Hirschsprung disease (Q46328): Difference between revisions
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Esta é uma anomalia do desenvolvimento que afeta o trato intestinal, caracterizada pela ausência congênita de células ganglionares mioentéricas (aganglionose) em um segmento do intestino grosso. Devido à ausência de inervação intrínseca das camadas musculares do segmento afetado, ocorre perda da função motora. Isso resulta em um cólon anormalmente grande ou dilatado (megacólon congênito) com oclusão intestinal ou constipação. Essa condição se torna evidente logo após o nascimento. | |||
| description / en | description / en | ||
This is a developmental anomaly affecting the intestinal tract characterised by congenital absence of myenteric ganglion cells (aganglionosis) in a segment of the large bowel. Due to the absence of intrinsic innervation of the muscle layers of the affected segment, there is a loss of motor function. This results in an abnormally large or dilated colon (congenital megacolon) with intestinal occlusion or constipation. This condition becomes evident shortly after birth. | |||
Revision as of 14:50, 13 August 2026
This is a developmental anomaly affecting the intestinal tract characterised by congenital absence of myenteric ganglion cells (aganglionosis) in a segment of the large bowel. Due to the absence of intrinsic innervation of the muscle layers of the affected segment, there is a loss of motor function. This results in an abnormally large or dilated colon (congenital megacolon) with intestinal occlusion or constipation. This condition becomes evident shortly after birth.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | LB16.1 |
||
| English | Hirschsprung disease |
This is a developmental anomaly affecting the intestinal tract characterised by congenital absence of myenteric ganglion cells (aganglionosis) in a segment of the large bowel. Due to the absence of intrinsic innervation of the muscle layers of the affected segment, there is a loss of motor function. This results in an abnormally large or dilated colon (congenital megacolon) with intestinal occlusion or constipation. This condition becomes evident shortly after birth. |
