Kidney transplant in Gitelman's syndrome. Report of the first case

Abstract

This paper is the first report of a patient with Gitelman's syndrome who, after developing a chronic nephropathy leading to end-stage renal disease, underwent kidney transplantation. The clinical findings of this disease, which include hypokalemia, high angiotensin II and aldosterone levels, sustained hyporesponsiveness to the pressor action of angiotensin II and norepinephrine, normo/hypotension and hypovolemia; the clinical course after kidney transplantation highlights the importance and the need for carefully controlling the hemodynamic status of these patients. In fact, the persistence of normo/hypotension and hypokalemia, in the presence of increased levels of Ang II and aldosterone to which the transplanted kidney should normally respond, raises interesting questions about the mechanisms responsible for the regulation of patient's vascular tone and potassium homeostasis after transplantation, which could expose the patient to post-transplant hypoperfusive renal failure and its long-term complications.