Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis

Collins, Margaret H.; Azzarelli, Biagio; West, Karen W.; Chong, Sonny K. F.; Maguiness, Karen; Stevens, John C.

doi:10.1016/s0022-3468(96)90418-2

Cited by 13 publications

(7 citation statements)

References 24 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These animals developed hypertrophic gut walls proximal to the stenosis. Although clinical reports of HHG are uncommon, it has been documented in patients with idiopathic sigmoid stenosis [17], colonic strictures secondary to therapeutic delayed-release high-dose pancreatic enzyme supplements for cystic fibrosis [21], intraluminal mucosal webs of the ileum [22], and in association with Hirschsprung disease [23,24]. This report further expands the list.…”

Section: Discussionmentioning

confidence: 70%

Hypercellular/Heterotopic Ganglia in Omphalomesenteric Duct Remnants

Collins

Coventry

et al. 2001

Pediatr Dev Pathol

View full text Add to dashboard Cite

Omphalomesenteric duct remnants (ODR) including Meckel diverticula often present with symptoms of bowel obstruction. Their histologic features are varied and include heterotopic gastrointestinal mucosa and/or pancreatic tissue within the wall. Abnormalities of the submucosal plexus of Meissner, however, have not been documented in the literature. Thus, we have examined a number of ODR for evidence of ganglion abnormalities. Fifty-three cases of ODR were retrieved from our archives, along with 25 nonduodenal small bowel control specimens obtained from autopsies of patients without clinical or pathologic evidence of enteropathy. Histologic criteria for the diagnosis of abnormal hypercellular/heterotopic ganglia (HHG) in ODR were defined as a single submucosal ganglion/plexus containing > 10 neurons, or > 5 submucosal ganglions per high-power (x 40) field, or heterotopic ganglion cells within the muscularis mucosa or the lamina propria. HHG, histologically indistinguishable from intestinal neuronal dysplasia type B, were found in more than half of the ODR (ODR: 51.9%, n = 50 vs. control: 4%, n = 25, P = 3.6 x 10(-6), particularly those excised for obstructive complications (ODR with acute abdomen: 65.7%, n = 35, vs. ODR without acute abdomen: 33.3%, n = 15, P = 0.035). HHG were present in equal numbers of inflamed and noninflamed ODR (inflamed: 53.6%, n = 28, vs. noninflamed: 59.1%, n = 22, P = 0.70). A similar incidence was found in ODR with heterotopia and without (with heterotopia: 61.1%, n = 18, vs. without: 53.1%, n = 32, P = 0.59). ODR frequently contained HHG histomorphologically similar to those found in intestinal neuronal dysplasia, type B (IND-B). The overrepresentation of HHG in symptomatic ODR patients suggests an association with bowel obstruction. The data did not demonstrate a relationship with either inflammation or heterotopia.

show abstract

Section: Discussionmentioning

confidence: 70%

Hypercellular/Heterotopic Ganglia in Omphalomesenteric Duct Remnants

Collins

Coventry

et al. 2001

Pediatr Dev Pathol

View full text Add to dashboard Cite

show abstract

“…Response to injury could also explain the IND-like hyperplasia of submucosal ganglia observed in intestinal allografts 47 and fibrosing colonopathy. 48 Inherent in models of acquired IND B is the hypothesis that enteric neural progenitors persist after ganglia are formed and can be recruited later to form submucosal ganglion cells. During embryologic development, submucosal ganglion cells form from the descendants of progenitors located in the myenteric plexus, which migrate centrifugally toward the bowel lumen.…”

Section: Pathogenesis Of Ind Bmentioning

confidence: 99%

Intestinal Neuronal Dysplasia Type B: An Updated Review of a Problematic Diagnosis

Kapur

Reyes‐Múgica

2018

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

Context.-Intestinal neuronal dysplasia type B (IND B) is a controversial histopathologic phenotype that has been associated with intestinal dysmotility, either as an isolated condition or in conjunction with established pathologic disorders (eg, Hirschsprung disease). Many factors contribute to the debate over the existence and/or clinical significance of IND B, including a large body of published data based on inconsistent diagnostic criteria and methods, which have fostered many unwarranted conclusions that lack sufficient scientific basis.Objective.-To critically analyze existing published data regarding IND B to provide supporting evidence-based diagnostic practice and to stimulate necessary and scientifically sound research.Data Sources.-This update focuses on published literature related to the pathology of IND B because without a reliable pathologic diagnosis, studies of epidemiology, pathogenesis, natural history, management, and outcome are all suspect. Problems with existing data are identified explicitly with suggestions as to how future investigations should be designed and evaluated to better understand this entity.Conclusions.-Inconsistencies in diagnostic criteria and methods used to define IND B justifiably encumber the universal acceptance of IND B as a neuropathologic etiology for intestinal dysmotility. IND B will remain a controversial diagnosis until rigorous, well-controlled scientific studies are conducted to establish reproducible and reliable diagnostic criteria that reliably translate from one laboratory to another.

show abstract

“…More considerable literature has been devoted to IND B, in part because public debate exists as to the existence and/or significance of this pathological entity71, 73–77. IND B can occur as an isolated condition, in association with Hirschsprung's disease78, 79, or in conjunction with other forms of obstructive or pseudo‐obstructive intestinal pathology, such as colonic strictures or cystic fibrosis71, 80–84. Patients with isolated IND B may present with clinical findings identical to IND A, or as older infants with chronic constipation.…”

Section: Hyperganglionosismentioning

confidence: 99%

Neuropathology of paediatric chronic intestinal pseudo-obstruction and related animal models

Kapur

2001

J. Pathol.

View full text Add to dashboard Cite

Chronic intestinal pseudo-obstruction (CIP) in paediatric patients is due to heterogeneous aetiologies that include primary disorders of the enteric nervous system. These conditions are poorly delineated by contemporary diagnostic approaches, in part because the complex nature of the enteric nervous system may shelter significant physiological defects behind subtle or quantitative anatomical changes. Until recently, relatively few experimental animal models existed for paediatric CIP. However, the availability of rodent models, particularly novel mutants created in the last few years by genetic manipulations, has brought unprecedented opportunities to investigate molecular, cellular, physiological, and histological details of enteric neuropathology. Information gleaned from studies of these animals is likely to change diagnostic and therapeutic approaches to paediatric CIP and related conditions.

show abstract

Neuropathy and vasculopathy in colonic strictures from children with cystic fibrosis

Cited by 13 publications

References 24 publications

Hypercellular/Heterotopic Ganglia in Omphalomesenteric Duct Remnants

Hypercellular/Heterotopic Ganglia in Omphalomesenteric Duct Remnants

Intestinal Neuronal Dysplasia Type B: An Updated Review of a Problematic Diagnosis

Neuropathology of paediatric chronic intestinal pseudo-obstruction and related animal models

Contact Info

Product

Resources

About