Type I Plasminogen Deficiency

A rare autosomal recessive disorder characterized by chronic muscosal pseudomembranous lesions consisting of subepithelial FIBRIN deposition and INFLAMMATION. The most common manifestation is ligneous ('wood-like') conjunctivitis, a redness and subsequent formation of pseudomembranes mostly on the CONJUNCTIVA of the eye that progress to white, yellow-white, or red thick masses with a wood-like consistency and replace the normal mucosa. The lesions may be triggered by local injury and/or infection and often recur after local excision. Lesions of other mucous membranes often occur in the mouth, nasopharynx, trachea, and female genital tract. Some affected children also have congenital occlusive HYDROCEPHALUS. A slightly increased female:male ratio has been observed (1.4:1 to 2:1). Mutations in the PLG gene have been identified. OMIM: 217090

Also Known As:

Networked: 123 relevant articles (10 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Congenital Plasminogen Deficiency
2.	Virus Diseases (Viral Diseases)
3.	Conjunctivitis
4.	Venous Thrombosis (Deep-Vein Thrombosis)
5.	Inflammation (Inflammations)

Experts

1.	Schuster, V: 6 articles (01/2017 - 09/2000)
2.	Schuster, Volker: 5 articles (03/2011 - 07/2003)
3.	Sartori, Maria Teresa: 3 articles (01/2022 - 01/2006)
4.	Saggiorato, Graziella: 3 articles (07/2019 - 07/2003)
5.	Tefs, Katrin: 3 articles (09/2007 - 07/2003)
6.	Caputo, Roberto: 2 articles (01/2022 - 12/2008)
7.	Crea, Roberto: 2 articles (01/2022 - 11/2020)
8.	Di Pasquale, Irene: 2 articles (01/2022 - 07/2019)
9.	Burcu, Ayse: 2 articles (03/2021 - 11/2012)
10.	Sivolella, Stefano: 2 articles (07/2019 - 04/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Type I Plasminogen Deficiency:

1.	PlasminogenIBA 03/01/2017 - "Adjunctive topical plasminogen application appears to be an effective and safe method of controlling pseudomembrane recurrence in patients with ligneous conjunctivitis." 04/01/2002 - "Effective treatment of ligneous conjunctivitis with topical plasminogen." 03/01/2002 - "Improved plasminogen variants for treatment of severe type I plasminogen deficiency would be of great clinical importance." 01/01/2006 - "Difficulties in the mutation analysis of plasminogen gene: a study in two patients with ligneous conjunctivitis." 03/01/1998 - "This study provides direct evidence that plasminogen deficiency is one cause of ligneous conjunctivitis and suggests that plasminogen-deficient mice may be an excellent model for the development of therapeutic strategies for the treatment of this debilitating disease."
2.	Heparin (Liquaemin)FDA LinkGeneric 01/01/2019 - "Fresh frozen plasma (Octaplas) and topical heparin in the management of ligneous conjunctivitis." 10/01/2012 - "Topical fresh frozen plasma and heparin treatment of ligneous conjunctivitis in a Canadian hospital setting." 10/01/2011 - "Heparin in the long-term management of ligneous conjunctivitis: a case report and review of literature." 01/01/2009 - "Fresh frozen plasma treatment of ligneous conjunctivitis: use of heparin and allogeneic serum." 11/01/1995 - "Topical heparin in the treatment of ligneous conjunctivitis."
3.	Ophthalmic Solutions (Eye Drops)IBA 12/01/2008 - "Long-term efficacy of surgical removal of pseudomembranes in a child with ligneous conjunctivitis treated with plasminogen eyedrops." 08/01/2013 - "Here we report the results of pre-clinical studies performed to investigate the therapeutic effectiveness of an eye-drop plasminogen preparation in B6.129P2-Plg(tm1Jld) transgenic mice, a model of ligneous conjunctivitis. " 10/01/2011 - "Furthermore, this study is one of the first to present over 5-year follow-up of a patient with ligneous conjunctivitis effectively managed with long-term heparin eye drops." 01/01/2023 - "Ligneous conjunctivitis and use of human plasminogen eyedrops: The Italian experience." 01/01/2022 - "Treatment of Ligneous Conjunctivitis with Plasminogen Eyedrops."
4.	Cyclosporine (Ciclosporin)FDA LinkGeneric 07/15/1991 - "Response of reactivated ligneous conjunctivitis to topical cyclosporine." 08/15/1989 - "Immunohistologic findings and results of treatment with cyclosporine in ligneous conjunctivitis." 02/15/1989 - "Immunohistologic findings and results of treatment with cyclosporine in ligneous conjunctivitis." 11/01/2012 - "Treatment of ligneous conjunctivitis with amniotic membrane transplantation and topical cyclosporine." 01/01/2021 - "We present twin babies with ligneous conjunctivitis managed with fresh frozen plasma, topical heparin, and topical cyclosporine, without surgical excision of membranes."
5.	Lubricant Eye Drops (Artificial Tears)IBA 01/01/2023 - "We report an infant with unilateral ligneous conjunctivitis who responded well to topical, commercially-available heparin-containing artificial tears treatment. "
6.	CortisoneFDA Link 03/05/2022 - "This treatment with the combination of plasminactivator, heparin, cortisone and cyclosporine has proven to be effective in preventing the recurrence of ligneous conjunctivitis."
7.	Contraceptive Agents (Contraceptives)IBA 07/01/2003 - "Contraceptive pills induce an improvement in congenital hypoplasminogenemia in two unrelated patients with ligneous conjunctivitis."
8.	Immunoglobulin A (IgA)IBA 01/01/2017 - "Letter Regarding "Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature" by Y. " 01/01/2016 - "Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature." 01/01/2016 - "A novel plasminogen gene mutation, deficiency of plasminogen antigen and activity, and anti-plasminogen IgG and IgA antibodies were identified in a patient with adult-onset ligneous conjunctivitis. "
9.	Amyloid (Amyloid Fibrils)IBA 12/01/1968 - "[Amyloid and plasma cell conjunctivitis, clinically "ligneous conjunctivitis" (histopathological study apropos of 24 biopsy cases)]." 03/01/2000 - "We report a case of juvenile colloid milium associated with ligneous conjunctivitis and gingival deposits of an amyloid-like homogenous eosinophilic material. "
10.	SolutionsIBA 08/01/2013 - "The results of the present study clearly showed that the administration for six times a day of plasminogen eye-drop solution in the lesioned eye of animals knock-out for plasminogen gene and developing ligneous conjunctivitis caused a dose and time related reduction of the extent of the ocular lesion. "

