The diagnosis of iminoglycinuria was established in two patients on th
e basis of increased urinary excretion of proline, hydroxyproline and
glycine in the presence of normal plasma concentrations of these respe
ctive compounds. Routine metabolic screening was performed in these in
fants in order to find the cause for the developmental delay observed
in one infant and the siblings deaths noted in the family of the other
. These two patients gave further support to the previous suggestion t
hat renal iminoglycinuria is a benign disorder with no recognizable di
nical pattern. tts detection, therefore, requires screening programs o
r aminoacid studies.