{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,23]],"date-time":"2026-01-23T08:02:14Z","timestamp":1769155334503,"version":"3.49.0"},"reference-count":5,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,11,4]],"date-time":"2019-11-04T00:00:00Z","timestamp":1572825600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Plasminogen deficiency is characterized by fibrin\u2010rich 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.<\/jats:p><jats:p>Here, we share our experience with an 8\u2010year\u2010old plasminogen\u2010deficient female with severe lung involvement; we successfully delivered local tissue plasminogen activator followed by nebulized fresh frozen plasma.<\/jats:p>","DOI":"10.1002\/ppul.24563","type":"journal-article","created":{"date-parts":[[2019,11,4]],"date-time":"2019-11-04T14:25:05Z","timestamp":1572877505000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["A novel combined treatment for plasminogen deficiency with lung involvement"],"prefix":"10.1002","volume":"55","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2879-8910","authenticated-orcid":false,"given":"Ayse A.","family":"Kilinc","sequence":"first","affiliation":[{"name":"Department of Pediatric Pulmonology, Cerrahpasa Faculty of Medicine \u0130stanbul University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gurkan","family":"Tarcin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Cerrahpasa Faculty of Medicine \u0130stanbul University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebuh","family":"Kurugoglu","sequence":"additional","affiliation":[{"name":"Department of Pediatric Radiology, Cerrahpasa Faculty of Medicine \u0130stanbul University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Volker","family":"Schuster","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine University of Leipzig Leipzig Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haluk","family":"Cokugras","sequence":"additional","affiliation":[{"name":"Department of Pediatric Pulmonology, Cerrahpasa Faculty of Medicine \u0130stanbul University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiraje","family":"Celkan","sequence":"additional","affiliation":[{"name":"Department of Pediatric Haematology, Cerrahpasa Faculty of Medicine \u0130stanbul University \u0130stanbul Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,11,4]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1538-7836.2007.02776.x"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11239-016-1416-6"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.1105"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0039-6257(03)00056-0"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.26779"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.24563","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/ppul.24563","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.24563","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,7]],"date-time":"2023-09-07T23:26:33Z","timestamp":1694129193000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.24563"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,4]]},"references-count":5,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,1]]}},"alternative-id":["10.1002\/ppul.24563"],"URL":"https:\/\/doi.org\/10.1002\/ppul.24563","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,11,4]]},"assertion":[{"value":"2019-09-15","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-10-16","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2019-11-04","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}