Kidney dysfunction following adrenalectomy in autosomal dominant polycystic kidney disease complicated with primary aldosteronism: A case report

Abstract

Abstract. The present study reported a case of autosomal dominant polycystic kidney disease complicated with primary aldosteronism in a 49-year-old woman. The patient was referred for refractory hypertension. Laboratory examinations revealed low potassium and renin levels. Computed tomography indicated a right adrenal tumor and multiple renal cysts. Adrenal vein sampling revealed a high aldosterone level on the side of the tumor. The patient was diagnosed with autosomal dominant polycystic kidney disease complicated by primary aldosteronism and adrenalectomy was performed. Over the following 7 months, the estimated glomerular filtration rate decreased from 76 to 48 ml/min/1.73 m 2 , which was attributed to glomerular hyperfiltration correction induced by hyperaldosteronism remission, indicating kidney dysfunction. Clinicians must therefore monitor for the unmasking of kidney dysfunction following adrenalectomy in such cases. IntroductionAutosomal dominant polycystic kidney disease (ADPKD) is a hereditary kidney disease with a poor renal outcome. Pathogenesis involves cyst formation and growth, which leads to an increase in kidney volume and a decline in glomerular filtration rate (1). Approximately 50% of ADPKD patients finally need renal replacement therapy (1). In addition, ADPKD is sometimes complicated with hypertension (1).Epidemiologically, recent prevalence of ADPKD in Japan remains unclear. However, Higashihara et al (2) reported a prevalence of ADPKD of 117 in 1,000,000 in Japan at the end of 1994. In Western countries, ADPKD affects both sexes and all ethnicities, with an estimated frequency of between 1 in 400 to 1 in 1,000 live births (1).Primary aldosteronism (PA) is an endocrine disease that causes secondary hypertension and increases the risk of cardiovascular disease. Among cases of hypertension, Utsumi et al (3) reported that 6-11% are secondary to PA, although prevalence varies worldwide. The pathogenesis of PA is excess secretion of aldosterone from bilateral or unilateral adrenal gland or tumors (3). The early discovery and treatment of PA is a crucial clinical issue to prevent progression of atherosclerosis. The classical symptoms of PA are refractory hypertension and hypokalemia. However, those symptoms do not always occur, which makes early discovery difficult (3). Adrenalectomy is widely performed to treat adrenal tumors; however, there is increasing focus on attenuating the progression of renal dysfunction following adrenalectomy to treat PA (3-5), the mechanism of which has not been fully elucidated.The primary aims of treating ADPKD and PA are controlling hypertension and preserving kidney function. However, only a few cases of the two diseases occurring together have been reported (6,7). These reports have focused on diagnosing these disorders, however to the best of our knowledge, the effect of adrenalectomy on renal function in patients with ADPKD and PA has not been fully elucidated.The present report documents the case of a patient with ADPKD who experienced a decline in estimated...

Primary Aldosteronism in a Patient with Autosomal Dominant Polycystic Kidney Disease Associated with Polycystic Liver Disease

“…Also, there are reported 3 cases of hypokalemia associated with hypertension due to secondary hyperaldosteronism in patient with ADPKD (21)(22)(23). Therefore, imaging by abdominal CT or MRI, are recommended for the correct diagnosis.…”