Persistent bowel dysfunction after surgery for Hirschsprung's disease: A neuropathological perspective

Sanne J. Verkuijl*, Florian Friedmacher, Patrick N. Harter, Udo Rolle, Paul M. A. Broens

*Bijbehorende auteur voor dit werk

    Onderzoeksoutputpeer review

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    Samenvatting

    Hirschsprung's disease (HD) is a congenital disorder, characterized by aganglionosis in the distal part of the gastrointestinal tract. Despite complete surgical resection of the aganglionic segment, both constipation and fecal incontinence persist in a considerable number of patients with limited treatment options. There is growing evidence for structural abnormalities in the ganglionic bowel proximal to the aganglionosis in both humans and animals with HD, which may play a role in persistent bowel dysfunction. These abnormalities include: (1) Histopathological abnormalities of enteric neural cells; (2) Imbalanced expression of neurotransmitters and neuroproteins; (3) Abnormal expression of enteric pacemaker cells; (4) Abnormalities of smooth muscle cells; and (5) Abnormalities within the extracellular matrix. Hence, a better understanding of these previously unrecognized neuropathological abnormalities may improve follow-up and treatment in patients with HD suffering from persistent bowel dysfunction following surgical correction. In the long term, further combination of clinical and neuropathological data will hopefully enable a translational step towards more individual treatment for HD.

    Originele taal-2English
    Pagina's (van-tot)822-833
    Aantal pagina's12
    TijdschriftWorld journal of gastrointestinal surgery
    Volume13
    Nummer van het tijdschrift8
    DOI's
    StatusPublished - 27-aug.-2021

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