Abstract
Summary The occurrence of severe hyperchloremic acidosis associated with depletion of potassium and with rickets in a child with an imperforate anus and a rectourethral fistula is reported. Data are presented suggesting that the primary cause of the acidosis was the loss of bicarbonate ions as the result of a change in the composition of the urine as it passed through the colon. Following reparative surgery, the ability of the patient to acidify the urine in response to ammonium chloride was normal. These considerations strongly suggest that the pathogenesis of the acidosis was entirely extrarenal.
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