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. 2008 Apr;4(2):393-407.
doi: 10.2147/tcrm.s1617.

Developments in the management of autosomal dominant polycystic kidney disease

Amirali Masoumi  1 , Berenice Reed-Gitomer, Catherine Kelleher, Mir Reza Bekheirnia, Robert W Schrier
Affiliations

Developments in the management of autosomal dominant polycystic kidney disease

Amirali Masoumi et al. Ther Clin Risk Manag. 2008 Apr.

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent life- threatening, hereditary disease. ADPKD is more common than sickle cell anemia, cystic fibrosis, muscular dystrophy, hemophilia, Down's syndrome, and Huntington's disease combined. ADPKD is a multisystemic disorder characterized by the progressive development of renal cysts and marked renal enlargement. Structural and functional renal deterioration occurs in ADPKD patients and is the fourth leading cause of end-stage renal disease (ESRD) in adults. Aside from the renal manifestations, extrarenal structural abnormalities, such as liver cysts, cardiovascular abnormalities, and intracranial aneurysms may lead to morbidity and mortality. Recent studies have identified prognostic factors for progressive renal impairment including gender, race, age, proteinuria, hematuria, hypertension and increased left ventricular mass index (LVMI). Early diagnosis and better understanding of the pathophysiology of the disease provides the opportunity to aggressivly treat hypertension with renin-angiotensin-aldosterone system inhibitors and thereby potentially reduce LVMI, prevent cardiovascular morbidity and mortality and slow progression of the renal disease.

Keywords: cerebral aneurysms; hypertension; left ventricular hypertrophy; liver cysts; renal pain.

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Figures

Figure 1
Figure 1
Correlation between GFR and renal volume in ADPKD patients.
Figure 2
Figure 2
Prevalence of hypertension in autosomal dominant polycystic kidney disease (■) in (a) males and (b) females 1995–2000 vs NHANES IV (□).
Figure 3
Figure 3
Survival Curves for end-stage renal failure (ESRD) in (a) male and (b) female ADPKD patients from 1985–1992 vs 1992–2001 (n = 97).
Figure 4
Figure 4
Correlation between blood pressure and left ventricular hypertrophy in ADPKD patients.
Figure 5
Figure 5
(a) Effect of rigorous (■) versus standard (◆) lood pressure control to decrease left ventricular mass index in autosomal dominant polycystic kidney disease (ADPKD) patients. (b) Effect of blood pressure control with amlodipine (◆) versus enalapril (■) to decrease left ventricular mass index in ADPKD patients.
Figure 6
Figure 6
Renal volumes in age-adjusted VEO children and non-VEO children.
See this image and copyright information in PMC

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