Thin Basement Membrane Nephropathy

[摘要] Thin basement membrane nephropathy (TBMN) is the most common cause of persistent hematuria in children and adults, the other main causes being IgA nephropathy and Alport syndrome (1–3). In addition to hematuria, patients with TBMN usually have minimal proteinuria, normal renal function, and uniformly thinned glomerular basement membranes (GBM), as determined by electron microscopy. TBMN, which affects at least 1% of the population, is a lifelong nonprogressive disorder associated with family history. TBMN was first described approximately 80 yr ago as a curable form of hemorrhagic nephritis (4). Later, many cases of this microscopic, painless hematuria with good prognosis were shown to be inherited, and this fact is emphasized in the numerous names used for the disease in the literature, such as “congenital hereditary hematuria” (5), “hereditary hematuria” (6), “familial hematuric nephritis” (7), “familial benign hematuria” (8), “benign familial hematuria” (9,10), “familial benign essential hematuria” (11,12), “benign hereditary nephritis” (13), and “benign essential hematuria” (14). TBMN has also been referred to as familial hematuria (15), but that is a misnomer, as it does not distinguish it from the progressive Alport syndrome (3). Commonly used names are “thin membrane nephropathy” (16–18), “thin basement membrane disease” (19–25), “thin GBM nephropathy” (26), and “thin GBM syndrome” (27). The term “thin basement membrane nephropathy” is currently most widely used (1,2,28–36), and this name is to be preferred, as it refers to a renal disorder that is associated with observable structural changes in the basement membrane (3) without necessarily being a “true disease.”

[学科分类] 泌尿医学

[关键词] Bone marrow necrosis;Sickle cell disease;Hyperhemolysis syndrome