Purpose: Abnormalities in renal tubular function have been observed in
hydronephrotic urinary tract disease, resulting in metabolic acidosis
, hyperkalemia and excessive free water diuresis. The frequency of the
se abnormalities, particularly in our infant population, was the impet
us for our study. Materials and Methods: We studied 50 infants selecte
d from 199 patients followed for hydronephrosis before any surgical in
tervention during a 5-year period. Mean patient age was 1.5 +/- 1.0 mo
nths at the time of diagnosis by ultrasound, voiding cystourethrograph
y and a radionuclide renal scan. Lesions were classified as unilateral
or bilateral and graded according to severity of renal pelvic dilatat
ion or grade of vesicoureteral reflux. Results: At least 1 abnormality
of tubular function was present in 29 patients (58%) of whom the pred
ominant abnormality was renal tubular acidosis in 23 (79%, 46% of the
total study group). Renal tubular acidosis was diagnosed on the basis
of a serum total carbon dioxide of 19 mM./l. or less with urinary pH 5
.5 or greater. The defect appeared to be distal in most cases. Other a
bnormalities included defects in urinary concentrating ability in 10 p
atients (4 with unilateral urinary tract dilatation). Distal tubular a
ldosterone resistance in 6 patients (3 with unilateral dilatation) was
demonstrated by hyperkalemia with a low transtubular potassium gradie
nt of 3 or less and low fractional excretion of potassium. Although co
mmon in unilateral lesions, renal tubular dysfunction became more prev
alent with an increase in severity score and bilaterality. Conclusions
: Renal tubular dysfunction is frequent in hydronephrotic infants with
unilateral or bilateral disease. Although rarely life threatening and
usually self-limiting, the metabolic consequences of these abnormalit
ies require investigation to allow for appropriate medical management.