Download PDF
Thromb Haemost 1989; 61(03): 529-531
DOI: 10.1055/s-0038-1646629
International Committee Communications
Schattauer GmbH Stuttgart

Report on the Diagnosis and Treatment of Homozygous Protein C Deficiency

Report of the Working Party on Homozygous Protein C Deficiency of the ICTH-Subcommittee on Protein C and Protein S
Richard A Marlar
1   The Departments of Pediatrics, Biochemistry & Genetics, University of Colorado Health Sciences Center, Laboratory Services, Veterans Administration Medical Centel Denver, Colorado, USA
2   The Blood Center of Southeastern Wisconsin, Milwaukee, Wisconsin, USA
,
Robert R Montgomery
2   The Blood Center of Southeastern Wisconsin, Milwaukee, Wisconsin, USA
,
Andre W Broekmans
3   The Leiden University Hospital, Hemostasis and Thrombosis Research Unit, Leiden, The Nethertands
› Author Affiliations
Further Information

Publication History

Received 23 February 1989

Accepted 02 March 1989

Publication Date:
24 July 2018 (online)

Also available at
 PDF Download  Permissions and Reprints
 

  • References

  • 1 Marlar RA, Montgomery RR, Broekmans AW. and the Working Party. Diagnosis and treatment of homozygous protein C deficiency. J Ped 1989 (in press)
    PubMedSearch in Google Scholar
  • 2 Clouse LH, Comp PC. The regulation of hemostasis: the protein C system. N Engl J Med 1986; 314: 1298-1304
    CrossrefPubMedSearch in Google Scholar
  • 3 Griffin JH, Evatt B, Zimmerman TS, Kleiss AJ, Wideman C. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 1370-1373
    CrossrefPubMedSearch in Google Scholar
  • 4 Broekmans AW, Veltkamp JJ, Bertina RM. Congenital protein C deficiency and venous thromboembolism: a study of three Dutch families. N Engl J Med 1983; 309: 340-344
    CrossrefPubMedSearch in Google Scholar
  • 5 Marlar RA. Protein C in thromboembolic disease. Semin Thromb Hemostas 1985; 11: 387-393
    Thieme ConnectPubMedSearch in Google Scholar
  • 6 Branson HE, Marble R, Katz J, Griffin JH. Inherited protein C deficiency and coumarin-responsive chronic relapsing purpura fulminans in a newborn infant. Lancet 1983; 2: 1165-1168
    PubMedSearch in Google Scholar
  • 7 Sills RH, Marlar RA, Montgomery RR, Deshphande GN, Humbert JR. Severe homozygous protein C deficiency. J Ped 1984; 105: 409-413
    CrossrefPubMedSearch in Google Scholar
  • 8 Estellés A, Garcia-Plaza I, Dasi A, Aznar J, Duart M, Sanz G, Pérez-Requejo JL, España F, Jimenez C, Abeledo G. Severe inherited “homozygous” protein C deficiency in a newborn infant. Thromb Haemostas 1984; 52: 53-56
    PubMedSearch in Google Scholar
  • 9 Marciniak E, Wilson HD, Marlar RA. Neonatal purpura fulminans: a genetic disorder releated to the absence of protein C in blood. Blood 1985; 65: 15-20
    PubMedSearch in Google Scholar
  • 10 Wehinger H, Geiger E, Freudenberg V, Schurmann J, Alexandrakis E, Witt I. Schwerer hereditärer Protein-C-Mangel bei einem Neugeborenen mit Purpura fulminans - erfolgreiche Behandlung mit Phenprocoumon. Klin Pädiat 1985; 197: 116-120
    Thieme ConnectPubMedSearch in Google Scholar
  • 11 Rappaport ES, Speights VO, Helbert B, Trowbridge A, Koops B, Montgomery RR, Marlar RA. Protein C deficiency. South Med J 1987; 80: 240-242
    CrossrefPubMedSearch in Google Scholar
  • 12 Seligsohn U, Berger A, Abend M, Rubin L, Attias D, Zivelin A, Rapaport SI. Homozygous protein C deficiency manifested by massive thrombosis in the newborn. N Engl J Med 1984; 310: 559-562
    CrossrefPubMedSearch in Google Scholar
  • 13 Groncy PK, Oseas RS, Marlar RA, Montgomery RR. Intraperitoneal administration of prothrombin complex concentrates in the treatment of severe protein C deficiency. Blood 1985; 66 (suppl) 178 a
    PubMedSearch in Google Scholar
  • 14 Peters C, Casella JR, 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: 272-276
    PubMedSearch in Google Scholar
  • 15 Yuen P, Cheung A, Lin HJ, Ho F, Mimuro J, Yoshida N, Aoki N. Purpura fulminans in a Chinese boy with congenital protein C deficiency. Pediatrics 1986; 77: 670-676
    PubMedSearch in Google Scholar
  • 16 Pulido JS, Lingua RW, Cristol S, Byrne S. Protein C deficiency associated with vitreous hemorrhage in a neonate. Am J Ophtalmol 1987; 104: 546-547
    PubMedSearch in Google Scholar
  • 17 Petrini P, Egberg N, Segnestam K. Brother and sister with congenital protein C deficiency. Abstr Xth Int Congr on Thrombosis; Athens: 05/1988
    Search in Google Scholar
  • 18 Hinz H, Weil J, Buchman S, Azzam S, Auberger K, Beck C. Homozygoter Säugling in einer Sippe mit erblichem Protein-C-Mangel. Klin Wochenschr 1987; 65: 576-589
    CrossrefPubMedSearch in Google Scholar
  • 19 Garcia-Plaza I, Jimenez-Astorga C, Borrego D, Marty ML. Coumarin prophylaxis for fulminant purpura syndrome due to homozygous protein C deficiency. Lancet 1985; 1: 634-635
    PubMedSearch in Google Scholar
  • 20 Andrew M, Paes B, Milner R, Johnston M, Mitchell L, Tollefsen D, Powers P. Development of the human coagulation system in the full- term infant. Blood 1987; 70: 165-172
    PubMedSearch in Google Scholar
  • 21 Manco-Johnson M, Hays T, Warady BA, Marlar RA. Severe protein C deficiency in newborn infants. J Pediatr 1988; 113: 359-363
    CrossrefPubMedSearch in Google Scholar