Advertisement

Der homozygote Typ-I-Plasminogenmangel: Klinik und Therapie

  • A.-M. Mingers
Conference paper

Zusammenfassung

Ein homozygoter Typ-I-Plasminogen(Plg)-Mangel, d.h. das Fehlen der Plg-Aktivität infolge fehlenden Plg-Antigens, war beim Menschen bis zu unserer Erstbeobachtung 1994 [21–23] in der Weltliteratur nicht beschrieben worden. Dieser Hämostasedefekt wurde wohl darum nicht eher diagnostiziert, weil er offensichtlich ein gänzlich anderes Krankheitsbild verursacht, als nach unserer bisher allgemein üblichen Betrachtungsweise des Hämostasesystems erwartet wird.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. 1.
    Aoki N (1984) Genetic abnormalities of fibrinolytic system. Semin Thromb Hemost 10: 42–50PubMedCrossRefGoogle Scholar
  2. 2.
    Astedt B, Pandolfi M (1971) Ontogenesis of tissue plasminogen activator in human. Thromb Diathes Haemorrh 25: 469–480Google Scholar
  3. 3.
    Bateman B, Pettit RH, Isenberg SJ, Simons KB (1986) Ligneous conjunctivitis: An autosomal recessive disorder. J Pediatr Ophthal Strabismus 23: 137–140Google Scholar
  4. 4.
    Berlin AJ, Carim M, Langston RHS, Price RL (1982) Scleral grafting in the management of ligneous conjunctivitis. Ophthalmic Surg 15: 288–291Google Scholar
  5. 5.
    Bilezikan SB, Nossel HL (1977) Unique pattern of fibrinogen cleavage by human leukocyte proteases. Blood 50: 21–28Google Scholar
  6. 6.
    Bleyer WA, Hakami N, Shepard TH (1971) The development of hemostasis in the human fetus and newborn infant. Pediatr 79: 838–853CrossRefGoogle Scholar
  7. 7.
    Bugge TH, Flick MJ, Daugherty CC, Degen JL (1995) Plasminogen deficiency causes severe thrombosis but is compatible with development and reproduction. Genes Dev 9: 794–807PubMedCrossRefGoogle Scholar
  8. 8.
    Carmeliet P, Schoongans L, Kieckens L et al. (1994) Physiological consequences of loss of plasminogen activator gene function in mice. Nature 368: 419–424PubMedCrossRefGoogle Scholar
  9. 9.
    Cohen SR (1990) Ligneous conjunctivitis: an ophthalmic disease with potentially fatal tracheobronchial obstruction. Laryngeal and tracheobronchial features. Ann Otol Rhinol Laryngol 99: 509–512PubMedGoogle Scholar
  10. 10.
    Eagle RC, Brooks JSJ, Katowitz JA, Weinberg JC, Perry HD (1986) Fibrin as a major constituent of ligneous conjunctivitis. Am J Ophthalmol 101: 494–495Google Scholar
  11. 11.
    Ekelund H, Hedner U, Ástedt B (1970) Fibrinolysis in human foetuses. Acta Paediatr Scand 59: 369–376PubMedCrossRefGoogle Scholar
  12. 12.
    Gramse M, Bingenheimer C, Havemann K (1980) Degradation of human fibrinogen by chymotrypsin-like neutral protease from human granulocytes. Thromb Res 19:201–209Google Scholar
  13. 13.
    Gramse M, Bingenheimer C, Schmidt W, Egbring R, Havemann K (1978) Degradation products of fibrinogen by elastase-like neutral protease from human granulocytes. J Clin Invest 61: 1027–1033PubMedCrossRefGoogle Scholar
  14. 14.
    Heiden M, Seitz R, Egbring R (1996) The Role of Inflammatory Cells and their Proteases in Extravascular Fibrinolysis. Semin Thromb Heamost 22: 497–501CrossRefGoogle Scholar
  15. 15.
    Heimburger N, Stief TW, Römisch J (1990) Entzündungsreaktionen und Hämostase: Wechselwirkungen. In: Tilsner V, Matthias FR (Hrsg) Infektion, Entzündung und Blutgerinnung. Editiones Roche & Basel Grenzach-Wyhlen, S 3–17Google Scholar
  16. 16.
    Hidayat AA, Riddle PJ (1987) Ligneous conjunctivitis: A clinico-pathologic study of 17 cases. Ophthalmology 94: 949–959PubMedGoogle Scholar
  17. 17.
    Jochum M (1994) Leukozytenproteinasen und Zytokine bei akuten Entzündungen. In: Hopt UT, Büsing M, Becker HD (Hrsg) Akute Pankreatitis-Transplantationspankreatitis. Karger, Basel, S 82–93Google Scholar
  18. 18.
    Jochum M, Machleidt W, Neuhof H, Fritz H (1993) Pathophysiology of shock, sepsis, and organ failure. In: Schlag G, Redl H (eds) Pathophysiology of shock, sepsis, and organ failure. Springer, Berlin Heidelberg New York Tokyo, pp 46–60Google Scholar
  19. 19.
    Kramer MD, Schaefer B, Reinartz J (1995) Plasminogen activation by human keratino- cytes: Molecular pathways and cell-biological consequences. Biol Chem Hoppe Seyler 376: 131–141Google Scholar
  20. 20.
    Lijnen HR, Carmeliet P, Bouché A, Moons L, Ploplis VA, Plow EF, Collen D (1996) Restoration of thrombolytic potential in plasminogen-defkient mice by bolus administration of plasminogen. Blood 88: 870–876PubMedGoogle Scholar
  21. 21.
