title: Variable clinical presentation in primary lymphoedema: report of two cases.
abstract: Lymphoedema is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system. Lymphoedema may be primary or secondary and can be inherited. Primary lymphoedema (primary lymphatic dysplasia) is a chronic oedema caused by a developmental abnormality of the lymphatic system. Primary lymphoedema most commonly affects the lower limbs, but other body parts can also be affected. It can be associated with some specific syndromes (i.e. Hennekam syndrome) and genetic disorders. In this article, we report on two patients with congenital multisegmental lymphoedema and Hennekam syndrome, both primary lymphoedemas.
authors.affiliation: Division of Pediatric Cardiology Division of Pediatric Gastroenterology, Erciyes University Faculty of Medicine, Kayseri, Turkey.
authors.forename: Abdullah
authors.initials: A
authors.lastname: Ozyurt
authors.forename: Eylem
authors.initials: E
authors.lastname: Sevinc
authors.forename: Ali
authors.initials: A
authors.lastname: Baykan
authors.forename: Duran
authors.initials: D
authors.lastname: Arslan
authors.forename: Mustafa
authors.initials: M
authors.lastname: Argun
authors.forename: Ozge
authors.initials: O
authors.lastname: Pamukcu
authors.forename: Kazim
authors.initials: K
authors.lastname: Uzum
mesh_terms: D000293:Adolescent; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000175:diagnosis* / Q000628:therapy; D005832:Genital Diseases, Male / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D008201:Lymphangiectasis, Intestinal / Q000175:diagnosis* / Q000628:therapy; D008209:Lymphedema / Q000175:diagnosis* / Q000628:therapy