Interstitial cells of Cajal are decreased in patients with gastroschisis associated intestinal dysmotility

Abstract

Intestinal samples from infants with GAID who underwent stoma formation demonstrated fewer ICC than controls. There was no improvement or cell recovery during the study period. The ability to modulate ICC may have significant implications for the management of GAID.

Risk factors and outcomes of tapering surgery for small intestinal dilatation in pediatric short bowel syndrome

“…These distinct etiologies may explain the observed differences in the need for tapering surgery between SBS subgroups. Dysmotility is common also in gastroschisis, where the exposure of intestines to amniotic fluid, formation of inflammatory peel, and intramural histopathology including smooth muscle hyperplasia and decreased number of Cajal cells are considered responsible for abnormal motility [33][34][35][36][37][38]. Possibly because of small number of children with isolated gastroschisis, they showed no increased risk for tapering in the present study.…”

“…Four patients with both SBA and gastroschisis were analyzed in the SBA group. Median residual small bowel length was 40 (25-60) cm while colon length was 32 (24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40)(41) cm, corresponding to 25% (17%-44%) and 93% (61%-100%) of age-adjusted reference values, respectively [28]. The ICV was missing in 28 (47%) and no ileum was remaining in 27 (45%).…”

How to Conduct Clinical Trials in Children: A Tutorial

“…Gastric emptying rates are influenced by pediatric comorbidities, including prematurity, gastroesophageal reflux disease, congenital heart disease, and type I diabetes . Intestinal dysmotility accompanies gastroschisis, Hirschsprung's disease, and infantile pyloric stenosis . Children with amino acid n‐acyltransferase deficiency, or those receiving chronic total parenteral nutrition, may experience alterations in protein binding, whereas renal filtration is expected to be compromised in children with nephropathies, such as focal segmental glomerulosclerosis …”

“…33 Intestinal dysmotility accompanies gastroschisis, Hirschsprung's disease, and infantile pyloric stenosis. [34][35][36] Children with amino acid n-acyltransferase deficiency, or those receiving chronic total parenteral nutrition, may experience alterations in protein binding, whereas renal filtration is expected to be compromised in children with nephropathies, such as focal segmental glomerulosclerosis. 10 For anticipated scenarios, such as those described above, special consideration may need to be given to assay sensitivity and specimen volumes.…”

Pharmacokinetic Considerations when Prescribing in Children

“…Gastric emptying times are demonstrated to be slower in Type I diabetic patients and intestinal dysmotility is prominent in pediatric patients who've suffered from gastroschisis, Hirschsprung disease or infantile pyloric stenosis where there may be abnormal or altered expression of the interstitial 'pacemaker' cells [85][86][87]. Protein binding may be altered in children with higher levels of circulating displacers as in children with amino acid n-acyltransferase deficiency, which manifests with defects within the bile acid synthesis or patients on chronic total parenteral nutrition wherein cholestasis is a frequently encountered problem.…”