Natural Inhibitors and Lipids in Patients with Sickle Cell Disease.

Microvascular occlusion in sickle cell disease (SCD) is a multifactorial process. Disordered coagulation may play a role in the pathogenesis of vaso occlusive crisis (VOC). The aim of this study was to evaluate the patients and to investigate their Protein C (PC), Protein S (PS) and AT-III levels during normal and crisis periods. A total of 18 patients with SCD were included in this study at the Antalya State Hospital, Thalassemia Center. The mean number of VOC episodes of the patients per year was 4.1 - 3.2. Complications in patients included 4 cases of osteonecrosis (23.5%), 2 cases of holealithiasis (11.7%), 2 cases of leg ulcers (11.7%), and 3 splenectomies (17.6%). The patients during noncrisis periods have lower cholesterol and higher triglycerides levels than the controls (p< 0.001). Hepatic and renal functions were normal in all patients. The mean totals of the PS, PC and AT-III levels were statistically lower both in non crisis and in crisis periods than the control (p< 0.001), but there was no statistical difference between the levels durining noncrisis and crisis periods. In conclusion, PC, PS and AT-IIII deficiencies in patients with SCD are certain. However, these deficiencies do not change during noncrisis and crisis situations and does not play a role on the period of crisis. Abnormal lipid patterns may be a predisposing condition for a crisis.

Intravenous gamma-globulin treatment in a patient with subacute sclerosing panencephalitis.

A 10-year-old boy with subacute sclerosing panencephalitis was treated with intravenous gamma-globulin and inosiplex and followed for 18 months. Clinical improvement, demonstrated by decreasing scores on the Neurologic Disability Index, was observed. There were no side effects. We recommend intravenous immune globulin as an alternative therapy in the treatment of subacute sclerosing panencephalitis.