In the past, autosomal dominant polycystic kidney disease (ADPKD) had been termed adult polycystic disease. Now, however, it is clear that many of the features of ADPKD occur during childhood. In fact, ADPKD has been diagnosed in utero. 1 Very-early-onset ADPKD has been the designation for infants whose diagnosis is made within the first 18 mo of life. 2 As compared with children who receive a diagnosis of ADPKD later, the children with very-early-onset ADPKD have larger kidneys and more renal cysts when adjusted for age. 3
CHILDREN WITH ADPKDThe most consistent characteristic of children with ADPKD is the relationship between renal volume and BP. Children with ADPKD and hypertension, defined as Ͼ95th percentile, have significantly larger kidneys than children who have ADPKD and have BP Ͻ95th percentile. 4 Children with BP between the 75th and 95th percentiles have been designated as having borderline hypertension, whereas BP Ͻ75th percentile is designated as normotension. 4 Children who have ADPKD and either hypertension or borderline hypertension exhibit a left ventricular mass index (LVMI) that is significantly greater than in children with normotension. Children who have ADPKD with hypertension have the largest kidneys by ultrasonography, and there is a highly significant correlation in renal volume with systolic as well as diastolic BP. 4 In a prospective 5-yr follow-up study, children with ADPKD and hypertension demonstrated a progressive increase in renal volume and LVMI. 5 During this 5-yr follow-up, 52% of the children with borderline hypertension developed frank hypertension. Of interest, in contrast to children who have ADPKD with hypertension, who needed more than one antihypertensive agent, the BP in children with borderline hypertension could be well controlled to Ͻ50th percentile by monotherapy with an angiotensin-converting enzyme inhibitor
ABSTRACTThe relationship between renal volume and hypertension in autosomal dominant polycystic kidney disease (ADPKD) occurs in childhood. Hypertension is associated not only with increased kidney volume but also with significantly increased left ventricular mass index. Moreover, this increase in left ventricular mass index occurs in children who have ADPKD with borderline hypertension (75th to 95th percentile) and is prevented with angiotensin-converting enzyme inhibitor (ACEI) monotherapy. Progression from borderline to overt hypertension (Ն95th percentile) occurs during a 5-yr follow-up in approximately 50% of children with ADPKD and borderline hypertension. Renal cyst enlargement in ADPKD in adults is associated with stimulation of both the circulating and intrarenal renin-angiotensin-aldosterone system. In addition to hypertension, the resultant angiotensin in ADPKD is a pivotal factor in cyst proliferation and expansion, increased sympathetic and endothelin activity, oxidant injury, and fibrosis. There is a close correlation between the level of hypertension, left ventricular hypertrophy, deterioration of GFR, and the progressive enlargement of the cys...