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Featured Studies
Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency.
Shapiro AD, Nakar C, Parker JM, et al.
Plasminogen deficiency.
Schuster V, Hugle B, Tefs K.
Safety & efficacy of human plasma derived plasminogen ophthalmic drops for treatment of ligneous conjunctivitis: report of phase 2/3 clinical trial.
Nakar CT, Caputo R, Price FW, et al.
Published Studies
- Celkan, T. Plasminogen Deficiency. J Thromb Thrombolysis. 2017;43(1):132‐138.
- Mehta R, Shapiro AD. Plasminogen deficiency. Haemophilia. 2008;14(6):1261-1268.
- Schuster V, Hugle B, Tefs K. Plasminogen deficiency. J Thromb Haemost. 2007;5(12): 2315-2322.
- Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003;48(4):369-388.
- Shapiro AD, Menegatti M, Palla R, et al. An international registry of patients with plasminogen. Haematologica. 2020;105(3):554‐561.
- Tefs K, Gueorguieva M, Klammt J, et al. Molecular and clinical spectrum of type I plasminogen deficiency: a series of 50 patients. Blood. 2006;108(9):3021-3026.
- Bourrienne MC, Loyau S, Faille D, et al. Functional Fibrinolysis Assays Reveal Different Mechanisms underlying Plasminogen Dysfunction in Ligneous Conjunctivitis. Thromb Haemost. 2020;120(5):758‐767.
- Castellino FJ, Ploplis VA. Structure and function of the plasminogen/plasmin system. Thromb Haemost. 2005;93(4):647‐654.
- Collen D, Ong EB, Johnson AJ. Human plasminogen: in vitro and in vivo evidence for the biological integrity of NH2-terminal glutamic acid plasminogen. Thromb Res. 1975;7(4):515‐529. doi:10.1016/0049-3848(75)90099-7
- Klammt J, Kobelt L, Aktas D, et al. Identification of three novel plasminogen (PLG) gene mutations in a series of 23 patients with low PLG activity. Thromb Haemost. 2011;105(3):454-460.
- Ma Q, Ozel AB, Ramdas S, et al. Genetic variants in PLG, LPA, and SIGLEC 14 as well as smoking contribute to plasma plasminogen levels. Blood. 2014;124(20):3155-3164.
- Martin-Fernandez L, Marco P, Corrales I, et al. The unravelling of the genetic architecture of plasminogen deficiency and its relation to thrombotic disease. Sci Rep. 2016;6:39255.
- Petersen TE, Martzen MR, Ichinose A, Davie EW. Characterization of the gene for human plasminogen, a key proenzyme in the fibrinolytic system. J Biol Chem. 1990;265(11):6104‐6111.
- Saes JL, Schols SEM, Betbadal KF, et al. Thrombin and plasmin generation in patients with plasminogen or plasminogen activator inhibitor type 1 deficiency. Haemophilia. 2019;25(6):1073‐1082.
- Schuster V, Mingers AM, Seidenspinner S, Nüssgens Z, Pukrop T, Kreth HW. Homozygous mutations in the plasminogen gene of two unrelated girls with ligneous conjunctivitis. Blood. 1997;90(3):958‐966.
- Takada A, Takada Y. Physiology of plasminogen: with special reference to activation and degradation. Haemostasis. 1988;18 Suppl 1:25‐35.
- Tefs K, Gueorguieva M, Klammt J, et al. Molecular and clinical spectrum of type I plasminogen deficiency: a series of 50 patients. Blood. 2006;108(9):3021-3026.
- Tefs K, Tait CR, Walker ID, Pietzsch N, Ziegler M, Schuster V. A K19E missense mutation in the plasminogen gene is a common cause of familial hypoplasminogenaemia. Blood Coagul Fibrinolysis. 2003;14(4):411-416.
- Twining SS, Wilson PM, Ngamkitidechakul C. Extrahepatic synthesis of plasminogen in the human cornea is up-regulated by interleukins-1alpha and -1beta. Biochem J. 1999;339(Pt 3):705-712.
- Weidinger S, Patutschnick W, Schwarzfischer F. Further evidence of a silent plasminogen (PLG) allele in two paternity cases. Z Rechtsmed. 1988;101(2):99-104.
