258 P. SOUPART 
release of this amino acid from inside the cell where it is maintained normally at 
higher concentration than that of the extracellular fluid. In this group of hyper- 
aminoaciduria may be listed: 
a) Chronic protein deficiency of the kwashiorkor type (see p. 250). 
b) Fasting effects in a normal subject (see p. 250). 
c) Radiation effects, even of moderate dosage such as in usual therapeutic X- 
irradiation (see p. 250). 
d) Miscellaneous conditions: shock, hepatic coma, vitamin C deficiency as well 
as pernicious anemia’’. In hepatic disorders, the condition may lead progressively 
to a generalized aminoaciduria due to general increase of all plasma levels and is 
correlated to the severity of the clinical state. 
2) Aminoaciduria occurring in liver disorders. That functional liver insufficiency 
increases the ratio of amino nitrogen to total nitrogen in urine has long been known, 
but it is only noticeable in very severe involvement of the liver. First investigation 
of urinary amino acids in such condition, by means of microbiological methods, led 
I5 years ago to the recognition of an increased urinary excretion involving cystine, 
histidine and tryptophane in acute and subacute cirrhosis, acute yellow atrophy, 
viral hepatitis and hepatic coma. Frequency and intensity of the hyperamino- 
aciduria are closely correlated to the severity of the condition. When such diseases 
were investigated by means of ion-exchange chromatography it appeared that hyper- 
excretion also involved methionine, lysine, phenylalanine, tyrosine, glycine, alanine, 
BAIBA, 1-methylhistidine, taurine and ethanolamine. In this way it was demon- 
strated that the urinary amino acid pattern was that of a hyperaminoaciduria of 
the generalized common type, characterized by progressive installation, the speed 
of which was related to the extent of liver injury. Frequently, but not as a rule, 
blood plasma levels of some of the amino acids are found to be increased. It has 
been observed in some cases that the increased urinary output of an amino acid 
corresponded to a high clearance, whereas in other cases the clearance was low, but 
this point still calls for further investigation. 
3) Aminoaciduria occurring in vitamin D deficiency. JONXIS AND HUISMAN were the 
first to describe a hyperaminoaciduria in rickets. The aminoaciduria* progressively 
develops into a pattern of the generalized common type apparently without 
abnormalities of the plasma levels. This situation is easily corrected by adequate 
therapy. 
4) Aminoaciduria occuring in vitamin C deficiency. Scurvy is accompanied by a 
progressive aminoaciduria of the generalized common type. It was thought at one 
time that tyrosine and phenylalanine were specifically involved, but no evidence 
supporting this opinion has been found in spite of extensive investigation done by 
H. Vis (unpublished data). On the other hand, what Vis found as a typical, constant 
and very early character is free proline and hydroxyproline excretion, a highly 
specific finding since those amino acids are never found, even as traces, in normal 
urine, associated to a hyperexcretion of taurine and BAIBA, initiated by the process 
already described (see p. 251). 
In vitamin C deficiency interstitial tissue and more precisely collagen fibers are 
especially involved, which are rich in proline and hydroxyproline. These two amino 
* See footnote page 250. 
References p. 261/262 
