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El intestino ganglionar proximal en la enfermedad de Hirschsprung es fibrótico y rígido
Prisca C Obidike1, Britney A Hsu2, Chioma Moneme1
1Division of Pediatric Surgery, Department of Surgery, University of Virginia, Charlottesville, United States of America.
JCI insight
|February 10, 2026
Resumen
La enfermedad de Hirschsprung (HSCR, por sus siglas en inglés) involucra algo más que el intestino anglionar. Este estudio encontró un aumento de la fibrosis y la rigidez en el intestino ganglionar proximal de pacientes con HSCR y modelos de ratón, lo que contribuye a la disfunción intestinal persistente.
Área de la Ciencia:
- Gastroenterología y Gastroenterología.
- Biología del desarrollo Biología del desarrollo.
- Patología Patología Patología.
Sus antecedentes:
- La enfermedad de Hirschsprung (HSCR) es un trastorno intestinal congénito definido por la ausencia de ganglios nerviosos en el intestino distal.
- La disfunción intestinal posquirúrgica persiste en pacientes con HSCR, lo que sugiere anormalidades más allá del segmento anglionar resecado.
- El papel del intestino ganglionar proximal en la fisiopatología de la HSCR sigue siendo incompletamente entendido.
Objetivo del estudio:
- Para investigar posibles anomalías en el intestino ganglionar proximal de los pacientes con la enfermedad de Hirschsprung (HSCR).
- Para identificar las causas de la disfunción intestinal persistente en pacientes HSCR después de la cirugía.
- Para comparar el intestino ganglionar proximal en modelos de HSCR y pacientes humanos con controles.
Principales métodos:
- Utilizó el modelo de ratón Ednrb-null para HSCR.
- Se analizaron muestras de tejido de pacientes con HSCR y controles de edad y localización.
- Se evaluó la rigidez intestinal, la fibrosis y la expresión génica de la matriz extracelular (ECM).
Principales resultados:
- El colon ganglionar proximal en ratones HSCR mostró una mayor rigidez y fibrosis en comparación con sus compañeros de camada de tipo salvaje.
- Los pacientes humanos con HSCR presentaron una fibrosis submucosa significativamente mayor en el intestino ganglionar proximal frente a los controles.
- Se observó una expresión desregulada de genes relacionados con el ECM en el intestino ganglionar proximal de ratones HSCR.
Conclusiones:
- El aumento de la fibrosis y la rigidez en el intestino ganglionar proximal puede contribuir a la disfunción intestinal persistente en HSCR.
- La fisiopatología de la enfermedad de Hirschsprung se extiende al intestino ganglionar proximal, no solo al segmento anglionar.
- Los hallazgos sugieren que la HSCR es un trastorno más complejo que implica anormalidades intestinales generalizadas.
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