Unit:
ΠΜΣ Θρόμβωση-Αιμορραγία-Ιατρική των ΜεταγγίσεωνLibrary of the School of Health Sciences
Supervisors info:
Στυλιανή Κοκόρη, Επίκουρη καθηγήτρια αιματολογίας, Τμήμα Ιατρικής, Ε.Κ.Π.Α
Ελισάβετ Γρουζή, Αιματολόγος, Αιματολογικό Εργαστήριο – Νοσοκομειακή Υπηρεσία Αιμοδοσίας ΠΓΝ «ΑΤΤΙΚΟΝ»
Σερένα Βαλσάμη, Λέκτορας, Τμήμα Ιατρικής, Ε.Κ.Π.Α
Original Title:
Επίδραση της ελεύθερης αιμοσφαιρίνης στη νεφρική λειτουργία - Παροξυντική νυκτερινή αιμοσφαιρινουρία και νεφρική συμμετοχή
Translated title:
Influence of the free hemoglobin in renal function - Paroxysmal nocturnal hemoglobinouria and renal involvement
Summary:
Paroxysmal nocturnal hemoglobinuria (PNH) is a clonal hematopoietic stem cell disorder that manifests with hemolytic anemia, bone marrow failure, and thrombosis. PNH is the result of a somatic mutation in PIGA, an X-linked gene whose product is required for the first step in GPI anchor biosynthesis. Two of the missing GPI anchored proteins (CD55 and CD59) regulate complement. The absence of CD55 and CD59 in PNH is responsible for the intravascular haemolysis which is a classic characteristic of the disease. The red blood cells are destroyed and hemoglobin be released. The free hemoglobin connects with proteins such as haptoglobin, hemopexin and alboumin to ensure that renal is protected from the toxic effects of hemoglobin. In excessive hemolysis free hemoglobin is absorbed into the proximal tubular cells and causes damage. The proposed mechanisms include oxidation, mitochondrial damage, apoptosis, iflammation and fibrosis. Acute renal failure (ARF) and chronic kidney disease (CKD) are well-recognized complications of paroxysmal nocturnal
hemoglobinuria (PNH).
Main subject category:
Health Sciences
Keywords:
Paroxysmal nocturnal hemoglobinuria, Renal damage, Acute renal failure, Chronic kidney disease
Number of references:
150