Therapies and Procedures

1.	Therapeutics 03/01/2021 - "To evaluate the efficacy of topical fresh frozen plasma (FFP) therapy on clinical symptoms, findings, and prognosis after anterior segment surgeries in patients with ligneous conjunctivitis (LC). " 05/01/1977 - "[Diagnosis and therapy of ligneous conjunctivitis]." 09/01/2021 - "[Pharmaceutical supply of human plasminogen replacement therapy for the treatment of ligneous conjunctivitis: Logistics for a university hospital]." 01/01/2019 - "Retraction: Treatment of Ligneous Conjunctivitis Using Topical Plasminogen Therapy in An 8-Week-Old Female Infant." 08/29/2018 - "Treatment of Ligneous Conjunctivitis Using Topical Plasminogen Therapy in an 8-Week-Old Female Infant."
2.	Transplantation 04/01/2004 - "In this first report of amniotic membrane transplantation in a case of ligneous conjunctivitis, we found that conjunctival reconstruction shows promising results in this difficult-to-manage disease." 11/01/2018 - "To the authors' knowledge, this is the first reported case of the successful treatment of canine ligneous conjunctivitis with amniotic membrane transplantation." 11/01/2018 - "Amniotic membrane transplantation for ligneous conjunctivitis in a doberman." 04/01/2004 - "After confirmation of the diagnosis of ligneous conjunctivitis, she underwent amniotic membrane transplantation. " 04/01/2004 - "To report a case of ligneous conjunctivitis treated with amniotic membrane transplantation. "
3.	Conservative Treatment 01/01/2023 - "We present an interesting case of ligneous conjunctivitis caused by a viral infection that responded well to conservative management. "
4.	Refractive Surgical Procedures 10/24/2023 - "It has been used in many ocular surface diseases including persistent epithelial defects (corneal or conjunctival), chemical or thermal burns, limbal stem cell deficiency, cicatrising conjunctivitis, ocular graft versus host disease, microbial keratitis, corneal perforation, bullous keratopathy, dry eye disease, corneal haze following refractive surgery and cross-linking, band keratopathy, ocular surface neoplasia, pterygium surgery, and ligneous conjunctivitis. "
5.	Corneal Transplantation (Keratoplasty) 09/01/1993 - "Thirty-five patients (81%) with disorders including high-risk keratoplasty, atopic and vernal keratoconjunctivitis, ligneous conjunctivitis, ulcerative keratitis, and Mooren's ulcer had a beneficial result, with resolution, reduction, or prevention of inflammation. "