Recurrent multiple-branch retinal arteriolar occlusions in a patient with protein C deficiency
Clinical Investigations
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Abstract
A 34-year-old woman is presented who has recurrent, multiple, retinal arteriolar occlusions associated with a deficiency in protein C, a vitamin K-dependent anticoagulant. Protein C is a naturally occurring anticoagulant and if there is a deficiency, it can lead to episodes of thrombosis; the deficiency can be acquired or congenital. This is the first documented case of retinal arteriolar occlusion associated with a congenital deficiency of this protein. The mechanism for arteriolar occlusion in this patient is discussed, and whilst the role of protein C deficiency remains presumptive, it is strongly implicated.
Keywords
Public Health Documented Case Retinal Arteriolar Congenital Deficiency Arteriolar Occlusion
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References
- 1.Bertina RM, Broekmans AW, Linden IK van der, Mertens K (1982) Protein C deficiency in a Dutch family with thrombotic disease. Thromb Haemost 48:1–5Google Scholar
- 2.Bertina RM, Broekmans AW, Krommenhoek vas Es C, Wijngaarden A van (1984) The use of a functional and immunological assay for plasma protein C in the study of the heterogeneity of congenital protein C deficiency. Thromb Haemost 51:1–5Google Scholar
- 3.Brenner B, Shapira A, Bahari C, Haimovich L, Seligsohn U (1987) Hereditary protein C deficiency during pregnancy. Am J Obstet Gynecol 157:1160–1161Google Scholar
- 4.Broekmans AW, Veltkamp JJ, Bertina RM (1983) Congenital protein C deficiency and venous thromboembolism. New Engl J Med 1983 309:340–344Google Scholar
- 5.Brown GC, Magaral LE, Shields J, Goldberg RE, Walsh P (1981) Retinal arterial obstruction in children and young adults. Ophthalmology 88:18–25Google Scholar
- 6.Carroll D (1970) Retinal migraine. Headache 10:9–13Google Scholar
- 7.Clouse LH, Clomp PC (1986) The regulation of haemostasis: the protein C system. New Engl J Med 314:1298–1304Google Scholar
- 8.Coppeto JR, Lessell S, Sciarra R, Bear L (1986) Vascular retinopathy in migraine. Neurology 36:267–270Google Scholar
- 9.Esmon CT (1983) Protein C: biochemistry, physiology and clinical implications. Blood 62: 1155–1158Google Scholar
- 10.Estelles A, Garcia-Plaza I, Dasi A, Aznar J, Duart M, Sanz G, Perez-Requejo JL, Espana F, Jimenez C, Abeledo G (1984) Severe inherited homozygous protein C deficiency in a newborn infant. Thromb Haemost 52:53–56Google Scholar
- 11.Francis RB, McGehee WG (1985) Defibrination during warfarin therapy in a man with protein C deficiency. Thromb Haemost 53:249–251Google Scholar
- 12.Gitting JW (1985) Branch retinal artery occlusion in a young woman. Surv Ophthalmol 30: 52–58Google Scholar
- 13.Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C (1981) Deficiency of protein C in congenital thrombotic disease. J Clin Invest 68:1370–1373Google Scholar
- 14.Horellou MH, Conard J, Bertina RM, Samama M (1984) Congenital protein C deficiency and thrombotic disease in nine French families. Br Med J 289:1285–1287Google Scholar
- 15.Katz B (1985) Bilateral sequential migrainous ischaemic optic neuropathy. Am J Ophthalmol 99:489Google Scholar
- 16.Katz B (1986) Migrainous central retinal artery occlusion. J Clin Neuro-Ophthalmol 6: 69–71Google Scholar
- 17.Malm J, Laurell M, Dahlback B (1988) Changes in the plasma levels of vitamin K-dependent proteins C and S and of C4B-binding protein during pregnancy and oral contraception. Br J Haematol 68:437–443Google Scholar
- 18.Mammen EF, Thomas WR, Seegers WH (1960) Activation of purified prothrombin to autoprothrombin II (platelet co-factor II or autoprothrombin II-A). Thromb Diath Haemor 5:218–249Google Scholar
- 19.Mannucci PM, Owen WG (1987) Basic clinical aspects of proteins C and S. In: Bloom AL, Thomas DP (eds) Haemostasis and thrombosis, 2nd edn. Churchill Livingstone, Edinburgh Harlow New York, pp 452–464Google Scholar
- 20.Mannucci PM, Tripodi A (1987) Laboratory screening of inherited thrombotic syndromes. Thromb Haemost 57:247–251Google Scholar
- 21.Mannucci PM, Vigano S (1982) Deficiencies of protein C, an inhibitor of blood coagulation. Lancet II: 463–467Google Scholar
- 22.Mannucci PM, Vigano S, Bottasso B, Candotti G, Bozzetti P, Rossi E, Pardi G (1984) Protein C antigen during pregnancy, delivery and puerperium. Thromb Haemost 52:217Google Scholar
- 23.Marciniak E, Wilson HD, Marlar RA (1985) Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in the blood. Blood 65:15–20Google Scholar
- 24.Marlar RA (1985) Protein C in thromboembolic disease. Sem Thromb Haemost 11:387–393Google Scholar
- 25.Pabinger-Fasching I, Bertina RM, Lechner K, Niessner H, Korninger C (1983) Protein C deficiency in two Austrian families. Thromb Haemost 50: 810–813Google Scholar
- 26.Pulido JS, Lingua RM, Cristol S, Byrne SF (1987) Protein C deficiency associated with vitreous haemorrhage in a neonate. Am J Ophthalmol 104:546–547Google Scholar
- 27.Rubinstein K, Paton A (1968) Fluorescein angiography in oclusion of the retinal arteries. Trans Ophthalmol Soc UK 87:345–354Google Scholar
- 28.Russell RWR, Fytche TJ, Sanders MD (1966) A study of retinal vascular occlusions using fluorescein angiography Lancet II:821–825Google Scholar
- 29.Samama M, Horellou MH, Soria J, Conard J, Nicolas G (1984) Successful progressive anticoagulation in a severe protein C deficiency and previous skin necrosis at the initiation of oral anticoagulant therapy. Thromb Haemost 51:132–133Google Scholar
- 30.Seegers WH, Novoa E, Henry RL, Hassouna HI (1976) Relationship of new vitamin-K-dependent protein C and old autoprothrombin II-A. Thromb Res 8:543–552Google Scholar
- 31.Seligsohn E, Berger A, Abend M, Rubin L, Attias D, Zivelin A, Rapaport SI (1984) Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. New Engl J Med 310:559–562Google Scholar
- 32.Smith DB, Ens GE (1987) Protein C deficiency: a cause of amaurosis fugax. J Neurol Neurosurg Psychiatry 50:361–362Google Scholar
- 33.Stenflo J (1976) A new vitamin-K-dependent protein: purification from bovine plasma and preliminary characterisation. J Biol Chem 251:355–363Google Scholar
- 34.Troost BD (1986) Migraine. In: Duane TD (ed) Clinical ophthalmology, vol 2. Harper and Row, London, pp 1–22Google Scholar
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