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[Hirschsprung disease].

D Pellerin1

  • 1Clinique chirurgicale infantile, hôpital Necker-Enfants-Malades, Paris.

Bulletin De L'Academie Nationale De Medecine
|November 1, 1991
PubMed
Summary
This summary is machine-generated.

Hirschsprung's disease knowledge has evolved, transforming congenital megacolon concepts. Early colonic decompression remains crucial for preventing lethal enterocolitis in Hirschsprung's disease patients.

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Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Developmental Biology

Context:

  • Over 40 years of research have significantly advanced understanding of Hirschsprung's disease.
  • Personal experience with 500 patients at Hôpital des Enfants Malades, Paris, informs this review.
  • The study integrates findings from Radiology, Manometry, Histochemistry, and Immunocytochemistry.

Purpose:

  • To review the evolution of knowledge regarding Hirschsprung's disease.
  • To highlight contributions to understanding its etiopathogenesis.
  • To emphasize critical clinical management strategies.

Summary:

  • Advances in molecular biology illuminate neural crest cell migration and the disease's pathogenesis.
  • Pathological associations suggest a genetic basis for neuro-transducer defects in Hirschsprung's disease.

Related Experiment Videos

  • Despite scientific progress, the risk of enterocolitis necessitates prompt clinical intervention.

    • Impact:

    • This work reframes the understanding of congenital megacolon.
    • It underscores the importance of early diagnosis and management of Hirschsprung's disease.
    • Highlights the ongoing need for research into the genetic underpinnings of Hirschsprung's disease.