Şinasi Özsoylu

High-Dose Intravenous Corticosteroid for a Patient with Diamond-Blackfan Syndrome Refractory to Classical Prednisone Treatment

A 2.5-year-old boy with congenital pure red cell aplasia (PRCA) became unresponsive to conventional prednisone (2 mg/kg) treatment following a febrile period related to diphteria-pertussis-tetanus (DP

Effect of Intravenous Adrenalin Infusion and Corticosteroid Treatment in Patients with von Willebrand’s Disease

Intravenous adrenalin infusion (5 micrograms/kg, at most 160 micrograms in 10 min) normalized partial thromboplastin time (PTT) and ristocetin induced platelet aggregation (RIPA) in 4 of the 14 patients with von Willebrands disease (VWD) in a short period of time. Although mean factor VIII (F-VIII) procoagulant activity was almost doubled 5 min following infusion, this was mainly observed in patients with relatively high baseline (> 2%) AHF activity. Mean F-VIII procoagulant activity rose by more than 100% following 10 days of corticosteroid treatment (deltacortil 2 mg/kg/day, at most 60 mg/day). PTT became normal in 6 of the 11 patients, but RIPA normalized only in 2. The improvement of RIPFA did not correspond to bleeding time in every patient. These results may suggest that if the baseline AHF activity is relatively high (greater than or equal to 9.5%), corticosteroid could be tried before schedule surgical intervention in patients with VWD.