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.
© 2013 S. Karger AG, Basel.
Publication Date: 2013-07-25 PubMed ID: 23886660DOI: 10.1159/000353218Google Scholar: Lookup
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- Journal Article
- Research Support
- Non-U.S. Gov't
- Anatomy
- Diagnosis
- Disease control
- Disease Diagnosis
- Disease Etiology
- Disease Management
- Disease Outbreaks
- Disease Prevention
- Disease Surveillance
- Disease Transmission
- Disease Treatment
- Equine Diseases
- Equine Health
- Equine Science
- Genetics
- Horses
- Infection
- Infectious Disease
- Veterinary Medicine
- Veterinary Research
- Veterinary Science
Summary
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This research explores 3-D modeling to accurately understand and quantify the structure of impacted neurons in horses with ileocolonic aganglionosis (ICA), a lethal genetic disorder. The study proposes a new term, ‘hypoganglionosis’, to distinguish between cases with a complete absence of neurons and a less severe form of ICA.
Understanding Ileocolonic Aganglionosis
- The research focuses on a congenital and inherited absence of neurons in the enteric nervous system known as Ileocolonic Aganglionosis (ICA).
- ICA has been observed in various species, including humans, where it is termed Hirschsprung’s disease, and horses, where it is known as overo lethal white syndrome (OLWS).
- The disease can impede nutrient absorption from food in humans and is always lethal in horses.
The Research Gap
- Despite general understanding of ICA’s functional mechanisms, there is a lack of quantitative knowledge on the structure of myenteric and submucosal neurons in healthy horses.
- No prior studies have specifically focused on horses with ICA.
The Study: 3-D Modelling for Accurate Analysis
- To address these ongoing uncertainties, the researchers used design-based stereology to describe the 3-D structure, including the total number and true size, of myenteric and submucosal neurons in horses affected by ICA.
- The study found that ICA impacts all submucosal neurons and 99% of myenteric neurons. The remaining myenteric neurons went through severe atrophy, up to 63.8%.
Proposal of a New Terminology
- The researchers propose a new terminology, ‘hypoganglionosis’, to categorize a less severe form of ICA. This terminology serves to distinguish between a complete absence of neurons (aganglionosis) and a less severe form of ICA.
Implications and Future Direction
- The study lays the foundation for future research into determining which subpopulation of myenteric neurons is affected by ICA.
- This research represents progress towards structurally understanding this lethal disease.
Cite This Article
APA
Muniz E, Lobo Ladd AA, Lobo Ladd FV, da Silva AA, Kmit FV, Borges AS, Teixeira R, da Mota LS, Belli CB, de Zoppa AL, da Silva LC, de Melo MP, Coppi AA.
(2013).
3-D technology used to accurately understand equine ileocolonic aganglionosis.
Cells Tissues Organs, 198(2), 160-168.
https://doi.org/10.1159/000353218 Publication
Researcher Affiliations
- Laboratory of Experimental Neurogastroenterology, Universidade Paranaense, Umuarama, Brazil.
MeSH Terms
- Animals
- Cell Count
- Ganglia / pathology
- Hirschsprung Disease / diagnosis
- Hirschsprung Disease / genetics
- Hirschsprung Disease / veterinary
- Horse Diseases / diagnosis
- Horse Diseases / genetics
- Horses
- Ileum / pathology
- Imaging, Three-Dimensional / methods
- Male
- Neurons / pathology
- Organ Size
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