title: [Congenital intestinal lymphangiectasis].
abstract: We discuss a newborn with congenital intestinal lymphangiectasia. Primary intestinal lymphangiectasia is a rare disease which represents a congenital disorder of mesenteric lymphatics and is associated with typical clinical signs. The diagnosis can be made on the basis of the typical histological findings in the endoscopic biopsies, the laboratory findings and the radiographic findings. Treatment is palliative by introduction of medium chain triglycerides and by restricting the dietary fat intake. Substitution therapy may be necessary. The longer-term prognosis appears to be good.
authors.affiliation: De Wever Ziekenhuis, afd. Kindergeneeskunde, Heerlen.
authors.forename: W G
authors.initials: WG
authors.lastname: van Kranen
authors.forename: J M
authors.initials: JM
authors.lastname: Sijstermans
authors.forename: P M
authors.initials: PM
authors.lastname: Theunissen
mesh_terms: D001706:Biopsy; D019411:Clinical Laboratory Techniques; D003952:Diagnostic Imaging; D004041:Dietary Fats / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008201:Lymphangiectasis, Intestinal / Q000151:congenital* / Q000175:diagnosis / Q000178:diet therapy; D008297:Male
chemical_list: D004041:Dietary Fats
vernacular_title: Congenitale intestinale lymfangiëctasie.