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Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus.
J Pediatr Gastroenterol Nutr 2009; 49(1):42-51JP

Abstract

BACKGROUND

Cystic fibrosis (CF) is a multisystem disorder intrinsically associated with inflammation of mucosal surfaces. Because inflammation can result in enteric neuromuscular dysfunction we hypothesized that terminal ileitis in patients with CF may predispose to distal ileal obstruction syndrome (DIOS).

METHODS AND PATIENTS

Full-thickness terminal ileal tissues from 6 children with CF and severe DIOS, 6 infants with complicated meconium ileus (MI), and 6 children with non-CF intestinal atresia were studied.

RESULTS

Lymphocyte-predominant mucosal and transmural ileal inflammation was present in 6 of 6 patients with DIOS. Lymphocytic ganglionitis was present in 4 of 6 although numbers of myenteric neurons were not decreased (5/5). Myocyte proteins were preserved (6/6). Mild submucosal fibrosis was common in DIOS (5/6) and transformation of submucosal fibroblasts to a myofibroblastic phenotype was noted in 4 of 6. Inflammatory changes were distinct from those described in fibrosing colonopathy. Antroduodenal manometry in an individual who had experienced MI/DIOS was consistent with a neuropathic pseudo-obstructive process. Submucosal or transmural lymphocyte predominant inflammation was also present in 6 of 6 infants with complicated MI, which, when coupled with submucosal myofibroblast proliferation (5/6), appeared highly predictive of CF rather than non-CF atresia. Histological findings at birth were similar, although milder, than those seen in DIOS, suggesting that these changes are a primary abnormality in CF.

CONCLUSIONS

Submucosal or transmural inflammation of the ileum is common in newborns with CF and MI and older children with DIOS. Severe recurrent DIOS should be investigated with seromuscular and mucosal biopsy of the ileum to seek a transmural ileitis potentially amenable to anti-inflammatory therapies.

Authors+Show Affiliations

Department of Gastroenterology, UCL Institute of Child Health and Great Ormond Street Hospital, London, UK.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19710558

Citation

Smith, Virpi V., et al. "Lymphocytic Leiomyositis and Myenteric Ganglionitis Are Intrinsic Features of Cystic Fibrosis: Studies in Distal Intestinal Obstruction Syndrome and Meconium Ileus." Journal of Pediatric Gastroenterology and Nutrition, vol. 49, no. 1, 2009, pp. 42-51.
Smith VV, Schäppi MG, Bisset WM, et al. Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus. J Pediatr Gastroenterol Nutr. 2009;49(1):42-51.
Smith, V. V., Schäppi, M. G., Bisset, W. M., Kiparissi, F., Jaffe, A., Milla, P. J., & Lindley, K. J. (2009). Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus. Journal of Pediatric Gastroenterology and Nutrition, 49(1), pp. 42-51. doi:10.1097/MPG.0b013e318186d35a.
Smith VV, et al. Lymphocytic Leiomyositis and Myenteric Ganglionitis Are Intrinsic Features of Cystic Fibrosis: Studies in Distal Intestinal Obstruction Syndrome and Meconium Ileus. J Pediatr Gastroenterol Nutr. 2009;49(1):42-51. PubMed PMID: 19710558.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Lymphocytic leiomyositis and myenteric ganglionitis are intrinsic features of cystic fibrosis: studies in distal intestinal obstruction syndrome and meconium ileus. AU - Smith,Virpi V, AU - Schäppi,Michela G, AU - Bisset,W Michael, AU - Kiparissi,Fevronia, AU - Jaffe,Adam, AU - Milla,Peter J, AU - Lindley,Keith J, PY - 2009/8/28/entrez PY - 2009/8/28/pubmed PY - 2009/11/5/medline SP - 42 EP - 51 JF - Journal of pediatric gastroenterology and nutrition JO - J. Pediatr. Gastroenterol. Nutr. VL - 49 IS - 1 N2 - BACKGROUND: Cystic fibrosis (CF) is a multisystem disorder intrinsically associated with inflammation of mucosal surfaces. Because inflammation can result in enteric neuromuscular dysfunction we hypothesized that terminal ileitis in patients with CF may predispose to distal ileal obstruction syndrome (DIOS). METHODS AND PATIENTS: Full-thickness terminal ileal tissues from 6 children with CF and severe DIOS, 6 infants with complicated meconium ileus (MI), and 6 children with non-CF intestinal atresia were studied. RESULTS: Lymphocyte-predominant mucosal and transmural ileal inflammation was present in 6 of 6 patients with DIOS. Lymphocytic ganglionitis was present in 4 of 6 although numbers of myenteric neurons were not decreased (5/5). Myocyte proteins were preserved (6/6). Mild submucosal fibrosis was common in DIOS (5/6) and transformation of submucosal fibroblasts to a myofibroblastic phenotype was noted in 4 of 6. Inflammatory changes were distinct from those described in fibrosing colonopathy. Antroduodenal manometry in an individual who had experienced MI/DIOS was consistent with a neuropathic pseudo-obstructive process. Submucosal or transmural lymphocyte predominant inflammation was also present in 6 of 6 infants with complicated MI, which, when coupled with submucosal myofibroblast proliferation (5/6), appeared highly predictive of CF rather than non-CF atresia. Histological findings at birth were similar, although milder, than those seen in DIOS, suggesting that these changes are a primary abnormality in CF. CONCLUSIONS: Submucosal or transmural inflammation of the ileum is common in newborns with CF and MI and older children with DIOS. Severe recurrent DIOS should be investigated with seromuscular and mucosal biopsy of the ileum to seek a transmural ileitis potentially amenable to anti-inflammatory therapies. SN - 1536-4801 UR - https://www.unboundmedicine.com/medline/citation/19710558/Lymphocytic_leiomyositis_and_myenteric_ganglionitis_are_intrinsic_features_of_cystic_fibrosis:_studies_in_distal_intestinal_obstruction_syndrome_and_meconium_ileus_ L2 - http://dx.doi.org/10.1097/MPG.0b013e318186d35a DB - PRIME DP - Unbound Medicine ER -