Hypertension in Children with Cystic Kidney Diseases

Abstract

Cystic kidney diseases are one of the most common urogenital malformations and are responsible for a substantial morbidity and mortality. Autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant polycystic kidney disease (ADPKD), multicystic dysplastic kidney (MCDK) and juvenile nephronophthisis (JNP) are the most common and most serious cystic kidney diseases.Hypertension is the main risk factor for progression of various nephropathies, including cystic kidney diseases. The prevalence of hypertension varies depending on the method of BP measurement and the type of cystic disease. Ambulatory BP monitoring (ABPM) is a more potent tool for detection of hypertension than the clinic BP. Hypertension is most common in children with ARPKD, the prevalence ranges between 60 -100% and hypertension accounts for substantial cardiovascular morbidity and mortality in these patients. In children with ADPKD the prevalence of hypertension is 15 -38% despite normal renal function and is the most important treatable factor for progression of the disease. Hypertension is a relative uncommon finding in children with MCDK (0 -20%) and is more often caused by contralateral kidney damage than by affected multicystic kidney. Children with JNP are usually normotensive until the late stages of renal insufficiency.The drugs of first choice in children with cystic kidney diseases are ACE-inhibitors. Most children with ARPKD have severe hypertension and require combination therapy with 2 -4 drugs (beta-blockers, diuretics, calcium channel blockers). Treatment of hypertension is important not only to delay progression of the kidney disease but also to decrease the cardiovascular morbidity and mortality.

Nephronophthisis-Pathobiology and Molecular Pathogenesis of a Rare Kidney Genetic Disease

“…Blood pressure has been a focus of more recent studies including meta-analysis of hypertension in children with PKD [42], and was thought to be secondary to upregulation in renal renin angiotensin aldosterone system (RAAS). In NPHP, blood pressure rises, usually secondary to poor kidney function, though is uncommon [43]. Situs inversus and ventricular septal defects are described in NPHP, particularly in NPHP2 [44].…”