2005, Number 3
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Gac Med Mex 2005; 141 (3)
Protein C congenital deficiency. A case report.
Lemus-Varela ML, Arríaga-Dávila JJ, Salinas-López MP
Language: Spanish
References: 12
Page: 229-232
PDF size: 49.93 Kb.
ABSTRACT
Protein C is a plasmatic protein that is synthesized by the liver with the help of vitamin K. It regulates thrombin formation and consequently prevents thrombosis. We present a case of a newborn male with change in the color of the right foot index finger who after 4 h showed cyanosis that reached malleolus level. Upon admission we observed generalized pallor, tachycardia and a necrotic lesion in the right foot. We suspected a septic process and thus administered cefotaxime, vancomycin and heparin. Platelet levels were 70,000mm3, thromboplastin 16/12 sec., partial thromboplastin 5829 sec. PC functionality 20% and protein S 100%. Even though the patient evolved favourably and showed partial recovery, an intratuberous amputation was needed. One year later a prosthesis was fitted. We need to carry out studies that support the use of PC monoclonal antibodies in order to offer better baseline treatment to patients with PC congenital deficiency and improve their quality of life.
REFERENCES
Best Taylor. Bases Fisiológicas de la Práctica Médica. Ed. Panamericana. Buenos Aires, 1986.
Andrew M, Brooker LA. Trastornos de la coagulación en los recién nacidos. En tratado de neonatologia de Avery. Taeusch HW, Ballard RA. Harcourt S.A. USA. 2000, PP 1045-1079.
Esmon CT, Cell mediated events that control blood coagulation and vascular injury. Annu Rev Cell Biol 1993;9:1-26 Review
Fukudome K, Esmon CT. Identification, cloning and regulation of a novel endothelial cell protein C/ activated protein C receptor. J Biol Chem 1994;269:26486-26491.
Rezaie AR, Cooper ST, Church FC, Esmon CT. Protein C inhibitor is a potent inhibitor of the thrombin-thrombomodulin complex. J Biol Chem 1995;270:25336-25339.
Heeb MJ. España F, Griffin JH. Inhibition and complexation of activated protein C by two major lnhibitors in plasma. Blood 1989;73:446-454.
Montgomery RR, Scott JP. Enfermedades hemorrágicas y trombóticas. En: Tratado de Pediatría de Nelson, (Ed) McGraw-Hill Inc 16a Edición Philadelphia Pennsylvania USA, 2000, pp 1645-1077.
Edstrom CS, Christensen RD, Andrew M. Developmental aspects of blood hemostasis and disorders of coagulation and fibrinikysis in the neonatal period. En: Christentsen RD. Hematologic problems of the neonate. WB Saunders. Philadelphia Pennsylvania 1st. edition, 2001, pp 239-271.
Ruiz-Arguelles GJ, González-Estrada S, Garces-Eisele J. Primary trombophilia in Mexico: a prospective study. Am J Hematol 1999;60:1-5.
Dreyfus M, Masterson M, David M, Rivard GE. Replacement therapy with a monoclonal antibody purified protein C concentrate in newborns with severe congenital protein C deficiency. Semin thromb hemost.1995;21:371-381.
Pescatore SL. Clinical management of protein C deficiency. Expert Opin Pharmacother. 2001;3:431-439.
Hattenbach LO, Beeg T, Kreuz W, Zubcov A. Ophthalmic manifestation of congenital protein C deficiency. J AA POS 1999;3:188-190.