- Zhang L, Seiffert D, Fowler BJ, et al. Plasminogen has a broad extrahepatic distribution. Thromb Haemost. 2002;87(3):493‐501.Thromb Haemost. 2002;87(3):493‐501.
- Okamoto A, Sakata T, Mannami T, et al. Population-based distribution of plasminogen activity and estimated prevalence and relevance to thrombotic diseases of plasminogen deficiency in the Japanese: the Suita study. J Thromb Haemost. 2003; 1 (11):2397-2403.
- Shapiro AD, Menegatti M, Palla R, et al. An international registry of patients with plasminogen deficiency (HISTORY). Haematologica. 2020;105(3):554‐561.
- Tait RC, Walker ID, Conkie JA, et al. Plasminogen levels in healthy volunteers–influence of age, sex, smoking and oral contraceptives. Thromb Haemost. 1992;68(5): 506-510.
- Tait RC, Walker ID, Conkie JA, Islam SI, McCall F. Isolated familial plasminogen deficiency may not be a risk factor for thrombosis. Thromb Haemost. 1996;76(6):1004‐1008.
- Barabino S, Rolando M. Amniotic membrane transplantation in a case of ligneous conjunctivitis. Am J Ophthalmol. 2004;137 (4):752-753.
- Caputo R, Pucci N, Mori F, Secci J, Novembre E, Frosini R. Long-term efficacy of surgical removal of pseudomembranes in a child with ligneous conjunctivitis treated with plasminogen eyedrops. Thromb Haemost. 2008;100(6):1196‐1198.
- De Cock R, Ficker LA, Dart JG, Garner A, Wright P. Topical heparin in the treatment of ligneous conjunctivitis. Ophthalmology. 1995;102(11):1654-1659.
- Fine G, Bauer K, Al-Mohaya M, Woo SB. Successful treatment of ligneous gingivitis with warfarin. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107(1):77-80.
- Gürlü VP, Demir M, Alimgil ML, Erda S. Systemic and topical fresh-frozen plasma treatment in a newborn with ligneous conjunctivitis. Cornea. 2008;27(4):501‐503.
- Holland EJ, Chan CC, Kuwabara T, Palestine AG, Rowsey JJ, Nussenblatt RB. Immunohistologic findings and results of treatment with cyclosporine in ligneous conjunctivitis. Am J Ophthalmol. 1989;107(2):160-166.
- Kilinc AA, Tarcin G, Kurugoglu S, Schuster V, Cokugras H, Celkan T. A novel combined treatment for plasminogen deficiency with lung involvement. Pediatr Pulmonol. 2020;55(1):E1‐E3.
- Kizilocak H, Ozdemir N, Dikme G, et al. Treatment of plasminogen deficiency patients with fresh frozen plasma. Pediatr Blood Cancer. 2018;65(2):e26779.
- Nakar CT, Caputo R, Price FW, et al. Safety & efficacy of human plasma derived plasminogen ophthalmic drops for treatment of ligneous conjunctivitis: report of phase 2/3 clinical trial. Blood. 2015;126(23):2288.
- Pergantou H, Likaki D, Fotopoulou M, Katsarou O, Xafaki P, Platokouki H. Management of ligneous conjunctivitis in a child with plasminogen deficiency. Eur J Pediatr. 2011;170(10):1333‐1336.
- Pons V, Olivera P, Garcia-Consuegra R, et al. Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases. J Thromb Thrombolysis. 2016;41(3):544-547.
- Sartori MT, Saggiorato G, Pellati D, et al. Contraceptive pills induce an improvement in congenital hypoplasminogenemia in two unrelated patients with ligneous conjunctivitis. Thromb Haemost. 2003;89(01):86-91.
- Schott D, Dempfle CE, Beck P, et al. Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency. N Engl J Med. 1998;339(23):1679-1686.
- Shapiro AD, Nakar C, Parker JM, et al. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency. Blood. 2018;131(12):1301-1310.
- Shimabukuro M, Iwasaki N, Nagae Y, et al. Ligneous conjunctivitis: a case report. Jap J Ophthalmol. 2001;45(4):375-377.
