The Renal Aminoacidurias

Abstract

Abstract The normal mechanisms whereby the renal tubules handle amino acids are reviewed as a basis for interpretation of the physiologic causes of the renal aminoacidurias. Under normal circumstances, the renal tubules reabsorb in excess of 93% of the amino acids filtered from the plasma. When the filtered load of amino acids is increased, as by intravenous injection of amino acids, there is an increase in both the amounts reabsorbed and those excreted, but the ability of the renal tubule to respond to an increased filtered load of amino acids is so great that a maximum rate of reabsorption has not been found in the human. However, the tubule does exhibit selectivity with respect to the reabsorption of each of the naturally occurring L-amino acids; furthermore, there is a much greater efficiency of reabsorption of the L as compared to the D isomers of the individual amino acids. The excretion of amino acids is influenced to an important degree by the glomerular filtration rate, as exemplified by studies in pregnant subjects as well as patients with renal disease affecting glomerular filtration. Several renal aminoacidurias of clinical interest are discussed with special reference to their diagnostic and metabolic significance.