Literature DB >> 28876531

Treatment of plasminogen deficiency patients with fresh frozen plasma.

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.
© 2017 Wiley Periodicals, Inc.

Entities:  

Keywords:  ligneous conjunctivitis; ligneous gingivitis; plasminogen

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Year:  2017        PMID: 28876531     DOI: 10.1002/pbc.26779

Source DB:  PubMed          Journal:  Pediatr Blood Cancer        ISSN: 1545-5009            Impact factor:   3.167


  2 in total

1.  Absence of functional compensation between coagulation factor VIII and plasminogen in double-knockout mice.

Authors:  Rikke Stagaard; Carsten Dan Ley; Kasper Almholt; Lisbeth Høier Olsen; Tom Knudsen; Matthew J Flick
Journal:  Blood Adv       Date:  2018-11-27

Review 2.  An international registry of patients with plasminogen deficiency (HISTORY).

Authors:  Amy D Shapiro; Marzia Menegatti; Roberta Palla; Marco Boscarino; Christopher Roberson; Paolo Lanzi; Joel Bowen; Charles Nakar; Isaac A Janson; Flora Peyvandi
Journal:  Haematologica       Date:  2020-01-30       Impact factor: 9.941

  2 in total

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