Hypertension in autosomal dominant polycystic kidney disease

Bell, Patricia; Hossack, Kenneth F.; Gabow, Patricia A.; Dürr, Jacques A.; Johnson, Ann; Schrier, Robert W.

doi:10.1038/ki.1988.233

Cited by 110 publications

(56 citation statements)

References 24 publications

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“…24 In a better controlled study, Bell and co-workers studied NT and HT ADPKD patients on a low (20 mmol/day) and high (200 mmol/day) sodium intake, and generally found no differences in responses of PRA or BP to the change in sodium intake or the administration of captopril. 25 Very similar findings have recently been reported by Ramunni et al…”

Section: Renin-angiotensin System (Ras)supporting

confidence: 80%

“…Many of these studies can be criticised because the ADPKD patients were not matched with the control subjects for BP [22][23][24][25][26] and renal function. 23,24 It is likely that the raised BP itself and the expansion of extra-cellular fluid volume that occurs, particularly in those with renal impairment, will tend to suppress PRA and it is, therefore, not appropriate to use healthy …”

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confidence: 99%

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Review: Autosomal Dominant Polycystic Kidney Disease: Role of the Renin-Angiotensin System in Raised Blood Pressure in Progression of Renal and Cardiovascular Disease

Lawson

Doulton

MacGregor

2006

J Renin Angiotensin Aldosterone Syst

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Raised blood pressure (BP) is extremely common in individuals with autosomal dominant polycystic kidney disease (ADPKD) and is almost invariably raised once they develop renal failure. The underlying mechanisms for the rise in BP in individuals with ADPKD are unclear. The progressive number and enlargement of renal cysts, causing structural damage to the kidneys and, thereby, affecting tubular function as well as causing distortion of the glomeruli and renal ischaemia, is likely to be of primary importance.There is some evidence from animal models that there may be over-activity of the intra-renal renin-angiotensin system (RAS) that could account for the rise in BP. Studies in man have shown conflicting results, but a recent more carefully controlled study using both measurements of activity and pharmacological blockade of the RAS clearly demonstrated no evidence of over-activity of the circulating RAS in ADPKD compared to matched individuals with essential hypertension.A more likely explanation for the rise in BP that occurs in ADPKD is retention of sodium and water due to tubular damage. Disappointingly, in spite of good evidence that RAS blocking drugs slow the progression of other renal, particularly glomerular, diseases, there is little evidence to suggest this is true for patients with ADPKD. Nevertheless, there is no doubt that lowering BP in ADPKD is just as important, if not more important, as in essential hypertension to prevent cardiovascular disease and strokes, with a recommended BP target of < 120/80 mmHg.

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Section: Renin-angiotensin System (Ras)supporting

confidence: 80%

mentioning

confidence: 99%

Review: Autosomal Dominant Polycystic Kidney Disease: Role of the Renin-Angiotensin System in Raised Blood Pressure in Progression of Renal and Cardiovascular Disease

Lawson

Doulton

MacGregor

2006

J Renin Angiotensin Aldosterone Syst

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“…Extrarenal manifestations such as cyst formation in the liver, pancreas, and spleen may also occur with autosomal dominant polycystic kidney disease as well as cranial aneurysms and secondary hypertension (1,2). Positional cloning identified pkd2 as one of the genes mutated in ϳ15% of affected families (3)(4)(5).…”

mentioning

confidence: 99%

PKD2 Interacts and Co-localizes with mDia1 to Mitotic Spindles of Dividing Cells

Rundle

Gorbsky

Tsiokas

2004

Journal of Biological Chemistry

110

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Mutations in pkd2 result in the type 2 form of autosomal dominant polycystic kidney disease, which accounts for ϳ15% of all cases of the disease. PKD2, the protein product of pkd2, belongs to the transient receptor potential superfamily of cation channels, and it can function as a mechanosensitive channel in the primary cilium of kidney cells, an intracellular Ca 2؉ release channel in the endoplasmic reticulum, and/or a nonselective cation channel in the plasma membrane. We have identified mDia1/Drf1 (mammalian Diaphanous or Diaphanous-related formin 1 protein) as a PKD2-interacting protein by yeast two-hybrid screen. mDia1 is a member of the RhoA GTPase-binding formin homology protein family that participates in cytoskeletal organization, cytokinesis, and signal transduction. We show that mDia1 and PKD2 interact in native and in transfected cells, and binding is mediated by the cytoplasmic C terminus of PKD2 binding to the mDia1 N terminus. The interaction is more prevalent in dividing cells in which endogenous PKD2 and mDia1 co-localize to the mitotic spindles. RNA interference experiments reveal that endogenous mDia1 knockdown in HeLa cells results in the loss of PKD2 from mitotic spindles and alters intracellular Ca 2؉ release. Our results suggest that mDia1 facilitates the movement of PKD2 to a centralized position during cell division and has a positive effect on intracellular Ca 2؉ release during mitosis. This may be important to ensure equal segregation of PKD2 to the daughter cell to maintain a necessary level of channel activity. Alternatively, PKD2 channel activity may be important in the cell division process or in cell fate decisions after division.

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“…The cysts are thought to induce focal areas of renal ischemia and enhanced renin release, increasing renin-aldosterone system activity, with the result that extracellular volume expansion is often present in the early stages of ADPKD (19). It is not unusual for young adults with ADPKD to exhibit hypertension before they experience evident renal dysfunction (20,21). The mechanism of hypertension in ADPKD is thought to be complex.…”

Section: Discussionmentioning

confidence: 99%

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

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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...

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Hypertension in autosomal dominant polycystic kidney disease

Cited by 110 publications

References 24 publications

Review: Autosomal Dominant Polycystic Kidney Disease: Role of the Renin-Angiotensin System in Raised Blood Pressure in Progression of Renal and Cardiovascular Disease

Review: Autosomal Dominant Polycystic Kidney Disease: Role of the Renin-Angiotensin System in Raised Blood Pressure in Progression of Renal and Cardiovascular Disease

PKD2 Interacts and Co-localizes with mDia1 to Mitotic Spindles of Dividing Cells

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

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