TY  - JOUR
A1  - Verkuijl, Sanne J.
A1  - Friedmacher, Florian Michael
A1  - Harter, Patrick Nikolaus
A1  - Rolle, Udo
A1  - Broens, Paul M. A.
T1  - Persistent bowel dysfunction after surgery for Hirschsprung's disease: a neuropathological perspective
T2  - World journal of gastrointestinal surgery
N2  - 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.
KW  - Hirschsprung disease
KW  - Aganglionosis
KW  - Proximal
KW  - Ganglionic
KW  - Constipation
KW  - Incontinence
Y1  - 2021
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/62740
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-627405
SN  - 1948-9366
VL  - 13
IS  - 8
SP  - 822
EP  - 833
PB  - Beijing Baishideng BioMed Scientific Co.
CY  - Beijing
ER  -