- Tabbara KF. Prevention of ligneous conjunctivitis by topical and subconjunctival fresh frozen plasma. Am J Ophthalmol. 2004; 138 (2):299-300.
- Watts P, Suresh P, Mezer E, et al. Effective treatment of ligneous conjunctivitis with topical plasminogen. Am J Ophthalmol. 2002;133(4):451-455.
- Weinzierl MR, Collmann H, Korinth MC, Gilsbach JM, Rohde V. Management of hydrocephalus in children with plasminogen deficiency. Eur J Pediatr Surg. 2007;17(2):124-128.
Ligneous Conjunctivitis (Eyes)
- Barabino S, Rolando M. Amniotic membrane transplantation in a case of ligneous conjunctivitis. Am J Ophthalmol. 2004;137 (4):752-753.
- Bourrienne MC, Loyau S, Faille D, et al. Functional Fibrinolysis Assays Reveal Different Mechanisms underlying Plasminogen Dysfunction in Ligneous Conjunctivitis. Thromb Haemost. 2020;120(5):758‐767.
- Caputo R, Pucci N, Mori F, Secci J, Novembre E, Frosini R. Long-term efficacy of surgical removal of pseudomembranes in a child with ligneous conjunctivitis treated with plasminogen eyedrops. Thromb Haemost. 2008;100(6):1196‐1198.
- Ciftci E, Ince E, Akar N, Dogru U, Tefs K, Schuster V. Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency. Eur J Pediatr. 2003;162(7-8):462-465.
- De Cock R, Ficker LA, Dart JG, Garner A, Wright P. Topical heparin in the treatment of ligneous conjunctivitis. Ophthalmology. 1995;102(11):1654-1659.
- Firat T. Ligneous conjunctivitis. Am J Ophthalmol. 1974;78(4):679-688.
- Gürlü VP, Demir M, Alimgil ML, Erda S. Systemic and topical fresh-frozen plasma treatment in a newborn with ligneous conjunctivitis. Cornea. 2008;27(4):501‐503.
- Hidayat AA, Riddle PJ. Ligneous conjunctivitis. A clinicopathologic study of 17 cases. Ophthalmology. 1987;94(8):949-959.
- Holland EJ, Chan CC, Kuwabara T, Palestine AG, Rowsey JJ, Nussenblatt RB. Immunohistologic findings and results of treatment with cyclosporine in ligneous conjunctivitis. Am J Ophthalmol. 1989;107(2):160-166.
- Kraft J, Lieb W, Zeitler P, Schuster V. Ligneous conjunctivitis in a girl with severe type I plasminogen deficiency. Graefes Arch Clin Exp Ophthalmol. 2000;238(9):797-800.
- Magdaleno-Tapial J, Hernández-Bel P, Valenzuela-Oñate C, et al. Congenital plasminogen deficiency with long standing pseudomembranous conjunctival and genital lesions. JAAD Case Rep. 2018;5(1):44‐46.
- Mingers AM, Philapitsch A, Zeitler P, Schuster V, Schwarz HP, Kreth HW. Human homozygous type I plasminogen deficiency and ligneous conjunctivitis. APMIS. 1999;107(1):62-72.
- Nakar CT, Caputo R, Price FW, et al. Safety & efficacy of human plasma derived plasminogen ophthalmic drops for treatment of ligneous conjunctivitis: report of phase 2/3 clinical trial. Blood. 2015;126(23):2288.
- Pergantou H, Likaki D, Fotopoulou M, Katsarou O, Xafaki P, Platokouki H. Management of ligneous conjunctivitis in a child with plasminogen deficiency. Eur J Pediatr. 2011;170(10):1333‐1336.
- Sartori MT, Saggiorato G, Pellati D, et al. Contraceptive pills induce an improvement in congenital hypoplasminogenemia in two unrelated patients with ligneous conjunctivitis. Thromb Haemost. 2003;89(01):86-91.
- Schott D, Dempfle CE, Beck P, et al. Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency. N Engl J Med. 1998;339(23):1679-1686.
- Shimabukuro M, Iwasaki N, Nagae Y, et al. Ligneous conjunctivitis: a case report. Jap J Ophthalmol. 2001;45(4):375-377.
