{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T07:56:06Z","timestamp":1766735766990,"version":"3.48.0"},"reference-count":11,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2025,12,26]],"date-time":"2025-12-26T00:00:00Z","timestamp":1766707200000},"content-version":"vor","delay-in-days":25,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,12,24]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Background: Plasminogen deficiency is an ultra-rare autosomal recessive disorder characterized by impaired fibrinolysis and the formation of fibrin-rich pseudomembranes. Ligneous conjunctivitis is the most common manifestation, whereas central nervous system involvement, such as hydrocephalus, is rare. Early recognition is essential to prevent irreversible complications. Case Presentation: We report a 3-year-old girl with progressive macrocephaly, antenatal hydrocephalus, and persistent pseudomembranous conjunctivitis. Imaging showed fused lateral ventricles and agenesis of the corpus callosum, consistent with obstructive hydrocephalus requiring multiple ventriculoperitoneal shunt revisions. Genetic testing confirmed type I plasminogen deficiency with a homozygous PLG mutation. Recombinant plasminogen was unavailable, and she was managed with fresh frozen plasma (FFP) infusions plus ophthalmic and anticonvulsant therapy. Outcome: Regular FFP stabilized her ocular and neurological condition, though recurrent shunt complications necessitated repeated hospitalizations. Conclusion: This case illustrates the diagnostic challenges of type I plasminogen deficiency and highlights FFP as a practical therapeutic option in resource-limited settings.<\/jats:p>","DOI":"10.1093\/omcr\/omaf260","type":"journal-article","created":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T12:48:46Z","timestamp":1761655726000},"source":"Crossref","is-referenced-by-count":0,"title":["Fresh frozen plasma therapy in type I plasminogen deficiency: a case of ligneous conjunctivitis with hydrocephalus"],"prefix":"10.1093","volume":"2025","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-8461-6620","authenticated-orcid":false,"given":"Ibrahim","family":"AboGhayyada","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University , Wadi Al-Hariyah Street, Hebron 9020000 ,","place":["Palestine"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad","family":"Zeidan","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University , Wadi Al-Hariyah Street, Hebron 9020000 ,","place":["Palestine"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-9578-1105","authenticated-orcid":false,"given":"Taha Z","family":"Makhlouf","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University , Wadi Al-Hariyah Street, Hebron 9020000 ,","place":["Palestine"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amir","family":"AbuGhiyatha","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Mansoura University , Gehan Al-Sadat Street, Mansoura 35516 ,","place":["Egypt"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mosab","family":"Ghnimat","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University , Wadi Al-Hariyah Street, Hebron 9020000 ,","place":["Palestine"]},{"name":"Pediatrics Department, Beit Jala Hospital , Beit Jala Street, Bethlehem 4322 ,","place":["Palestine"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mohammad","family":"Najajreh","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Palestine Polytechnic University , Wadi Al-Hariyah Street, Hebron 9020000 ,","place":["Palestine"]},{"name":"Pediatrics Department, Beit Jala Hospital , Beit Jala Street, Bethlehem 4322 ,","place":["Palestine"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,12,26]]},"reference":[{"key":"2025122602495691300_ref1","doi-asserted-by":"publisher","first-page":"367","DOI":"10.1038\/s12276-020-0397-x","article-title":"Therapeutics targeting the fibrinolytic system","volume":"52","author":"Lin","year":"2020","journal-title":"Exp Mol Med"},{"key":"2025122602495691300_ref2","doi-asserted-by":"publisher","first-page":"274","DOI":"10.1016\/j.bjhh.2016.04.006","article-title":"Gingival swelling associated with 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cases","volume":"87","author":"Almeida","year":"2024","journal-title":"Arq Bras Oftalmol"}],"container-title":["Oxford Medical Case 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