Premium
A novel combined treatment for plasminogen deficiency with lung involvement
Author(s) -
Kilinc Ayse A.,
Tarcin Gurkan,
Kurugoglu Sebuh,
Schuster Volker,
Cokugras Haluk,
Celkan Tiraje
Publication year - 2020
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.24563
Subject(s) - medicine , fibrin , respiratory distress , lung , respiratory disease , respiratory system , respiratory tract , conjunctiva , surgery , plasminogen activator , tissue plasminogen activator , pathology , immunology
Plasminogen deficiency is characterized by fibrin‐rich pseudomembrane formation on all mucosal surfaces, particularly the conjunctiva. Respiratory system involvement is common; fibrin often obstructs the upper or lower respiratory tract, causing death. Although many treatments have been applied, no definitive treatment (especially of the respiratory involvement) yet exists. Although excision of tracheobronchial tree membranes affords temporary improvement, this should be performed only for patients in severe respiratory distress; the procedure triggers fibrin redeposition. Here, we share our experience with an 8‐year‐old plasminogen‐deficient female with severe lung involvement; we successfully delivered local tissue plasminogen activator followed by nebulized fresh frozen plasma.