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Renal Tubular Acidosis in a Case of Shwachman's Syndrome

ABSTRACT. We describe metabolic acidosis in a 15‐month‐old girl with clinical features of Shwachman's syndrome. Renal function tests indicated that the patient had type 1 renal tubular acidosis. Based on our findings and other reports of renal tubular dysfunction in patients with Shwachman's syndrome, we conclude that it is important to look for a possible renal tubular defect in this syndrome.