SICKLE CELL NEPHROPATHY

Course overview

SICKLE CELL DISEASE (SCD), IS AN AUTOSOMAL RECESSIVE HEMOGLOBIN DISORDER THAT RESULTS FROM THE REPLACEMENT OF GLUTAMATE FOR VALINE AT THE SIXTH AMINO ACID OF THE BETA-GLOBIN CHAIN. THIS REPLACEMENT RESULTS IN THE FORMATION OF HEMOGLOBIN S (HBS) TETRAMERS THAT ACCUMULATE DURING OXIDATIVE STRESS, TISSUE HYPOXIA, OR DEHYDRATION. THE CONSEQUENCE OF THIS IS RED BLOOD CELL SICKLING, EARLY DESTRUCTION OF ERYTHROCYTES, AND WIDESPREAD VASO-OCCLUSIVE EPISODES (VOC), SUBSEQUENTLY CAUSING MULTIORGAN DAMAGE. SICKLE CELL NEPHROPATHY (SCN) IS A GROUP OF RENAL COMPLICATIONS THAT INCLUDE HEMATURIA, PROTEINURIA, HYPOSTHENURIA, RENAL PAPILLARY NECROSIS, RENAL TUBULAR DISORDERS, ACUTE AND CHRONIC KIDNEY INJURY, SICKLE CELL GLOMERULOPATHY, AND RENAL MEDULLARY CARCINOMA. THIS ACTIVITY ILLUSTRATES THE EVALUATION AND TREATMENT OF SICKLE CELL NEPHROPATHY AND EXPLAINS THE ROLE OF THE INTERPROFESSIONAL TEAM IN MANAGING PATIENTS WITH THIS CONDITION.