Immunoglobulin A Nephropathy in a Kidney Transplant Recipient with Kabuki Syndrome

Kabuki syndrome is a rare genetic disorder that causes multiple congenital anomalies, including characteristic facial features reminiscent of Kabuki syndrome. It is often associated with congenital anomalies of the kidneys and urinary tract as well as immune abnormalities. While various autoimmune diseases have been reported in patients with this syndrome, only one case of membranoproliferative glomerulonephritis has been documented. We herein report a case of Kabuki syndrome in which immunoglobulin A nephropathy developed in a renal allograft, which subsequently improved with the administration of pulse steroids and an angiotensin II receptor blocker.