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Abstract
The relationship between renal tubular acidosis (RTA) and copper metabolism has been
investigated in a group of 18 patients with primary biliary cirrhosis. RTA, considered
when urinary pH remained above 5.4 after an oral load of ammonium chloride of 0.1
g/kg body wt, was found in 6 patients (33%). Plasma copper concentration (PCu) and
urinary copper excretion (UCuV) were significantly higher in patients with RTA (PCu
= 182.2 micrograms/dl, UCuV = 536.8 micrograms/24 h) than in those without (PCu =
134.2; UCuV = 170.3). Plasma copper concentration and urinary copper excretion correlated
with minimal urinary pH achieved after the ammonium chloride load. A higher degree
of cholestasis was present in patients with RTA than in those without, and a linear
correlation was observed between PCu and UCuV and serum bilirubin. It is concluded
that the increased UCuV is related to the cholestasis in primary biliary cirrhosis
and that the RTA might be caused by the deposition of copper in the distal renal tubule.