[A rare cause of neonatal exudative enteropathy: congenital Langerhans cell histiocytosis (histiocytosis X)].
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.
Carlier-Mercier
L
L
Service de Gastroentérologie et Nutrition Pédiatriques, Hôpital Trousseau, Paris.
Fontaine
J L
JL
Boccon-Gibod
L
L
Girardet
J P
JP
Josset
P
P
Gouraud
F
F
Pinturier
M F
MF
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
D000305:Adrenal Cortex Hormones; D014747:Vinblastine
Une cause rare d'entéropathie exsudative néonatale: l'histiocytose congénitale à cellules de Langerhans (histiocytose X).