Calò Lorenzo A, Marchini Francesco, Davis Paul A, Rigotti Paolo, Pagnin Elisa, Semplicini Andrea
Department of Clinical Medicine, 4th Medical Clinic, Medical and Surgical Sciences, Padova Hospital, Italy.
J Nephrol. 2003 Jan-Feb;16(1):144-7.
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.
本文首次报道了一名患有吉特曼综合征的患者，该患者在发展为导致终末期肾病的慢性肾病后接受了肾移植。这种疾病的临床特征包括低钾血症、血管紧张素II和醛固酮水平升高、对血管紧张素II和去甲肾上腺素的升压作用持续低反应性、正常血压/低血压和血容量不足；肾移植后的临床病程突出了仔细控制这些患者血流动力学状态的重要性和必要性。事实上，在血管紧张素II和醛固酮水平升高（移植肾通常应对此作出反应）的情况下，正常血压/低血压和低钾血症持续存在，这引发了关于移植后患者血管张力调节和钾稳态机制的有趣问题，这些问题可能使患者面临移植后低灌注性肾衰竭及其长期并发症。
