Patient's mother has ADPKD and suffered end stage renal disease at age 60. Patient's mother (and sister) first had onset of high blood pressure at age 30 and used ARB medications to control. The child shows onset of high blood pressure at age 17 and is set to begin blood pressure medications. Ultrasound shows cysts in both kidneys but normal size kidneys and normal function. Is the earlier age of onset of high blood pressure for the child any indication of potential for faster progressing disease and potentially earlier end stage renal?
An RCT that randomised children with ADPKD to ACEi therapy demonstrated that those with hypertension were at greater risk for increased renal volume and a reduction in renal function relative to those without hypertension. ACEi's did not have a significant effect on renal growth.
Since renal failure progression in ADPKD is associated with a progressive increase in renal volume, from this study it is therefore possible that the earlier onset of hypertension in kids with ADPKD may be associated with the earlier onset of ESRD in this group.
Study URL: http://cjasn.asnjournals.org/content/4/4/820.long
Interesting so ace inhibitor therapy did not impact renal growth. There are some who believe that aggressive blood pressure control in ADPKD can perhaps slow renal growth, but according to this study, maybe not.
ADPKD is a tubular disease , the loss of PKD 1 or PKD 2 gene expression in the fenotype( at the level of the tubules, and not just renal tubules , it can also happen in the liver , the pancreas , or even in blood vessels - some of the patients develop brain vascular aneurisms )will produce a deficit of polycystins which in the end will produce a hyperproliferation of tubular epitelial cells. From those altered cells the cysts grow and through processes of apoptosis and fibrosis , they destroy the surrounding parenchyma. in ADPKD , hypertension is an effect of this mechanism , and is directly proportional to the degree of damage. So , in my opinion, if hypertension starts and an early age and we have a confirmed diagnosis( Ravine criteria ), it will be associated with a faster progression of the disease.
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