Plasminogen Deficiency

1. Schuster V, Seregard S. Ligneous conjunctivitis. Survey of Ophthalmology. 2003;48:369-88.

2. Schott D, Dempfle C-E, Beck P, Liermann A, Mohr-Pennert A, Goldner M, et al. Therapy with a Purified Plasminogen Concentrate in an Infant with Ligneous Conjunctivitis and Homozygous Plasminogen Deficiency. New Engl J Med. 1998;339:1679-86.

3. Verhoeff FH. A case of ligneous conjunctivitis now 36 years in duration. American journal of ophthalmology. 1958;45:246-51.

4. McGrand JC, Rees DM, Harry J. Ligneous conjunctivitis. The British journal of ophthalmology. 1969;53:373-81.

5. Owen Jr C. Fibrinolysis and Thrombolysis. In: W N, E B, eds. A History of Blood Coagulation. Rochester, MN: Mayo Foundation for Medical Education and Research, 2001: 87-96.

6. Aoki N, Moroi M, Sakata Y, Yoshida N, Matsuda M. Abnormal plasminogen. A hereditary molecular abnormality found in a patient with recurrent thrombosis. J Clin Invest. 1978;61:1186-95.

7. Mingers AM, Heimburger N, Zeitler P, Kreth HW, Schuster V. Homozygous type I plasminogen deficiency. Semin Thromb Hemost. 1997;23:259-69.

8. Castellino FJ, Ploplis VA. Structure and function of the plasminogen/plasmin system. Thromb Haemost. 2005;93:647-54.

9. Sulniute R, Shen Y, Guo YZ, Fallah M, Ahlskog N, Ny L, et al. Plasminogen is a critical regulator of cutaneous wound healing. Thromb Haemost. 2016;115:1001-9.

10. Law RH, Abu-Ssaydeh D, Whisstock JC. New insights into the structure and function of the plasminogen/plasmin system. Curr Opin Struct Biol. 2013;23:836-41.

11. Miyashita C, Wenzel E, Heiden M. Plasminogen: a brief introduction into its biochemistry and function. Haemostasis. 1988;18:7-13.

12. Mehta R, Shapiro AD. Plasminogen deficiency. Haemophilia. 2008;14:1261-8.

13. Shen Y, Guo Y, Mikus P, Sulniute R, Wilczynska M, Ny T, et al. Plasminogen is a key proinflammatory regulator that accelerates the healing of acute and diabetic wounds. Blood. 2012;119:5879-87.

14. Drew AF, Kaufman AH, Kombrinck KW, Danton MJ, Daugherty CC, Degen JL, et al. Ligneous conjunctivitis in plasminogen-deficient mice. Blood. 1998;91:1616-24.

15. Tefs K, Gueorguieva M, Klammt J, Allen CM, Aktas D, Anlar FY, et al. Molecular and clinical spectrum of type I plasminogen deficiency: A series of 50 patients. Blood. 2006;108:3021-6.

16. Klammt J, Kobelt L, Aktas D, Durak I, Gokbuget A, Hughes Q, et al. Identification of three novel plasminogen (PLG) gene mutations in a series of 23 patients with low PLG activity. Thromb Haemost. 2011;105:454-60.

17. Tait RC, Walker ID, Conkie JA, Islam SI, McCall F, Mitchell R, et al. Plasminogen levels in healthy volunteers–influence of age, sex, smoking and oral contraceptives. Thromb Haemost. 1992;68:506-10.

18. Robbins KC. Dysplasminogenemias. Progress in cardiovascular diseases. 1992;34:295-308.

19. Schuster V, Hugle B, Tefs K. Plasminogen deficiency. J Thromb Haemost. 2007;5:2315-22.

20. Okamoto A, Sakata T, Mannami T, Baba S, Katayama Y, Matsuo H, 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;1:2397-403.

21. Ooe A, Kida M, Yamazaki T, Park SC, Hamaguchi H, Girolami A, et al. Common mutation of plasminogen detected in three Asian populations by an amplification refractory mutation system and rapid automated capillary electrophoresis. Thromb Haemost. 1999;82:1342-6.

22. ClinicalTrials.gov. Study of individuals affected with hypoplasminogenemia (HISTORY). https://clinicaltrials.gov/ct2/show/NCT03797495. Access date March 15, 2019.

23. Ma Q, Ozel AB, Ramdas S, McGee B, Khoriaty R, Siemieniak D, et al. Genetic variants in PLG, LPA, and SIGLEC 14 as well as smoking contribute to plasma plasminogen levels. Blood. 2014;124:3155-64.

24. Osaki T, Souri M, Song YS, Izumi N, Law R, Ichinose A. Molecular pathogenesis of plasminogen Hakodate: the second Japanese family case of severe type I plasminogen deficiency manifested late-onset multi-organic chronic pseudomembranous mucositis. Journal of thrombosis and thrombolysis. 2016;42:218-24.