    Mingers AM, Heimburger N, Lutz E (1995) Gerinnungsbefunde vor und nach Lys-Plas- minogen-Infusion bei homozygotem Typ-I-Plasminogen-Mangel. Infusionsther Transfu- sionsmed 22: 16Google Scholar
  22. 22.
    Mingers AM, Heimburger N, Lutz E (1996) Familiärer homozygoter und heterozygoter Typ-I-Plasminogenmangel. In: Scharrer I, Schramm W (Hrsg) 25. Hämophilie-Sympo- sium, Hamburg 1994. Springer, Berlin Heidelberg New York Tokyo, S 96–104Google Scholar
  23. 23.
    Mingers AM, Heimburger N, Lutz E, Seeger J (1995) Homocygotic and heterocygotic plasminogen tpye I deficiencies combined with ligneous conjunctivitis in three unrelated families. XHIth Meeting of the International Society of Heamotology, Istanbul. Turk J Haematol 14 [Suppl 1]: 58 (Abstract)Google Scholar
  24. 24.
    Niederhoff H, Künzer W (1985) Physiologie der Hämostase der Neugeborenen. Monatsschr Kinderheilkd 133: 130–136PubMedGoogle Scholar
  25. 25.
    Nüssgens Z, Roggenkämper P (1993) Ligneous conjunctivitis (ten years follow up). Ophthal Paediatr Genet 14: 137–140CrossRefGoogle Scholar
  26. 26.
    Parunovic A, Stefanovic D (1993) Conjunctivitis lignosa i gidrotsefalia (neobychnaia assotsiatsiia dvukh boleznei). Vestn Oftalmol 109: 30–31PubMedGoogle Scholar
  27. 27.
    Pfannkuch E, Schmidt R, Schmidt B, Seiler T (1987) Morphologische Untersuchungen zur Pathogenese der Conjunctivitis lignosa. Klin Monatsbl Augenheilkd 190: 40–45PubMedCrossRefGoogle Scholar
  28. 28.
    Ploplis VA, Carmeliet P, Vazirzadeh S, Van Viaenderen J, Moons L, Plow EF, Collen D (1989) Effects of disruption of the plasminogen gene on thrombosis, growth, and health in mice. Circulation 92: 2585–2593Google Scholar
  29. 29.
    Plow EF, Herren T, Redlitz A, Miles LA, Hoover-Plow JL (1995) The cell biology of the plasminogen system ( Review ). FASEB J 9: 939–945Google Scholar
  30. 30.
    Rubin A, Buck D, MacDonald MR (1989) Ligneous conjunctivitis involving the cervix. Br J Obstet Gynaecol 96: 1228–1230PubMedCrossRefGoogle Scholar
  31. 31.
    Schäfer BM, Maier K, Eickhoff U, Todd RF, Kramer MD (1994) Plasminogen activation in healing human wounds. Am J Pathol 144: 1269–1280PubMedGoogle Scholar
  32. 32.
    Schäfer BM, Stark HJ, Fusenik NE, Todd III RF, Kramer MD (1995) Differential expression of urokinase-type plasminogen activator (uPA), its receptor (uPA-R), and inhibitor type-2 (PAI-2) during differentiation of keratinocytes in an organotypic coculture system. Exp Cell Res 220: 415–423CrossRefGoogle Scholar
  33. 33.
    Schott D, Dempfle CE, Beck P et al. (1997) Plasminogen deficiency - successful treatment of conjunctivitis lignosa and prevention of blindness by substitution with lys-plasminogen. Presented to the 41. Kongress der Gesellschaft für Thrombose- und Hämostaseforschung, WienGoogle Scholar
  34. 34.
    Schutta HS, Williams EC, Baranski BG, Sutula TH (1991) Cerebral venous thrombosis with plasminogen deficiency. Stroke 22: 401–405PubMedCrossRefGoogle Scholar
  35. 35.
    Scurry J, Planner R, Fortune DM, Lee CS, Rode J (1993) Ligneous (pseudomembranous) inflammation of the female genital tract. A report of two cases. J Reprod Med 38: 407–412PubMedGoogle Scholar
  36. 36.
    Shigekiyo T, Uno Y, Tomonari A, Satoh K, Hondo H, Ueda S, Saito S (1992) Type I congenital plasminogen deficiency is not a risk factor for thrombosis. Thromb Haemost 67: 189–192PubMedGoogle Scholar
  37. 37.
    Tait RC, Walker ID, Conkie JA, Islam SIAM, McCall F (1996) Isolated familial plasminogen dificiency may not be a risk factor for thrombosis. Thromb Haemost 76: 1004–1008PubMedGoogle Scholar
  38. 38.
    Tilsner V (1990) Klinische Erfahrungen bei der Anwendung von Lys-Plasminogen zur Behandlung von arteriellen Gefäßverschlüssen. Ellipse 22: 301–306Google Scholar
  39. 39.
    Vassalli JD, Saurat JH (1996) Cuts and scrapes? Plasmin heals! Nature Medicine 2:284– 285CrossRefGoogle Scholar
  40. 40.
    Weißbach G (1991) Physiologie der Hämostase. In: Weißbach G (Hrsg) Blutgerinnungsdiagnostik in der Klinik. Gesundheit, Berlin, S 20Google Scholar
  41. 41.
    Weitz JI, Silverman EK, Thong B, Campbell EJ (1992) Plasma levels of elastase-specifk fibrinopeptides correlate with inhibitor phenotype. J Clin Invest 89: 733–766CrossRefGoogle Scholar
  42. 42.
    Zilliacus H, Ottelin AM (1966) Blood clotting and fibrinolysis in human foetuses. Biol Neonat 10: 108–112PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1998

Authors and Affiliations

  • A.-M. Mingers

There are no affiliations available

Personalised recommendations