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Treatment of plasminogen deficiency patients with fresh frozen plasma.
Kizilocak, Hande; Ozdemir, Nihal; Dikme, Gürcan; Koç, Begüm; Atabek, Ayse Ayzit; Çokugras, Haluk; Iskeleli, Güzin; Dönmez-Demir, Buket; Christiansen, Nina Merete; Ziegler, Maike; Ozdag, Hilal; Schuster, Volker; Celkan, Tiraje.
Afiliação
  • Kizilocak H; Department of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Ozdemir N; Department of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Dikme G; Department of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Koç B; Department of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Atabek AA; Department of Pediatric Pulmonology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Çokugras H; Department of Pediatric Pulmonology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Iskeleli G; Department of Ophthalmology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
  • Dönmez-Demir B; Biotechnology Institute, Ankara University, Ankara, Turkey.
  • Christiansen NM; Hospital for Children and Adolescents, University of Leipzig, Leipzig, Germany.
  • Ziegler M; Hospital for Children and Adolescents, University of Leipzig, Leipzig, Germany.
  • Ozdag H; Biotechnology Institute, Ankara University, Ankara, Turkey.
  • Schuster V; Hospital for Children and Adolescents, University of Leipzig, Leipzig, Germany.
  • Celkan T; Department of Pediatric Hematology-Oncology, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Pediatr Blood Cancer ; 65(2)2018 Feb.
Article em En | MEDLINE | ID: mdl-28876531
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.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Plasma / Plasminogênio / Transfusão de Componentes Sanguíneos / Conjuntivite / Doenças Genéticas Inatas Tipo de estudo: Diagnostic_studies Limite: Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Ano de publicação: 2018 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Plasma / Plasminogênio / Transfusão de Componentes Sanguíneos / Conjuntivite / Doenças Genéticas Inatas Tipo de estudo: Diagnostic_studies Limite: Child, preschool / Female / Humans / Infant / Male / Newborn Idioma: En Ano de publicação: 2018 Tipo de documento: Article