尿管異所開口から残存萎縮尿管への感染を発症した生体腎移植後のKabuki症候群の1例

Abstract

A 24-year-old woman with a high fever presented at our hospital. She had been diagnosed with Kabuki syndrome at the age of 4 years because she had the typical facial features of the condition ; she had undergone living donor renal transplantation 12 years prior. She was prescribed a course of antibiotics to treat pyelonephritis of the transplanted kidney and the high fever disappeared, but the fever developed again 3 days after the discharge. Abdominal computed tomography revealed a tubular structure of recent onset running from the left dorsal side to the lower part of the bladder. This structure was filled with pus, which we drained. We also performed laparoscopic ureterectomy of the left ureter to achieve a complete cure. No complication was observed after the surgery and the graft renal function did not deteriorate further.