教学讲义2000.doc

教学讲义（2000） Nephrotic syndrome The cardinal features of the nephritic syndrome include heavy proteinuria (defined as urine protein excretion over 3.5g/24 hours), hypoalbuminemia, edema and varying degrees of hyperlipidemia and lipiduria. The major causes of the nephritic syndrome are included as both primary glomerular disorders (minimal change, membranous nephropathy, focal segmental glomerulosclerosis, membranoproliferatie glomerulonephritis, etc) and secondary disorders (diabetes mellitus, systemic lupus erythematosus, amyloidosis, etc). Pathogenesy of nephrotic syndrome Three structural elements in the glomerular capillary wall constitute the permselectivity barrier: endothelial cells separated by fenestrae, the glomerular basement membrane made up of a network of matrix proteins, and specialized epithelial cells(podocytes) connected to each other via an interdigitating network of slit diaphragms. Normally, proteins with the size of albumin (67KDa) and larger are excluded from filtration, a restriction that depends substantially on the integrity of the slit diaphragms. In the nephritic syndrome, adjacent podocytes appear fused together to assume a flattened rather than foot-like morphology. Abnormalities arising from podocyte proteins or circulating factors can alter the selective permeability barrier. Hypoalbuminemia is in part a consequence of urinary protein loss. It is also due to the catabolism of filtered albumin by the proximal tubule as well as to redistribution of albumin within the body. This in part accounts for the inexact relationship between urinary protein loss, the level of the serum albumin, and other secondary consequences of heavy albuminuria. Hyperlipidaemia in nephritic syndrome is characterized by raised serum cholesterol levels, and to a lesser extent hypertriglyceridemia. There is increased hepatic synthesis of low density lipoprotein, very low density lipoprotein and lipoprotein in response to hypoalbuminemia, and urinary loss of high