Progetto di ricerca: “Paroxysmal Nocturnal Hemoglobinuria (PNH) is a rare hematological disease characterized by hemolytic anemia, bone marrow failure and thrombosis.
Some studies report kidney damage and pulmonary hypertension, in more than 50% ofpatients, due to chronic intravascular emolysis. In our thirty-year experience, from a clinical point of view, the above described organ failure, have never been observed.
The aim of our study is to evaluate, prospectively, the renal function through creatinine clearance monitoring, both in patients receiving the anti-C5 monoclonal antibody Eculizumab, that blocks the hemolysis due to the complement cascade, and in non-treated patients. Moreover, we will evaluate pulmonary function through the dosage, in urine and serum, of free radicals, FMD, spirometry with DLCO and nocturnal pulse oximetry. This will allow to confirm or not the organ failure reported in the literature and the related mechanisms”.
Titolo della ricerca:“Evaluation of renal and pulmonary function of patients with Paroxysmal Noctural Hemoglobinuria”.