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Total intestinal aganglionosis

Known as: AGANGLIONOSIS, TOTAL INTESTINAL, Near-total intestinal aganglionosis 
A complete lack of ganglia in the intestine. This is an extremely severe form of Hirschsprung Disease.
National Institutes of Health

Related topics

Related topics

14 relations

Broader (9)

Acquired megacolonAganglionosis, ColonicColonic DiseasesCongenital Digestive System Disorder
Colon structure (body structure)Congenital DisordersGastrointestinal systemIntestines

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Review
2015
Review
2015
Knockout mouse models of Hirschsprung’s disease
PurposeHirschsprung’s disease (HSCR) is a developmental disorder of the enteric nervous system, which occurs due to the failure… 
2003
2003
Mutation analysis of the RET gene in total intestinal aganglionosis by wave DNA fragment analysis system.
BACKGROUND/PURPOSE Total intestinal aganglionosis (TIA) extending from the duodenum to the rectum is the most rare form of… 
2001
2001
Three-dimensional morphology of gut innervation in total intestinal aganglionosis using whole-mount preparation.
BACKGROUND Total intestinal aganglionosis (TIA) is a rare form of Hirschsprung's disease (HD). The aim of this study was to… 
2001
2001
A homozygous missense mutation in the tyrosine kinase domain of the RET proto-oncogene in an infant with total intestinal aganglionosis
Germline mutations of the RET proto-oncogene (RET), its ligand glial cell–derived neurotrophic factor (GDNF), and neurturin (NTN… 
2001
2001
Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel involvement.
BACKGROUND/PURPOSE Germline mutations of the RET-mediated or SOX10-mediated signaling pathway genes have been reported in total… 
Review
1999
Review
1999
Near Total Intestinal Aganglionosis with Extreme Short-Bowel Syndrome - A Difficult Surgical Dilemma
Forty cases of total or near-total intestinal agangliosis (NTIA) were described to date in the English literature. Most cases had… 
1997
1997
Germline mutation of the RET proto-oncogene in children with total intestinal aganglionosis.
1986
1986
Renal anomalies and agenesis associated with total intestinal aganglionosis.
Four patients with total aganglionosis of the intestine had renal abnormalities. Two patients had cortical subcapsular cysts, one… 
Review
1981
Review
1981
Total small bowel and colon aganglionosis.
A case of total small bowel and colon aganglionosis is presented and 11 previous cases are briefly reviewed. The relatively long… 
1977
1977
Total intestinal aganglionosis. An autosomal recessive condition?
A case of total intestinal aganglionosis in a sib of a previously recorded patient is presented. The number of cases now reported… 
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