title: Neurofibromatosis presenting as a severe systemic vasculopathy.
abstract: In a boy with neurofibromatosis type 1 (NF-1), hypertension, septic infection of an aneurysm in the deltoid muscle, bowel infarction, multiple arterial aneurysms and venous thrombosis occurred within a period of 6 weeks. Histologically, vascular neurofibromatosis of the small vessels of the gut was found. This unusual occurrence of a multitude of clinical features within a few weeks was caused by vascular neurofibromatosis. Awareness of this condition in patients with NF-1 may help the paediatrician to avoid unnecessary diagnostic procedures and to initiate appropriate symptomatic therapy.
authors.affiliation: Universitätskinderklinik und Poliklinik, Würzburg, Germany.
authors.forename: T
authors.initials: T
authors.lastname: Lehrnbecher
authors.forename: A M
authors.initials: AM
authors.lastname: Gassel
authors.forename: V
authors.initials: V
authors.lastname: Rauh
authors.forename: T
authors.initials: T
authors.lastname: Kirchner
authors.forename: H I
authors.initials: HI
authors.lastname: Huppertz
mesh_terms: D001158:Arteries / Q000473:pathology; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000473:pathology; D014652:Vascular Diseases / Q000175:diagnosis* / Q000473:pathology