Autosomal Dominant Polycystic Kidney Disease

A 48-year-old man presented for evaluation of the transplantation of a kidney from a living donor. He had a 14-year history of autosomal dominant polycystic kidney disease with progression to end-stage renal disease that required hemodialysis. Initially, transplantation was not possible because his voluminous, bilateral polycystic kidneys did not leave any space in his abdomen (Panel A). He underwent bilateral nephrectomy; the removed kidneys weighed a total of 22 kg (48.4 lb; 21.6% of his total body weight), and the larger right kidney measured 51 cm (Panel B). Three months after the nephrectomy, he underwent successful kidney transplantation from a living related donor. At the 2-year follow-up, the patient had normal kidney function and a serum creatinine level of 1.27 mg per deciliter (112 μmol per liter).