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A 31-year-old man with Fabry disease was treated with hemodialysis because he had developed end-stage renal failure. However, hemodialysis treatment could not be continued due to uncontrolled systemic pain, and subsequently the patient received a renal transplant from his biological father who was not a carrier of Fabry disease. Good renal graft function and better pain control compared with before surgery were achieved, and the patient returned to society. Control of systemic pain, which is a primary symptom in patients with Fabry disease who develop renal failure can be improved. *Correspondence to: Yoshitaka Saito, Department of Urology, Kindai University, Faculty of Medicine, Japan, E-mail: c2008008@yahoo.co.jp Received: August 28, 2019; Accepted: September 06, 2019; Published: September 10, 2019 Introduction Half a century has passed since renal transplantation started in Japan, and its outcomes have improved remarkably. Advances in immunosuppressive drug therapy are largely responsible for this improvement, but the accumulation of experience, improvement of perioperative management, and broadening of the acceptable range of primary diseases (i.e., expanded indications) have likely contributed. According to the Annual Progress Report from the Japanese Renal Transplant Registry published by the Japanese Society for Clinical Renal Transplantation in 2017, the most common primary disease in 1,331 recipients who underwent living donor renal transplantation was chronic glomerulonephritis (346 patients, 26%); 1 of 124 (9.3%) patients with a genetic disorder or a congenital metabolic disorder had Fabry disease [1]. Fabry disease is an X-linked disorder of lipid metabolism and is one of main forms of sphingolipidoses. These are characterized by genetic deficiency of a lysosomal hydrolytic enzyme α-galactosidase A (α-Gal A) resulting in accumulation of globotriaosylceramide (GL-3), an intermediate metabolite of sphingoglycolipids, in organs throughout the body causing diverse symptoms. Patients with Fabry disease present with pain in extremities and hypohidrosis from childhood, and with renal dysfunction and cardiac dysfunction in adulthood. Recently, enzyme replacement therapy (ERT) [2,3] has been recommended as a fundamentally curative treatment. We report a case of good pain control using ERT after living donor renal transplantation in a patient with Fabry disease who was not fit to undergo maintenance hemodialysis.

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Successful pain control by enzyme replacement therapy continued after living donor renal transplantation for end-stage renal failure in a patient with Fabry disease: A case report