| Literature DB >> 28876531 |
Hande Kızılocak1, Nihal Ozdemir1, Gürcan Dikme1, Begüm Koç1, Ayşe Ayzıt Atabek2, Haluk Çokuğraş2, Güzin İskeleli3, Buket Dönmez-Demir4, Nina Merete Christiansen5, Maike Ziegler5, Hilal Ozdağ4, Volker Schuster5, Tiraje Celkan1.
Abstract
Congenital plasminogen (Plg) deficiency leads to the development of ligneous membranes on mucosal surfaces. Here, we report our experience with local and intravenous fresh frozen plasma (FFP). We retrospectively reviewed medical files of 17 patients and their eight first-degree relatives. Conjunctivitis was the main complaint. Thirteen patients were treated both with intravenous and conjunctival FFP. Venous thrombosis did not develop in any. Genetic evaluation revealed heterogeneous mutations as well as polymorphisms. Diagnosis and treatment of Plg deficiency is challenging; topical and intravenous FFP may be an alternative treatment.Entities:
Keywords: ligneous conjunctivitis; ligneous gingivitis; plasminogen
Mesh:
Substances:
Year: 2017 PMID: 28876531 DOI: 10.1002/pbc.26779
Source DB: PubMed Journal: Pediatr Blood Cancer ISSN: 1545-5009 Impact factor: 3.167