An Association of Chronic Hyperaldosteronism with Medullary Nephrocalcinosis

Abstract

SummaryBackgroundAn association between chronic hyperaldosteronism and medullary nephrocalcinosis has rarely been made, with only a handful of cases described in literature.Case ReportWe describe five cases of hyperaldosteronism with a long- standing history in whom associated medullary nephrocalcinosis was established.ConclusionsWe infer that a chronic hyperaldosteronic status, whether primary or secondary, is a causal factor in the etiopathogenesis of medullary nephrocalcinosis. This article illustrates and summarizes various postulated theories that support our proposed association between hyperaldosteronism and nephrocalcinosis. We conclude that chronic hyperaldosteronism should be included as one of the causes of nephrocalcinosis and that our case series emphasizes the need of a well-organized retrospective study to prove it further.

Infants With Congenital Adrenal Hyperplasia Are at Risk for Hypercalcemia, Hypercalciuria, and Nephrocalcinosis

“…When presented with a salt load, the reabsorption of sodium is diminished, as well as that of calcium, which is passively coupled with sodium and water [ 18 ]. It has been estimated that for every 100 mEq/dL increment of sodium excretion in the urine, there is a 40 mg/dL rise in calcium excretion [ 22 ].…”

“…Although hypercalciuria likely contributes to the development of nephrocalcinosis in children with CAH, excess mineralocorticoid supplementation might also play a role. There are three case reports of nephrocalcinosis thought to be due to hyperaldosteronism in young men (ages 15, 17, and 22 years) with CAH due to 11 β -hydroxylase deficiency [ 22 , 24 ]. All three cases were undiagnosed in childhood and presented with significant hypertension and hypokalemia.…”

“…Likewise, nephrocalcinosis and renal cysts have been reported in the setting of apparent mineralocorticoid excess syndrome, which is caused by 11 β -hydroxysteroid dehydrogenase deficiency in the kidney [ 25 ]. The pathogenesis of nephrocalcinosis due to hyperaldosteronism is unclear; however, chronic hypokalemia resulting in ammonia-mediated renal scarring has been postulated as a prime causal factor [ 22 , 25 ]. None of the patients in our study were noted to have long-standing hypokalemia and the three who developed nephrocalcinosis were all prescribed fludrocortisone within the recommended dose range of 0.05 to 0.2 mg/d [ 1 ].…”

Primary Hyperaldosteronism and Renal Medullary Nephrocalcinosis: A Controversial Association

“…There are few case reports linking PA with nephrocalcinosis and/or nephrolithiasis. 2 , 5 - 10 We report three cases where PA was possibly associated with medullary nephrocalcinosis. Postulated theories linking PA with nephrocalcinosis are also discussed.…”

“…The three commonest causes of nephrocalcinosis are PHPT, distal renal tubular acidosis (dRTA), and medullary sponge kidney. 2 , 3 The diagnosis is purely radiological and can be demonstrated on plain radiographs, ultrasound, or computed tomography (CT). The pathophysiology of nephrocalcinosis includes sustained hypercalcemia and hypercalciuria, increased tubular delivery of calcium to the interstitium, and subsequent deposition based on local conditions, including the pH and inhibitory ions.…”