- Tabbara KF. Prevention of ligneous conjunctivitis by topical and subconjunctival fresh frozen plasma. Am J Ophthalmol. 2004; 138 (2):299-300.
- Watts P, Suresh P, Mezer E, et al. Effective treatment of ligneous conjunctivitis with topical plasminogen. Am J Ophthalmol. 2002;133(4):451-455.
Ligneous Gingivitis (Gums)
- Chi AC, Prichard E, Richardson MS, Rasenberger KP, Weathers DR, Neville BW. Pseudomembranous disease (ligneous inflammation) of the female genital tract, peritoneum, gingiva, and paranasal sinuses associated with plasminogen deficiency. Ann Diagn Pathol. 2009;13(2):132-139.
- Fine G, Bauer K, Al-Mohaya M, Woo SB. Successful treatment of ligneous gingivitis with warfarin. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107(1):77-80.
- MacPherson M, Pho M, Cox J, Armstrong J, Darling MR, McCord C. Ligneous gingivitis secondary to plasminogen deficiency: a multidisciplinary diagnostic challenge. Oral Surg Oral Med Oral Pathol Oral Radiol. 2020;S2212-4403(19)31640-2.
- Sadasivan A, Ramesh R, Mathew DG. Ligneous Periodontitis in a Patient with Type 1 Plasminogen Deficiency: A Case Report and Review of the Literature. Case Rep Dent. 2020;2020:5680535. Published 2020 Mar 24.
Hydrocephalus (Brain)
- Ciftci E, Ince E, Akar N, Dogru U, Tefs K, Schuster V. Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency. Eur J Pediatr. 2003;162(7-8):462-465.
- Weinzierl MR, Collmann H, Korinth MC, Gilsbach JM, Rohde V. Management of hydrocephalus in children with plasminogen deficiency. Eur J Pediatr Surg. 2007;17(2):124-128.
- Biesinger BS, Male C, Kranebitter V, Binder J, Niederle MB, Reinprecht A, Binder C. Persistent Hydrocephalus, Shunt, and Subglottic Stenosis in a Newborn with Plasminogen Deficiency due to Delayed Treatment with Plasminogen Concentrates: A Case Report. Neonatology. 2023 Dec 1:1-5. doi: 10.1159/000534868. Epub ahead of print. PMID: 38043518.
Pulmonary (Lungs)
- Ciftci E, Ince E, Akar N, Dogru U, Tefs K, Schuster V. Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency. Eur J Pediatr. 2003;162(7-8):462-465.
- Cohen J, Cohen S, Cymberknoh MC, Gross M, Hirshoren N, Shoseyov D. Laryngeal obstruction in congenital plasminogen deficiency. Pediatr Pulmonol. 2012;47(9):923-925.
- Kilinc AA, Tarcin G, Kurugoglu S, Schuster V, Cokugras H, Celkan T. A novel combined treatment for plasminogen deficiency with lung involvement. Pediatr Pulmonol. 2020;55(1):E1‐E3.
Gastrointestinal (Stomach)
Renal, Genitourinary (Kidneys and Reproductive Organs)
- Chi AC, Prichard E, Richardson MS, Rasenberger KP, Weathers DR, Neville BW. Pseudomembranous disease (ligneous inflammation) of the female genital tract, peritoneum, gingiva, and paranasal sinuses associated with plasminogen deficiency. Ann Diagn Pathol. 2009;13(2):132-139.
- Ciftci E, Ince E, Akar N, Dogru U, Tefs K, Schuster V. Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency. Eur J Pediatr. 2003;162(7-8):462-465.
- Kayikcioglu F, Bulbul D, Celiker S, Kucukali T. Ligneous inflammation of the cervix: a case report. J Reprod Med. 2005;50(10):801‐804.
- Magdaleno-Tapial J, Hernández-Bel P, Valenzuela-Oñate C, et al. Congenital plasminogen deficiency with long standing pseudomembranous conjunctival and genital lesions. JAAD Case Rep. 2018;5(1):44‐46.
- Pantanowitz L, Bauer K, Tefs K, et al. Ligneous (pseudomembranous) inflammation involving the female genital tract associated with type-1 plasminogen deficiency. Int J Gynecol Pathol. 2004;23 (3):292-295.