title: [A rare cause of neonatal exudative enteropathy: congenital Langerhans cell histiocytosis (histiocytosis X)].
abstract: A case of Langerhans cell histiocytosis is reported in a neonate. Intestinal involvement was especially diffuse and severe, presenting as a protein-losing enteropathy secondary to massive mucosal infiltration by histiocytic cells. The infant died at the age of 3 1/2 months despite therapy with corticosteroids and vinblastine then etoposide and interferon. Such an outcome confirmed the severity of forms with neonatal onset and digestive involvement.
authors.affiliation: Service de Gastroentérologie et Nutrition Pédiatriques, Hôpital Trousseau, Paris.
authors.forename: L
authors.initials: L
authors.lastname: Carlier-Mercier
authors.forename: J L
authors.initials: JL
authors.lastname: Fontaine
authors.forename: L
authors.initials: L
authors.lastname: Boccon-Gibod
authors.forename: J P
authors.initials: JP
authors.lastname: Girardet
authors.forename: P
authors.initials: P
authors.lastname: Josset
authors.forename: F
authors.initials: F
authors.lastname: Gouraud
authors.forename: M F
authors.initials: MF
authors.lastname: Pinturier
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D001706:Biopsy; D004359:Drug Therapy, Combination; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000151:congenital; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011504:Protein-Losing Enteropathies / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D014747:Vinblastine / Q000627:therapeutic use
chemical_list: D000305:Adrenal Cortex Hormones; D014747:Vinblastine
vernacular_title: Une cause rare d'entéropathie exsudative néonatale: l'histiocytose congénitale à cellules de Langerhans (histiocytose X).