医学
斯科普斯
睑裂
眼科
皮肤病科
外科
梅德林
政治学
法学
作者
Roberto Caputo,Amy D. Shapiro,Maria Teresa Sartori,Andrea Leonardi,Bennie H. Jeng,Charles Nakar,Irene Di Pasquale,Francis W. Price,Neelam Thukral,Anna Lotti Suffredini,Laura Pino,Roberto Crea,Prasad Mathew,Mirella Calcinai
出处
期刊:Ophthalmology
[Elsevier]
日期:2022-08-01
卷期号:129 (8): 955-957
被引量:4
标识
DOI:10.1016/j.ophtha.2022.03.019
摘要
Ligneous conjunctivitis (LC) is the most frequent manifestation of plasminogen deficiency type 1 (approximately 80% of cases), 1 Schuster V. Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003; 48: 369-388 Abstract Full Text Full Text PDF PubMed Scopus (119) Google Scholar an ultrarare disease that affects 1.6 per 1 million individuals and is probably underdiagnosed. Ligneous conjunctivitis is characterized by recurrent fibrin-rich, “woody” pseudomembranes on the palpebral (tarsal) conjunctivae. 1 Schuster V. Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003; 48: 369-388 Abstract Full Text Full Text PDF PubMed Scopus (119) Google Scholar Corneal involvement, reported in 20% to 30% of patients, may lead to visual loss. 1 Schuster V. Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003; 48: 369-388 Abstract Full Text Full Text PDF PubMed Scopus (119) Google Scholar Several topical treatments have been tried for disease control or prevention without consistent results, and surgical excision of membranes alone provides only short-lived relief. 2 Watts P. Suresh P. Mezer E. et al. Effective treatment of ligneous conjunctivitis with topical plasminogen. Am J Ophthalmol. 2002; 133 (erratum in Am J Ophthalmol. 2002;134(2):310): 451-455 Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar ,3 Shapiro A.D. Nakar C. Parker J.M. et al. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency. Blood. 2018; 131: 1301-1310 Crossref PubMed Scopus (17) Google Scholar Systemic plasminogen replacement (fresh frozen plasma) was found to be effective, but is hampered by the need for repeated infusions. 4 Gürlü V.P. Demir M. Alimgil M.L. Erda S. Systemic and topical fresh-frozen plasma treatment in a newborn with ligneous conjunctivitis. Cornea. 2008; 27: 501-503 Crossref PubMed Scopus (19) Google Scholar ,5 Yurttaser Ocak S. Bas E. Treatment of ligneous conjunctivitis with fresh frozen plasm in twin babies. J AAPOS. 2021; 25: 50-52 Abstract Full Text Full Text PDF PubMed Scopus (1) Google Scholar Topical plasminogen concentrate treatment allows for easy administration and has demonstrated efficacy in several case studies. 2 Watts P. Suresh P. Mezer E. et al. Effective treatment of ligneous conjunctivitis with topical plasminogen. Am J Ophthalmol. 2002; 133 (erratum in Am J Ophthalmol. 2002;134(2):310): 451-455 Abstract Full Text Full Text PDF PubMed Scopus (59) Google Scholar ,6 Caputo R. Pucci N. Mori F. et al. Long-term efficacy of surgical removal of pseudomembranes in a child with ligneous conjunctivitis treated with plasminogen eyedrops. Thromb Haemost. 2008; 100: 1196-1198 PubMed Google Scholar ,7 Heidemann D.G. Williams G.A. Hartzer M. et al. Treatment of ligneous conjunctivitis with topical plasmin and topical plasminogen. Cornea. 2003; 22: 760-762 Crossref PubMed Scopus (40) Google Scholar
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