PMID: 8969725Dec 1, 1996Paper

Digestive tract involvement in Langerhans cell histiocytosis. The French Langerhans Cell Histiocytosis Study Group

The Journal of Pediatrics
F GeissmannN Brousse

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease with a wide clinical spectrum. Although little is known of gastrointestinal involvement in LCH, it may be a major clinical problem. We investigated clinical, pathologic, and immunohistochemical features of digestive tract LCH involvement in children. Selection criteria consisted of the presence of LCH with digestive symptoms, and histologic confirmation of gastrointestinal involvement. Seven children (2%) met the criteria among 348 cases of LCH in a French national retrospective survey from 1983 to 1993. Two children whose LCH was diagnosed in 1994 were also selected. Nine children with LCH and digestive tract involvement were studied. Clinical features at presentation included skin (9/9) and mucosal (4/9) involvement, failure to thrive (5/9), diarrhea (7/9), bloody stools (4/7), vomiting (4/9), and hypoalbuminemia (8/9). Five of the nine children died; factors associated with a poor prognosis included young age, organ dysfunction (stage 4), and need for parenteral nutrition. Unlike control biopsy specimens, LCH cells of children with digestive tract involvement disclosed expression of the mucosal homing receptor integrin alpha 4 beta 7 on frozen skin and digestive tract bio...Continue Reading

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Citations

Aug 19, 2007·PLoS Medicine·Brigitte SenechalFrederic Geissmann
May 16, 2008·Radiographics : a Review Publication of the Radiological Society of North America, Inc·Sabine SchmidtFrancois Gudinchet
May 20, 2015·Abdominal Imaging·Natally de Souza Maciel Rocha HorvatRonaldo Hueb Baroni
Dec 18, 2013·Fetal and Pediatric Pathology·Badr AbdullgaffarLaila Al-Otaibi
Jan 15, 2017·La Presse médicale·Jean Donadieu, Sébastien Héritier
Oct 24, 2008·Inflammatory Bowel Diseases·Henar Núñez RodríguezFrancisco Igea
Nov 8, 2001·Journal of Pediatric Gastroenterology and Nutrition·J LevyJ Garvin
Aug 16, 2019·The American Journal of Surgical Pathology·Zainab I AlruwaiiElizabeth A Montgomery
Mar 17, 2006·Journal of Pediatric Gastroenterology and Nutrition·Martin LacherUdo Kontny
Dec 2, 2004·Journal of Pediatric Gastroenterology and Nutrition·M SabriS Ranganathan
Oct 8, 1999·Journal of Pediatric Gastroenterology and Nutrition·R DohilJ Dimmick
Oct 8, 1999·Journal of Pediatric Gastroenterology and Nutrition·V R NanduriJ Pritchard
Oct 6, 2005·Journal of Pediatric Gastroenterology and Nutrition·A C PriorL Gomes
Jun 20, 2021·The Journal of Pediatrics·Milen MinkovUNKNOWN LCH Study Group of the Histiocyte Society
Feb 16, 2006·Actas dermo-sifiliográficas·Marta Valdivielso, Celso Bueno

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