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Journal of the American Society of Nephrology, Vol 5, 2032-2036, Copyright © 1995 by American Society of Nephrology

REGULAR ARTICLES

Cardiovascular abnormalities in children with autosomal dominant polycystic kidney disease

DD Ivy, EM Shaffer, AM Johnson, WJ Kimberling, A Dobin and PA Gabow
Children's Hospital, Denver, CO, 80218, USA.

It is known that adults with autosomal dominant polycystic kidney disease (ADPKD) have an increased incidence of cardiovascular abnormalities, including mitral valve prolapse. The cardiac manifestations of ADPKD in the pediatric population have not been well established. To determine the cardiac manifestations of children with ADPKD, echocardiography was performed in 154 children of 66 families in which one parent has ADPKD. Eighty-six affected children and 68 unaffected children were evaluated in a prospective, single-blinded manner by echocardiography. Affected children were defined as those with any cysts on a concurrent renal ultrasound or those predicted to be gene carriers by gene linkage analysis. A 12% incidence of mitral valve prolapse was found in the affected children compared with only 3% of the unaffected children (P < 0.05). ADPKD children, but not their unaffected siblings, demonstrate a significant correlation between left ventricular mass index and systolic blood pressure. Moreover, hypertensive ADPKD children have significantly larger left ventricular mass index than do normotensive ADPKD children. A 3.5% incidence of congenital heart disease was found in the affected group, whereas 2.9% of the unaffected children had congenital heart disease. It was concluded that systemic manifestations of ADPKD, particularly cardiovascular abnormalities, are present even in childhood and these warrant the clinician's attention.


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C. R Lawson, T. W Doulton, and G. A MacGregor
Review: Autosomal Dominant Polycystic Kidney Disease: Role of the Renin-Angiotensin System in Raised Blood Pressure in Progression of Renal and Cardiovascular Disease
Journal of Renin-Angiotensin-Aldosterone System, September 1, 2006; 7(3): 139 - 145.
[Abstract] [PDF]

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T. ECDER and R. W. SCHRIER
Hypertension in Autosomal-Dominant Polycystic Kidney Disease: Early Occurrence and Unique Aspects
J. Am. Soc. Nephrol., January 1, 2001; 12(1): 194 - 200.
[Full Text]


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