Glomerular filtration and shrunken pore syndrome in children and adults.

A major function of the kidney is to, by glomerular filtration, maintain the overall steady-state of 5-30 kDa proteins, many of which are signalling molecules. This function of the kidney has been overlooked, since predominantly low-molecular-mass substances <1 kDa have been used to measure or estimate glomerular filtration rate (GFR). The use of cystatin C (13 kDa) as a marker of GFR has allowed the discovery that the filtration of 5-30 kDa molecules can be selectively impaired defining the shrunken pore syndrome. The discovery, pathophysiology, morbidity (mainly cardiovascular manifestations) and mortality of this syndrome are described.