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Saudi Journal of Kidney Diseases and Transplantation
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ARTICLE Table of Contents   
Year : 1999  |  Volume : 10  |  Issue : 3  |  Page : 349-351
Hypertension in Autosomal Dominant Polycystic Kidney Disease


Director of Nephrology, Riyadh Armed Forces Hospital, Riyadh, Saudi Arabia

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How to cite this article:
Al-Khader AA. Hypertension in Autosomal Dominant Polycystic Kidney Disease. Saudi J Kidney Dis Transpl 1999;10:349-51

How to cite this URL:
Al-Khader AA. Hypertension in Autosomal Dominant Polycystic Kidney Disease. Saudi J Kidney Dis Transpl [serial online] 1999 [cited 2020 Aug 4];10:349-51. Available from: http://www.sjkdt.org/text.asp?1999/10/3/349/37242
Hypertension (HT) is present in virtually all patients with autosomal dominant polycystic kidney disease (ADPKD) by the time they reach end-stage renal failure. However, even before the presence of significant renal impairment, a large proportion of patients with ADPKD has HT: 66% of men, 41% of women and 16-33% of children.1 Hyper­tension usually develops by age of 31 years.

There are a number of factors that are known to predispose to HT: male gender, renal volume, and number of cysts and PKD1 genotype. [1] In both male and female hypertensives, the renal volume was greater than in normotensive ADPKD patients (624 ± 47 cm3 VS 390 ± 43 cm3, PO.0005) in males, and (446 ± 32 cm3 VS 338 ± 24 cm3, P<0.002) in females. [2] Similar findings were also present in children. Moreover, in children with ADPKD, the rate of growth of the kidney size was greater in those with HT (31.1 ± 5.7 cm3/year) than those with normal blood pressure (8.9 ±1.5 cm3/year), P≤O.001. [2]


   Etiology Top


It is believed that compression of the intra­renal vessels by the cysts leads to local ischemia and activation of the renin aldos­terone system (RAS). It was found that supine and upright plasma renin activity (PRA) in hypertensive patients with ADP-KD (0.36 ± 0.06 and 1.03 ±0.14µg/L per hour, respecti­vely) was significantly higher than comparable group with essential HT (0.22 ± 0.06 and 0.61 ± 0.08 (µg/L per hour, respectively, P<0.05 and 0.03, respectively). Similarly, supine aldosterone levels were significantly higher in normotensive ADPKD group compared to normotensive controls. It should be noted that the ADPKD group had normal renal function and the groups were matched. [3] It was also found that, after short term treatment with angiotensin converting enzyme (ACE) inhibitors, the PRA level (1.97(µg/L per hour) was significantly higher in ADPKD patients compared to patients with essential hypertension (0.67µg/L per hour) (PO.0006). After six weeks of ACE inhibition, the renal plasma flow was significantly increased and renal vascular resistance and filtration fraction significantly reduced in ADPKD patients. The renal dynamics, however, remained unchanged in essential hypertensives.


   Effect on Renal Function Top


ADPKD patients who are hypertensive by age of 35 years have a mean renal survival age of 51 years compared to 65 years in those who remained nomotensive by age of 35 years (PO.0001). [4]

Women with ADPKD, who have less incidence of HT, tend to reach end stage renal disease (ESRD) five to eight years later than men. [5] In a study of ADPKD patients who started with normal renal function, normotensive ADPKD patients were shown to have slower progression of renal disease compared to hypertensive ADPKD. Indeed, it is rare to find a patient with ADPKD who reaches ESRD and is not hypertensive. [1]


   Cardiovascular Morbidity and Mortality Top


Mortality because of uremia was reduced from 28% to 2% in a study comparing ADPKD patients diagnosed before 1975 and after 1975.6 However, mortality due to cardiac causes increased from 21% to 36%. Even in those who died before reaching dialysis, 68% had cardiac and neurological causes. Of those who had autopsy, 89% had cardiac hypertrophy, 81% had coronary artery atheroma and 76% had aortic atheroma. [6]

Echocardiography showed that left ventricular hypertrophy occurred in 48% of hypertensive ADPKD subjects. [7] Left ventricular mass index (LVMI) was increased in children with ADPKD even with normal blood pressure measured casually. Furthermore, blood pressure was higher on ambulatory 24­hour blood pressure measurement, which also revealed obliteration of the nocturnal blood pressure dip.

In 86 affected children, left ventricular myocarduak ubdex was found to be 78 gm/m compared to 73 gm/m 2 in 68 unaffected children of the same age group (P<0.06), and LVMI correlated with systolic blood pressure in the affected but not in the unaffected children (P<0.0001). [9] LVMI (87.1 gm/m 2 ) was significantly higher in the hypertensive ADPKD children than in the normotensive affected children (76.6 gm/m 2 ; P<.005). [8]


   Treatment Top


The best treatment of hypertension associated with ADPKD is ACE inhibitors. However, 5% can show deterioration in renal function especially in association with diuretic use or hemorrhage in a cyst.

The evidence that LVMI increases before frank hypertension dictates that there may be good reason to start therapy early.

There is no convincing evidence that treatment of blood pressure retards progression to ESRD in ADPKD patients.9 However, in a rat model, ACE inhibitor was shown to reduce cyst volume and progression of renal disease. [10]

 
   References Top

1.Bell P, Hossack K, Gabow P. Hypertension in autosomal dominant polycystic kidney disease. Kidney hit 1998;34:683.  Back to cited text no. 1    
2.Gabow PA, Chapman AB, Johnson AM, et al. Renal structure and hypertension in autosomal dominant polycystic kidney disease. Kidney Int 1990:38:1 1 77-80.  Back to cited text no. 2    
3.Chapman AB, Johnson A, Gabow PA, Schrier RW. The renin angiotensin aldos­terone system and autosomal dominant polycystic kidney disease. New Engl J Med 1990;323:1091-6.  Back to cited text no. 3  [PUBMED]  
4.Johnson AM, Gabow PA. Identification of patients with autosomal dominant polycystic kidney at highest risk for end-stage renal disease. J Am Soc Nephrol 1997:8:1560-7.  Back to cited text no. 4    
5.Gretz N, Zeier M, Geberth S, Strauch M, Ritz E. Is gender a determinant for evolution of renal failure? A study in autosomal dominant polycystic kidney disease. Am J Kidney Dis 1989; 14:178-83.  Back to cited text no. 5    
6.Fick GM, Johnson AM, Hammond WS, Gabow PA. Causes of death in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1995;5:2048-56.  Back to cited text no. 6  [PUBMED]  
7.Ivy DD, Shaffer EM, Johnson AM, Kimberling WJ, Dobin A, Gabow PA. Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1995;5:2032-6.  Back to cited text no. 7  [PUBMED]  
8.Zeier M, Geberth S, Schmidt KG, Mandel-baum A, Ritz E. Elevated blood pressure profile and left ventricular mass in children and young adults with autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1993;3:1451-7.  Back to cited text no. 8    
9.Khahrs S, Breyer JA, Beck CJ, et al. Dietary protein restriction, blood pressure control and the progression of polycystic kidney disease. J Am Soc Nephrol 1995;5: 2037-44.  Back to cited text no. 9    
10.Ogborn MR, Sareen S, Pinette G. Cilazpril delay progression of hypertension and uremia in rat polycystic kidneydisease. Am J Kidney Dis 1995;26:942-6.  Back to cited text no. 10  [PUBMED]  

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Correspondence Address:
Abdullah A Al-Khader
Director of Nephrology, Riyadh Armed Forces Hospital, P.O. Box 7897, Riyadh 11159
Saudi Arabia
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PMID: 18212444

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