276 K. SCHREIER 
of Naand H,O in the body fluid?!*, 214, Virus infections and fever due to other etiology , 
apparently disturb the regulation of water and amino acid distribution. 
4. Function of the liver 
Studies on the excretion of amino acids in urine by adult patients with mild to severe 
liver diseases have been published in great numbers?!°-???, The various hepatopathies 
in childhood are equally well studied?*8. Less is known concerning the influence of 
liver disorders in the post-natal period. To summarize, one can state that in chronic 
hepatopathy and especially in liver cirrhosis no significant hyperaminoaciduria is 
detectable. After a protein load, methionine, histidine, glycine etc. are usually 
excreted in greater amounts by these patients than by healthy individuals. In 
acute hepatitis, a hyperaminoaciduria of the overflow type is present in all age 
groups and particularly in infants. This type is modified only by the functional 
state of the kidneys. In acute yellow atrophy of the liver, the high excretion of 
tyrosine and leucine is one of the oldest known biochemical signs of this disorder 
(FRERICHS). 
5. Function of the kidneys 
Pathological alterations of glomerula generally produce no marked changes in amino 
acid excretion*4; 225, In relatively compensated cases of the nephrotic syndrome of 
childhood one does not find a significant hyperaminoaciduria?**, 226", However, in the 
acute phase mainly leucine, tyrosine, phenylalanine, arginine and tryptophane are 
excreted in higher amounts than normally?!®, 227-239. A pronounced hyperamino- 
aciduria is often the biochemical sign of disturbances of tubular function. Especially 
the so-called tubular necrosis is characterized by an excessive amount of amino acids 
in the urine?*!. When only a part of the nephrons is involved, the final urine shows 
only a slight or moderate hyperaminoaciduria; this is due to dilution by addition of 
normal urine. If inflammatory or degenerative changes lead to a sclerosis and hya- 
linization of the nephron the hyperaminoaciduria may disappear completely as is the 
case in prematures, when the effective renal plasma flow is so much decreased, that a 
pseudonormal amino acid excretion results. A familiar disorder corresponding to 
renal glucosuria has been described for amino acids?3?; 283, 
6. Presence of malignant diseases 
More or less marked hyperaminoacidurias have been reported in various malignancies. 
Most of these studies deal with individual cases in which no autopsy was performed. 
Therefore, it is impossible to say whether or not the liver or the kidney was involved. 
Only in leukemia have many cases been studied thoroughly. We have done follow- 
up studies on 20 cases using paper-chromatographic and to some extent micro- 
biological methods. The results were extremely variable. Later stages of the disease 
exhibited a massive excretion of S-aminoisobutyric acid (also found by others”**) and 
frequently an increase of alanine, glycine, phenylalanine, arginine, and other amino 
acids. f-Aminoisobutyric acid on the other hand has been found in the urine of 
approx. 5°% of the normal population?®>—?87, 
References p. 279/283 
