Cystic fibrosis associated with neuronal intestinal dysplasia type B: A case report

Wildhaber, JH; Seelentag, Walter; Spiegel, Roland; Schöni, Martin H.

doi:10.1016/s0022-3468(96)90419-4

Cited by 15 publications

(7 citation statements)

References 15 publications

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“…In a case report, a combination of cystic fibrosis and IND B was observed. It was postulated that a genetic anomaly of chromosome 7 q could be involved in the development of IND B (36). Another investigation showed a missing immunoreaction of neural cell adhesion molecules (NCAM), growth associated protein 43 (GAP-43), and synaptophysin (11), which are considered to be indicators of insufficient neuromuscular innervation.…”

Section: Discussionmentioning

confidence: 99%

Updated Results on Intestinal Neuronal Dysplasia (IND B)

Meier‐Ruge¹,

Ammann

Bruder³

et al. 2004

Eur J Pediatr Surg

103

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The diagnosis of IND B requires that biopsies are taken proximal to the ampulla recti (about 8-10 cm above the dentate line) with a sufficient amount of submucosa. The biopsies must be cut rectangular to the surface of the mucosa. A diagnosis of IND B can be made only if, in the submucosa of 30 serial sections, 15-20 % of all ganglia are giant ganglia with more than eight nerve cells. Ganglioneuromatosis (MEN2B) must be clearly differentiated from IND. The clinical course of IND B depends on the extent of disturbed bowel innervation, the severity of motility failure, and the coexistence of MH. The conservative management of isolated IND is possible in most children. In individual cases, however, a transient enterostomy or a segmental resection is unavoidable.

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Section: Discussionmentioning

confidence: 99%

Updated Results on Intestinal Neuronal Dysplasia (IND B)

Meier‐Ruge¹,

Ammann

Bruder³

et al. 2004

Eur J Pediatr Surg

103

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“…The absence of intramural ganglion cells only in the narrowed sigmoid segment, below the dilatation of left and transverse colon, excludes also a pseudo-Hirschsprung's disease or an association of the cystic fibrosis with intestinal neuronal dysplasia as the case reported by Wildhaber [2].…”

Section: Discussionmentioning

confidence: 75%

Another case of cystic fibrosis complicated by meconium ileus associated with Hirschsprung’s disease: a rare and important association

et al. 2008

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“…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.

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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.

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Cystic fibrosis associated with neuronal intestinal dysplasia type B: A case report

Cited by 15 publications

References 15 publications

Updated Results on Intestinal Neuronal Dysplasia (IND B)

Updated Results on Intestinal Neuronal Dysplasia (IND B)

Another case of cystic fibrosis complicated by meconium ileus associated with Hirschsprung’s disease: a rare and important association

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

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