TREATMENT OF PATIENTS WITH NIEMANN-PICK TYPE IS USING REPEATED AMNIOTIC EPITHELIAL-CELLS IMPLANTATION - CORRECTION OF AGGREGATION AND COAGULATION ABNORMALITIES

Variations of platelet aggregation and plasma levels of clotting facto rs V, IX, XI, and XII were studied in 5 patients with Niemann-Pick dis ease type Is in the course of a 3-year study of treatment with periodi c subcutaneous infusions of amniotic epithelial cells. Before commence ment of treatment, the concentrations of these factors were found to b e abnormal in four of five patients. It was possible to complete the s tudy protocol in only two patients. Platelet aggregation and plasma le vels of V, IX, XI, and XII clotting factors had been determined before each epithelial amniotic cells implantation and after 24, 48, and 72 hours. In both patients the aggregation test and the plasma levels of coagulation factors V, IX, XI, and XII were below the normal values of reference, Results showed that the epithelial amniotic cells treatmen t normalized platelet aggregation after each implantation in the two s tudied patients, both in terms of intensity of response (increase in l ight transmission after addition of adenosine diphosphate up to 350%) and in terms of obtaining an irreversible aggregation with 3 and 8 mu M of adenosine diphosphate, The data related to clotting factors showe d an increase of these concentrations up to 60% and some of these conc entrations normalized completely.