We describe a girl with lupus nephritis who presented with distal renal tub
ular acidosis and hyporeninemic hypoaldosteronism. While distal tubular dys
function is well recognized in adult systemic lupus erythematosus (SLE). on
ly a few pediatric patients have been reported. Evaluation of five pediatri
c patients with SLE revealed that distal tubular dysfunction in childhood a
nd adolescence is rare.