25. 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;13:132-9.

26. Hidayat AA, Riddle PJ. Ligneous Conjunctivitis: A Clinicopathologic Study of 17 Cases. Ophthalmology. 1987;94:949-59.

27. Drew A, Kaufman A, Kombrinck K, Danton M, Daugherty C, Degen J, et al. Ligneous conjunctivitis in plasminogen-deficient mice. Blood. 1998;91:1616-24.

28. Hidayat AA, Riddle PJ. Ligneous conjunctivitis. A clinicopathologic study of 17 cases. Ophthalmology. 1987;94:949-59.

29. Suzuki T, Ikewaki J, Iwata H, Ohashi Y, Ichinose A. The first two Japanese cases of severe type I congenital plasminogen deficiency with ligneous conjunctivitis: Successful treatment with direct thrombin inhibitor and fresh plasma. American Journal of Hematology. 2009;84:363-5.

30. Scully C, Gokbuget AY, Allen C, Bagan JV, Efeoglu A, Erseven G, et al. Oral lesions indicative of plasminogen deficiency (hypoplasminogenemia). Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 2001;91:334-7.

31. Toker H, Toker MI, Goze F, Turgut M, Yilmaz A. A ligneous periodontitis and conjunctival lesions in a patient with plasminogen deficiency. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 2007;103:e35-8.

32. Kurtulus Waschulewski I, Gokbuget AY, Christiansen NM, Ziegler M, Schuster V, Wahl G, et al. Immunohistochemical analysis of the gingiva with periodontitis of type I plasminogen deficiency compared to gingiva with gingivitis and periodontitis and healthy gingiva. Arch Oral Biol. 2016;72:75-86.

33. Pantanowitz L. Ligneous cervicitis. BJOG : an international journal of obstetrics and gynaecology. 2004;111:635.

34. Pantanowitz L, Bauer K, Tefs K, Schuster V, Balogh K, Pilch BZ, et al. Ligneous (Pseudomembranous) Inflammation Involving the Female Genital Tract Associated with Type-1 Plasminogen Deficiency. International Journal of Gynecological Pathology. 2004;23:292-5.

35. Altinkaya SO, Uzunlar O, Talas BB, Ozat M, Bilge U. Ligneous cervicovaginitis. Taiwanese journal of obstetrics & gynecology. 2008;47:363-6.

36. Lotan TL, Tefs K, Schuster V, Miller J, Manaligod J, Filstead A, et al. Inherited plasminogen deficiency presenting as ligneous vaginitis: a case report with molecular correlation and review of the literature. Hum Pathol. 2007;38:1569-75.

37. Cohen J, Cohen S, Cymberknoh MC, Gross M, Hirshoren N, Shoseyov D. Laryngeal obstruction in congenital plasminogen deficiency. Pediatr Pulmonol. 2012;47:923-5.

38. Çiftçi E, Ince E, Akar N, Dogru Ü, Tefs K, Schuster V. Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency. European journal of pediatrics. 2003;162:462-5.

39. Shapiro AD, Nakar C, Parker JM, Albert GR, Moran JE, Thibaudeau K, et al. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency. Blood. 2018;131:1301-10.

40. Schuster V, Seidenspinner S, Zeitler P, Escher C, Pleyer U, Bernauer W, et al. Compound-Heterozygous Mutations in the Plasminogen Gene Predispose to the Development of Ligneous Conjunctivitis: Presented in part on the XIVth International Congress on Fibrinolysis and Thrombolysis, Ljubljana, June 22-26, 1998; Fibrinolysis & Proteolysis 12: 20, 1998 (abstr 54, suppl 1). Blood. 1999;93:3457-66.

41. Veres G, Korponay-Szabo I, Maka E, Glasz T, Mamula P, Schuster V, et al. Duodenal ulceration in a patient with celiac disease and plasminogen I deficiency: coincidence or cofactors? Pediatrics. 2011;128:e1302-6.

42. Oskay T, Erdem C, Anadolu R, Peksan Y, Ozsoy N, Gul N. Juvenile colloid milium associated with conjunctival and gingival involvement. Journal of the American Academy of Dermatology. 2003;49:1185-8.

43. Weinzierl MR, Collmann H, Korinth MC, Gilsbach JM, Rohde V. Management of hydrocephalus in children with plasminogen deficiency. Eur J Pediatr Surg. 2007;17:124-8.

44. Demetriades AK, Haq IZ, Jarosz J, McCormick D, Bassi S. The ventriculocholecystic shunt: two case reports and a review of the literature. Br J Neurosurg. 2013;27:505-8.

45. Diamond JP, Chandna A, Williams C, Easty DL, Scully C, Eveson J, et al. Tranexamic acid-associated ligneous conjunctivitis with gingival and peritoneal lesions. The British journal of ophthalmology. 1991;75:753-4.

