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ISSN: 0031-3939
Pediatria Polska - Polish Journal of Paediatrics
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6/2019
vol. 94
 
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abstract:
Review paper

Hirschsprung disease and other intestinal neuropathies in children

Katarzyna Zdanowicz
1
,
Joanna Reszeć
2
,
Ewa Dzienis-Koronkiewicz
3
,
Adam Hermanowicz
3
,
Dariusz M. Lebensztejn
1

  1. Department of Paediatrics, Gastroenterology, Hepatology, Nutrition, and Allergology, Medical University of Bialystok, Bialystok, Poland
  2. Department of Medical Pathomorphology, Medical University of Bialystok, Bialystok, Poland
  3. Department of Paediatric Surgery and Urology, Medical University of Bialystok, Bialystok, Poland
Pediatr Pol 2019; 94 (6): 368–372
DOI: https://doi.org/10.5114/polp.2019.92954
Online publish date: 2019/12/31
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Proper intestinal motility depends on interaction between muscles, nerve cells, and tendinous connective tissue of muscularis propria. Intestinal motility disorders refer to varied intestinal neuromuscular pathologies, including enteric neuropathies. The most common symptoms of these diseases are delayed passage of meconium in newborns and chronic constipation in infants and older children. If organic causes of clinical features are detected, a further multidisciplinary team approach for the management of these patients is recommended. Entities discussed in this review include Hirschsprung disease, hypoganglionosis, intestinal neuronal dysplasia, ganglioneuromatosis, and chronic intestinal pseudo-obstruction. Emphasis is given to the clinical symptoms and diagnostic features that distinguish these conditions enabling faster diagnosis and appropriate treatment.
keywords:

chronic constipation, intestinal motility disorder, delayed passage of meconium, Hirschsprung disease