Acute tubular dysfunction with Fanconi syndrome: a new manifestation of mitochondrial cytopathies.

S b es itochondrial diseases are a heterogeneous group of energy metabolism disorders hat can affect almost any organ. Kidney disease s well-known in patients with mitochondrial isorders, most commonly manifesting as proxial tubulopathy with chronic tubular acidosis, ypically a minor feature in pediatric patients ith severe involvement of brain, liver, heart, or ther organs. Other renal presentations inlude chronic tubulointerstitial nephropathy, rogressive glomerular diseases, and nephrotic yndrome. Acute, severe, and fluctuant tubular ysfunction has not been previously described.