Abstract
Twelve children with primary renal tubular acidosis (RTA) studied with HCO3− titration and NH4Cl loading have been observed 1½–8 yrs. These include 7 ♂ with proximal RTA (PRTA) dx'd at 7–18 mos, and 3 ♂ and 2 ♀ with distal RTA dx'd at 3–13 yrs. Bone disease and/or nephrocalcinosis were present in DRTA but not PRTA. GFR (CIN) at onset was normal or slightly decreased in PRTA, but 40–60% of normal in DRTA. GFR is now normal in all patients. With rx pts had accelerated growth, reaching at least the 3rd percentile in 2–3 yrs, with some achieving the 50th percentile. Five with PRTA have normal blood pH and HCO3 off rx, 5–8 yrs after dx. Two remain on rx <2 yrs after dx. The acidification defect in pts with DRTA persists. Thus PRTA appears to be a self-limited disease of bicarbonate reabsorption in male infants, characterized by growth retardation and acidemia, without bone disease and nephrocalcinosis. DRTA appears to be a permanent defect in urinary acidification, with acidemia, growth retardation, bone disease, and nephrocalcinosis. Response to rx in both groups is good, with marked improvement in growth, and normalization of GFR in DRTA despite severe degrees of initial nephrocalcinosis. On the basis of these observations, it seems likely that Light-woods's syndrome was proximal RTA—not distal RTA as has been considered heretofore.
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