Q000235:genetics; D006736:Horses; D007082:Ileum / Q000473:pathology; D021621:Imaging, Three-Dimensional / Q000379:methods*; D008297:Male; D009474:Neurons / Q000473:pathology; D009929:Organ Size