Long-term management of homozygous protein C deficiency: Replacement therapy with subcutaneous purified protein C concentrate

Thrombosis and Haemostasis

Volumn 81, Issue 6, 1999, Pages 887-890

  Sanz Rodriguez, C ^a   Gil Fernandez J J ^a   Zapater, P ^a   Pinilla, I ^a   Granados, E ^a   De Soria, V Gómez G ^a   Cano, J ^b   Sala, N ^c   Fernandez Ranada J M ^a   Gómez, N Gómez ^a  

^a HOSPITAL UNIVERSITARIO DE LA PRINCESA   (Spain)

^b HOSPITAL INFANTIL UNIVERSITARIO NIÑO JESÚS   (Spain)

^c Institut de Recerca Oncologica   (Spain)

Author keywords

[No Author keywords available]

Indexed keywords

ANTICOAGULANT AGENT; PROTEIN C;

ARTICLE; CASE REPORT; CLINICAL FEATURE; DISSEMINATED INTRAVASCULAR CLOTTING; FEMALE; FULMINATING PURPURA; HUMAN; INTRAVENOUS DRUG ADMINISTRATION; NEWBORN; ORAL DRUG ADMINISTRATION; PRIORITY JOURNAL; PROTEIN BLOOD LEVEL; PROTEIN C DEFICIENCY; SKIN DEFECT; SUBCUTANEOUS DRUG ADMINISTRATION; VASCULAR ACCESS;

EID: 0033058659     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0037-1614593     Document Type: Article

References (18)

1. Marlar RA, Montgomery RR, Broekmans AW. Report on the diagnosis and treatment of homozygous protein C deficiency. Thromb Haemost 1989; 61: 529-31.
2. Sills RH, Marlar RA, Montgomery RR, Deshpande GN, Humbert JR. Severe homozygous protein C deficiency. J Pediatr 1984; 105: 409-13.
3. Marlar RA, Neumann A. Neonatal purpura fulminans due to homozygous protein C or protein S deficiency. Semin Thromb Haemostas 1990; 16: 299-309.
4. Estellés A, García-Plaza I, Dasí A, Aznar J, Duart M, Sanz G, Pérez-Requejo JL, España F, Jiménez C, Abeledo G. Severe inherited "homozygous" protein C deficiency in a newborn infant. Thromb Haemost 1984; 52: 53-6.
5. Peters C, Casella JF, Marlar RA, Montgomery RR, Zinkham WH. Homozygous protein C deficiency: observations on the nature of the molecular abnormality and the effectiveness of warfarin therapy. Pediatrics 1988; 81: 272-6.
6. Hartman KR, Manco-Johnson M, Rawlings JS, Bower DJ, Marlar RA. Homozygous protein C deficiency: early treatment with warfarin. Am J Pediatr Hematol Oncol 1989; 11: 395-401.
7. Seligsohn U, Berger A, Abend M, Rubin L, Attias D, Zivelin A, Rapaport SI. Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med 1984; 310: 559-62.
8. Auletta MJ, Headington JT. Purpura fulminans: a cutaneous manifestation of severe protein C deficiency. Arch Dermatol 1988; 124: 1387-91.
9. Dreyfus M, Magny JF, Bridey F, Schwarz HP, Planché C, Dehan M, Tchernia G. Treatment of homozygous protein C deficiency and neonatal purpura fulminans with a purified protein C concentrate. N Engl J Med 1991; 325: 1565-8.
10. Müller D-F, Ehrenthal W, Hafner G, Schranz D. Purpura fulminans in homozygous protein C deficiency: monitoring of treatment with protein C concentrate. Eur J Pediatr 1996; 155: 20-5.
11. Baliga V, Thwaites R, Tillyer ML, Minford A, Parapia L, Allgrove J. Homozygous protein C deficiency: management with protein C concentrate. Eur J Paediatr 1995; 154: 534-8.
12. Dreyfus M, Masterson M, David M, Rivard GE, Müller FM, Kreuz W, Beeg H, Thomas H, Minford A, Allgrove J, Cohen H, Jesse H, Cristoph H, Bergman F, Mitchell NE, Vivian H, Haworth C, Nelson K, Schwarz HP. Replacement therapy with a monoclonal antibody purified protein C concentrate in newborns with severe congenital protein C deficiency. Semin Thromb Hemostas 1995; 21: 371-81.
13. Goodwin TM, Gazit G, Gordon EM. Heterozygous protein C deficiency presenting as severe protein C deficiency and peripartum thrombosis: successful treatment with protein C concentrate. Obstet Gynecol 1995; 86: 662-4.
14. Reitsma PH, Bernardi F, Doig RG, Gandrille S, Greengard JS, Ireland H, Krawczak M, Lind B, Long GL, Poort SR, Saito H, Sala N, Witt I, Cooper DN. Protein C deficiency: a database of mutations, 1995 update. Thromb Haemost 1995; 73: 876-89.
15. Grandille S, Aiach M, and the French INSERM network on molecular abnormalities responsible for protein C and protein S deficiencies. Identification of mutations in 90 of 121 consecutive symptomatic French patients with type I protein C deficiency. Blood 1995; 86: 2598-605.
16. Millar DS, Wacey AI, Voke J, Kakkar VV, Cooper DN. A novel point mutation (Val297-Met) in the serine proteinase domain of protein C in a patient with venous and arterial thromboembolic disease. Blood Coagul Fibrinolysis 1993; 4: 631-3.
17. Monagle P, Andrew M, Halton J, Marlar R, Jardine L, Vegh P, Johnston M, Webber C, Massicotte MP. Homozygous protein C deficiency: description of a new mutation and successful treatment with low molecular weight heparin. Thromb Haemost 1998; 79: 756-61.
18. Minford AMB, Parapia LA, Stainforth C, Lee D. Treatment of homozygous protein C deficiency with subcutaneous protein C concentrate. Br J Haematol 1996; 93: 215-6.