Abstract 32

Extensive laboratory investigation into several pediatric diseases has shown that the hemostatic mechanism is very often deeply involved. As far as Protein C is concerned we found it permanently decreased in portal vein thrombosis (A) and temporarily in chronic diarrhea (B) and in veno occlusive diseases (C).

  • A) 29 subjects who had umbilical vein catheterization during the first days of life were examined at 11th year (mean age). All the patients had developed portal hypertension, splenomegaly, esophageal varicoses and low neutrophil and platelet counts. Both protein C biological activity and antigen were lower than normal, 42% and 51% respectively. Vitamin K administration was unsuccessful and the values remained unchanged during 4 years of follow-up.

  • B) In 7 patients (mean age 12 months) affected by chronic diarrhea. Protein C biological activity was 37% and Protein C antigen was 45%. These subjects had several deep vein thrombosis episodes in connection with central venous line. The follow up of this group showed that the level of Protein C became normal as soon as they recovered from the diarrhea. Vitamin K administration did not modify the hemostatic balance in these patients.

  • C) In the third group of 58 patients submitted to bone marrow transplantation, 12/58 have been reported to have venous occlusive disease. Protein C biological activity was statistically lower in patients with VOD at the 7th and 14th day after bone marrow transplantation.

In all groups the Protein C deficiency was correlated with a temporary or transient production. The low level of Protein C implies important and different therapeutical approaches such as permanent anticoagulant treatment in patients with portal vein thrombosis and temporary anticoagulant therapy as long as the chronic diarrhea lasts.