THIN BASEMENT MEMBRANE NEPHROPATHY (BENIGN FAMILIAL HEMATURIA)

Course overview

THIN BASEMENT MEMBRANE DISEASE OR THIN BASEMENT MEMBRANE NEPHROPATHY (TBMN) IS A GENETIC DISORDER AFFECTING THE GLOMERULAR BASEMENT MEMBRANE. TBMN IS CHARACTERIZED BY MICROSCOPIC HEMATURIA, USUALLY WITH NORMAL KIDNEY FUNCTION AND WITHOUT PROTEINURIA. TBMN IS CAUSED BY MUTATIONS IN GENES ENCODING TYPE IV COLLAGEN, RESULTING IN THE EXTENSIVE THINNING OF THE GLOMERULAR BASEMENT MEMBRANE. WHILE TBMN DOES HAVE OVERLAPPING FEATURES WITH ALPORT SYNDROME, THE COURSE AND PROGNOSIS OF THE TWO DISEASES DIFFER SIGNIFICANTLY, AND EACH REQUIRES DIFFERENT MANAGEMENT STRATEGIES. A DEFINITIVE DIAGNOSIS OF TMBN IS NEEDED TO OPTIMIZE PATIENT OUTCOMES. THIS ACTIVITY REVIEWS THE PATHOGENESIS, EVALUATION, AND MANAGEMENT OF TBMN AND DISCUSSES THE INTERPROFESSIONAL TEAM'S ROLE IN EVALUATING AND TREATING PATIENTS WITH THIS CONDITION.