A Young Patient with a Family History of Hypertension

Peixoto, Aldo J.

doi:10.2215/cjn.02240314

Cited by 3 publications

(5 citation statements)

References 58 publications

(54 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In Lewis PKD rat, MR antagonism with spironolactone has proven an effective means to control hypertension, but had no effect on cyst growth or kidney morphology [ 79 ]. There are clinical reports suggesting that ADPKD and primary aldosteronism are connected and there might be a role for aldosterone excess and the resultant hypokalemia in promoting cyst growth [ 80 ]. However, the question of whether antagonism of MR should be used effectively in combination with RAS antagonism to treat PKD, remains open [ 81 ].…”

Section: Discussionmentioning

confidence: 99%

Salt-deficient diet exacerbates cystogenesis in ARPKD via epithelial sodium channel (ENaC)

et al. 2019

View full text Add to dashboard Cite

Background: Autosomal Recessive Polycystic Kidney Disease (ARPKD) is marked by cyst formation in the renal tubules, primarily in the collecting duct (CD) system, ultimately leading to end-stage renal disease. Patients with PKD are generally advised to restrict their dietary sodium intake. This study was aimed at testing the outcomes of dietary salt manipulation in ARPKD. Methods: PCK/CrljCrlPkhd1pck/CRL (PCK) rats, a model of ARPKD, were fed a normal (0.4% NaCl; NS), high salt (4% NaCl; HS), and sodium-deficient (0.01% NaCl; SD) diets for 8 weeks. Immunohistochemistry, GFR measurements, balance studies, and molecular biology approaches were applied to evaluate the outcomes of the protocol. Renin-angiotensin-aldosterone system (RAAS) levels were assessed using LC-MS/MS, and renal miRNA profiles were studied. Findings: Both HS and SD diets resulted in an increase in cystogenesis. However, SD diet caused extensive growth of cysts in the renal cortical area, and hypertrophy of the tissue; RAAS components were enhanced in the SD group. We observed a reduction in epithelial Na + channel (ENaC) expression in the SD group, accompanied with mRNA level increase. miRNA assay revealed that renal miR-9a-5p level was augmented in the SD group; we showed that this miRNA decreases ENaC channel number in CD cells. Interpretation: Our data demonstrate a mechanism of ARPKD progression during salt restriction that involves activity of ENaC. We further show that miR-9a-5p potentially implicated in this mechanism and that miR-9a-5p downregulates ENaC in cultured CD cells. Our findings open new therapeutic possibilities and highlight the importance of understanding salt reabsorption in ARPKD.

show abstract

Section: Discussionmentioning

confidence: 99%

Salt-deficient diet exacerbates cystogenesis in ARPKD via epithelial sodium channel (ENaC)

et al. 2019

View full text Add to dashboard Cite

show abstract

“…The mechanism of hypertension in ADPKD is thought to be complex. A number of potential factors have been proposed, including the renin-aldosterone system, endothelial dysfunction, increased sympathetic activity, increased endothelin-l levels and arterial stiffness (22).…”

Section: Discussionmentioning

confidence: 99%

“…One of the reasons is that hyperaldosteronism may not only to increase cardiovascular risk and secondary hypertension but may also to contribute to the growth of cysts, with hypokalemia implicated as a direct growth factor for cysts (22). Therefore, the early diagnosis and correction of underlying PA is critical in patients with ADPKD.…”

Section: Discussionmentioning

confidence: 99%

“…So far, there have been 12 reported cases of combined ADPKD and PA (6,7,23-27). Adrenalectomies were performed in 8 patients (6,7,23-26) and anti-aldosterone drugs were administered to the other 4 cases (22,23,27). Worsening of kidney function following adrenalectomy for PA has been reported (23); the cause may be the correction of glomerular hyperfiltration once aldosterone levels have been stabilized (3)(4)(5).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

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

Hikita

Kanno

Watanabe

et al. 2017

Experimental and Therapeutic Medicine

View full text Add to dashboard Cite

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...

show abstract

“…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

View full text Add to dashboard Cite

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.

show abstract

A Young Patient with a Family History of Hypertension

Cited by 3 publications

References 58 publications

Salt-deficient diet exacerbates cystogenesis in ARPKD via epithelial sodium channel (ENaC)

Salt-deficient diet exacerbates cystogenesis in ARPKD via epithelial sodium channel (ENaC)

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

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

Contact Info

Product

Resources

About