The Association of Aldosterone-Producing Adrenal Adenoma in a Patient With Autosomal Dominant Polycystic Kidney Disease

Hypertension is a frequent early manifestation of autosomal dominant polycystic kidney disease (ADPKD). Many mechanisms cause hypertension in ADPKD; however, primary aldosteronism (PA) as a possible manifestation of hypertension in ADPKD is extremely rare. The diagnosis of PA in ADPKD is extremely challenging because multiple renal cysts can mask the identification of adrenal adenomas, and ADPKD is associated with hypertension in majority of cases. Here, we report a unique case of a young lady with ADPKD with hypertension masquerading PA.

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“…also showed similar case report of association of aldosterone-producing adrenal adenoma in a patient with ADPKD in 1994. [ 10 ]…”

Section: Discussionmentioning

confidence: 99%

Autosomal dominant polycystic kidney disease and hypertension masquerading primary aldosteronism

Mital

Shishir

2018

Indian J Nephrol

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“…The coexistence of hypertension and hypokalemia in secondary aldosteronism is an uncommon presentation in ADPKD patients; only two cases have been reported 9 10) . There are other case reports of ADPKD complicated by the coincidence of primary aldosteronism 11 12 13 14 15) . The diagnosis included hyperaldosteronemia and hyporeninemia.…”

Section: Discussionmentioning

confidence: 99%

Hypokalemic Hypertension Leading to a Diagnosis of Autosomal Dominant Polycystic Kidney Disease

Vutthikraivit

Assanatham

Sriphrapradang

2016

Electrolyte Blood Press

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Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disease. Hypertension is common and occurs before decline in renal function. However, the coexistence of hypertension and hypokalemia is rare in ADPKD patients. We report on a 32-year-old woman with secondary aldosteronism. Magnetic resonance imaging of the renal arteries revealed multiple cysts of varying sizes in both the kidneys and the liver, compatible with ADPKD. Increased reninangiotensin-aldosterone system activity was secondary to cyst expansion. After initiation of angiotensin II receptor blocker, her blood pressure was controlled without additional requirement of potassium.

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“…Avoiding delays in diagnosis will permit aggressive BP treatment, early inhibition of the RAAS and decreased cardiovascular risks. 5,6 Including our three patients, a total of 11 patients with PA and ADPKD have been reported in the literature (Table 1); [13][14][15][16][17][18] these consisted of two men and nine women. The mean age at diagnosis of PA was 40 years old (range, 23 to 57 years).…”

Section: Discussionmentioning

confidence: 99%

“…[13][14][15][16][17][18] Considering the rare coexistence of ADPKD and PA in our TAIPAI study cohort, a more liberal criterion of initial PRA and ARR levels for further confirmation of PA may be appropriate (Figure 1) .…”

Section: Discussionmentioning

confidence: 99%

“…To the best of our knowledge, all of the reported ADPKD patients with PA had PRAs of less than 0.77 ng•mL -1 •h -1 , with an average of 0.31 ± 0.22 ng•mL -1 •h -1 , and their ARRs were greater than 50, with an average of 216 ± 158 (Table 1). [13][14][15][16][17][18] Considering the rare coexistence of ADPKD and PA in our TAIPAI study cohort, a more liberal criterion of initial PRA and ARR levels for further confirmation of PA may be appropriate (Figure 1) .…”

Section: Discussionmentioning

confidence: 99%

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Delayed diagnosis of primary aldosteronism in patients with autosomal dominant polycystic kidney diseases

Kuo

et al. 2012

J Renin Angiotensin Aldosterone Syst

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Hypertension is a frequent early manifestation of autosomal dominant polycystic kidney disease (ADPKD). Several mechanisms can cause hypertension in ADPKD patients, although, primary aldosteronism (PA) as a possible manifestation of hypertension in ADPKD is extremely rare. We retrospectively reviewed the Taiwan Primary Aldosteronism Investigation (TAIPAI) database, which listed a total of 346 patients diagnosed with PA. Of these 346 patients, only three cases of concurrent PA and ADPKD were identified. These patients presented with hypertensive crisis and hypokalemia, and subsequent testing revealed aldosterone-producing adenomas (APAs) that were removed by laparoscopic adrenalectomy. Postoperatively, aldosterone-renin ratios (ARRs) and potassium levels normalized, and blood pressure improved. The diagnosis of PA in ADPKD is extremely challenging because multiple renal cysts can obscure the identification of adrenal adenomas, and ADPKD is associated with hypertension in almost all cases.(1) Because of frequent delays in the diagnosis of PA in ADPKD patients, future prospective studies to screen PA in hypertensive ADPKD patients may be necessary to evaluate the exact prevalence of coexistence of PA and ADPKD.

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The Association of Aldosterone-Producing Adrenal Adenoma in a Patient With Autosomal Dominant Polycystic Kidney Disease

Cited by 6 publications

References 21 publications

Autosomal dominant polycystic kidney disease and hypertension masquerading primary aldosteronism

Autosomal dominant polycystic kidney disease and hypertension masquerading primary aldosteronism

Hypokalemic Hypertension Leading to a Diagnosis of Autosomal Dominant Polycystic Kidney Disease

Delayed diagnosis of primary aldosteronism in patients with autosomal dominant polycystic kidney diseases

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