Altered Distribution of Interstitial Cells of Cajal in Normoganglionic and Transitional Zone of Hirschsprung Disease and Their Clinical Significance

Janković, Radmila; Sindjic-Antunovic, Sanja; Lukač, Marija; Vujović, Dragana; Jevtić, Jovan; Skender-Gazibara, Milica

doi:10.5457/p2005-114.251

Cited by 4 publications

(2 citation statements)

References 28 publications

(59 reference statements)

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“…Označene su i kao pejsmejker ćelije (8). Kahalove, kao i glatko-mišićne ćelije, nastaju od mezenhima creva, a smatra se da prekursorske ćelije ENS utiču na njihovu diferencijaciju (38,39).…”

Section: Migration Proliferation and Differentiationunclassified

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Origin of enteric nervous system cells and migration pathways during embryonic development

Đuknić¹,

Puškaš²,

Labudović‐Borović³

et al. 2022

Zdravstvena zaštita

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The enteric nervous system (ENS) is represented by a complex network of neurons, glial and other cells within the wall of the digestive tract. ENS is responsible for numerous, vital functions in our body. Thus, ENS regulates motility of the digestive tract, secretion into the intestinal lumen, exchange of fluid and electrolytes through the mucosa, as well as mucosal perfusion. In order to perform these important functions, proper embryonic development of ENS is necessary. ENS cells are derived from precursor cells of the neural crest (NCCs - neural crest cells). Two cell populations that contribute to the largest number of future ENS cells are the vagal and sacral NCCs. Vagal NCCs enter the primitive gut tube in the region of the future esophagus (foregut), and begin their migration, through the midgut towards the hindgut and the future anal region. Sacral NCCs enter the hindgut region following the extrinsic nerve fibers and continue their migration rostrally, towards vagal NCCs. Along with the migration process, these cells undergo other important processes, such as proliferation, neuro-glial differentiation, gangliogenesis, axonal pathway formation and synaptogenesis. All these processes are strictly regulated by numerous signaling pathways, which are still being actively researched. Modern lineage tracing and other technologies, that enabled following of individual precursor cells through their development pathways, will significantly contribute to the better understanding of development of ENS. This may have repercussions in improving the diagnosis and treatment of some developmental (Hirschsprung disease) and other ENS disorders.

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Section: Migration Proliferation and Differentiationunclassified

“…They were marked as pacemaker cells (8). Interstitial cells of Cajal, as well as smooth muscle cells, are derived from the intestinal mesenchyme, and it is deemed that ENS precursor cells influence their differentiation (38,39).…”

Section: Konflikt Interesamentioning

confidence: 99%

Origin of enteric nervous system cells and migration pathways during embryonic development

Đuknić¹,

Puškaš²,

Labudović‐Borović³

et al. 2022

Zdravstvena zaštita

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Update on the Pathogenesis of the Hirschsprung-Associated Enterocolitis

Zhang

et al. 2023

IJMS

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Despite the significant progress that has been made in terms of understanding the pathophysiology and risk factors of Hirschsprung-associated enterocolitis (HAEC), the morbidity rate has remained unsatisfactorily stable, and clinical management of the condition continues to be challenging. Therefore, in the present literature review, we summarized the up-to-date advances that have been made regarding basic research on the pathogenesis of HAEC. Original articles published between August 2013 and October 2022 were searched in a number of databases, including PubMed, Web of Science, and Scopus. The keywords “Hirschsprung enterocolitis”, “Hirschsprung’s enterocolitis”, “Hirschsprung’s-associated enterocolitis”, and “Hirschsprung-associated enterocolitis” were selected and reviewed. A total of 50 eligible articles were obtained. The latest findings of these research articles were grouped into gene, microbiome, barrier function, enteric nervous system, and immune state categories. The present review concludes that HAEC is shown to be a multifactorial clinical syndrome. Only deep insights into this syndrome, with an accrual of knowledge in terms of understanding its pathogenesis, will elicit the necessary changes that are required for managing this disease.

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Histopathological findings in enteric nervous plexuses in children with intestinal motility disorders: A single center experience

Janković,

Sinđić-Antunović,

Đuknić

et al. 2024

Medicinska istraživanja

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Introduction/Aim: The aim of the study was to determine the frequency of various histopathological findings in biopsies of children with intestinal hypomotility and the incidence and characteristics of Hirschsprung disease (HD). Methods: Biopsies of colon and rectum taken due to intestinal hypomotility and chronic constipation at the Department of Pediatric Surgery of the University Children's Hospital in Belgrade over the 10-year period (from 2009 to 2018) were reviewed using pathology reports from the archive of the Institute of pathology, Faculty of Medicine, University of Belgrade. Results: A total of 287 patients with intestinal motility disorder were identified, with 554 biopsy samples. Of the total number of patients, 56% (161/287) were without any morphological changes in enteric nervous system (ENS). The most common histopathological findings were HD (69/287; 24%) and immaturity of ganglion cells (29/287; 10%). Isolated hypoganglionosis of ENS was found in 5 (2%) cases. Heterotopia of ganglion cells was the only finding in 8 (3%) cases. Rare causes of intestinal dysmotility were: eosinophilic proctitis/colitis (EPC) (4/287), neuronal intestinal dysplasia B (2/287), unclassified disganglionoses (3/287). Rectosigmoid variant of HD was the most frequent HD variant (80.3%). Acetylcholinesterase method and immunohistochemical staining were used in 19.5% cases. Conclusions: HD and immaturity of ganglion cells were the most common pathological findings in ENS of constipated children. It is important to differentiate EPC from other lesions of enteric plexuses due to different natural history and therapy.

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Altered Distribution of Interstitial Cells of Cajal in Normoganglionic and Transitional Zone of Hirschsprung Disease and Their Clinical Significance

Cited by 4 publications

References 28 publications

Origin of enteric nervous system cells and migration pathways during embryonic development

Origin of enteric nervous system cells and migration pathways during embryonic development

Update on the Pathogenesis of the Hirschsprung-Associated Enterocolitis

Histopathological findings in enteric nervous plexuses in children with intestinal motility disorders: A single center experience

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