Treatment of plasminogen deficiency patients with fresh frozen plasma
| Autor: | Maike Ziegler, Ayse Ayzit Atabek, Haluk Cokugras, Tiraje Celkan, Buket Dönmez-Demir, Guzin Iskeleli, Hilal Özdağ, Nina M. Christiansen, Volker Schuster, Gürcan Dikme, Hande Kizilocak, Nihal Özdemir, Begum Sirin Koc |
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| Rok vydání: | 2017 |
| Předmět: |
Male
medicine.medical_specialty Blood Component Transfusion 030204 cardiovascular system & hematology Gastroenterology 03 medical and health sciences Plasma 0302 clinical medicine Internal medicine Ligneous conjunctivitis Medicine Humans Ligneous gingivitis Polymorphism Genetic business.industry Genetic Diseases Inborn Infant Newborn Infant Plasminogen Hematology medicine.disease Conjunctivitis Alternative treatment Venous thrombosis Oncology Child Preschool Pediatrics Perinatology and Child Health Mutation 030221 ophthalmology & optometry Female Fresh frozen plasma business Plasminogen deficiency |
| Zdroj: | Pediatric bloodcancer. 65(2) |
| ISSN: | 1545-5017 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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