46. Song Y, Izumi N, Potts LB, Yoshida A. Tranexamic acid-induced ligneous conjunctivitis with renal failure showed reversible hypoplasminogenaemia. BMJ case reports. 2014;2014.

47. Andrew M, Vegh P, Johnston M, Bowker J, Ofosu F, Mitchell L. Maturation of the hemostatic system during childhood. Blood. 1992;80:1998-2005.

48. Celkan T. Plasminogen deficiency. Journal of thrombosis and thrombolysis. 2017;43:132-8.

49. De Cock R, Ficker LA, Dart JG, Garner A, Wright P. Topical heparin in the treatment of ligneous conjunctivitis. Ophthalmology. 1995;102:1654-9.

50. Hiremath M, Elder J, Newall F, Mitchell S, Dyas R, Monagle P. Heparin in the long-term management of ligneous conjunctivitis: a case report and review of literature. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis. 2011;22:606-9.

51. Fine G, Bauer K, Al-Mohaya M, Woo SB. Successful treatment of ligneous gingivitis with warfarin. Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 2009;107:77-80.

52. Acs N, Vajo Z, Miklos Z, Siklosi G, Paulin F, Felicetta JV, et al. The effects of postmenopausal hormone replacement therapy on hemostatic variables: a meta-analysis of 46 studies. Gynecological endocrinology : the official journal of the International Society of Gynecological Endocrinology. 2002;16:335-46.

53. Teresa Sartori M, Saggiorato G, Pellati D, Casonato A, Spiezia L, Pontara E, et al. Contraceptive pills induce an improvement in congenital hypoplasminogenemia in two unrelated patients with ligneous conjunctivitis. Thromb Haemost. 2003;90:86-91.

54. Mannucci PM, Kluft C, Traas DW, Seveso P, D’Angelo A. Congenital plasminogen deficiency associated with venous thromboembolism: therapeutic trial with stanozolol. Br J Haematol. 1986;63:753-9.

55. 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. Thrombosis and Haemostasis. 2008;100:1196-8.

56. Kizilocak H, Ozdemir N, Dikme G, Koc B, Atabek AA, Cokugras H, et al. Treatment of plasminogen deficiency patients with fresh frozen plasma. Pediatric blood & cancer. 2017.

57. Nakar CT, Caputo R, Price FW, Sartori MT, Leonardi A, Suffredini AL, et al. Safety & Efficacy of Human Plasma Derived Plasminogen Ophthalmic Drops for Treatment of Ligneous Conjunctivitis: Report of Phase 2/3 Clinical Trial.  American Society of Hematology. Orlando, FL, 2015.

58. Nakar C, Hardesty B, Thukral N, Albert G, Rubinacci M, Thibaudeau K, et al. A Phase 2/3, Open-Label, Repeat Dose Study of the Pharmacokinetics, Efficacy, and Safety of Prometic Plasminogen Intravenous Infusion in Subjects with Hypoplasminogenemia.  International Society on Thrombosis and Haemostasis. Berlin, 2017.

59. Silva GB, Bariani C, Mendonca EF, Batista AC. Clinical manifestations due to severe plasminogen deficiency: a case report. Journal of dentistry for children (Chicago, Ill). 2006;73:179-82.

60. Kurtulus I, Gokbuget A, Efeoglu A, Cintan S, Tefs K, Schuster V, et al. Hypoplasminogenemia with ligneous periodontitis: a failed local therapeutic approach. Journal of periodontology. 2007;78:1164-75.

61. Cha S, Cohen D, Bhattacharyya I, Katz J. Ligneous gingivitis associated with plasminogen deficiency: a challenge in diagnosis. J Investig Clin Dent. 2011;2:207-11.

62. Galeotti A, Uomo R, D’Anto V, Valletta R, Vittucci AC, Macchiaiolo M, et al. Ligneous periodontal lesions in a young child with severe plasminogen deficiency: a case report. European journal of paediatric dentistry : official journal of European Academy of Paediatric Dentistry. 2014;15:213-4.

63. Neering SH, Adyani-Fard S, Klocke A, Ruttermann S, Flemmig TF, Beikler T. Periodontitis associated with plasminogen deficiency: a case report. BMC Oral Health. 2015;15:59.

64. Watts P, Suresh P, Mezer E, Ells A, Albisetti M, Bajzar L, et al. Effective treatment of ligneous conjunctivitis with topical plasminogen. American journal of ophthalmology. 2002;133:451-5.

65. Heidemann DG, Williams GA, Hartzer M, Ohanian A, Citron ME. Treatment of ligneous conjunctivitis with topical plasmin and topical plasminogen. Cornea. 2003;22:760-2.

66. 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;170:1333-6.

67. Hardesty BM, Plum S, Thibaudeau K, Lee M, Shapiro AD. Pharmacokinetics of Intravenous Infusion of Glu-Plasminogen Concentrate in Patients with Hypoplasminogenemia. Blood. 2015;126:3498.