Macroscopic hematuria in children with autosomal dominant polycystic kidney disease - report from four European tertiary centers.

In adults with autosomal dominant polycystic kidney disease (ADPKD), episodes of macroscopic hematuria are relatively common and are predictors of hypertension. We hypothesized that a history of macroscopic hematuria will be associated with hypertension also in children with ADPKD. We retrospectively analyzed 289 children (median age 10.2 years) with ADPKD who were followed in four European tertiary nephrology centers. The presence of ≥ 1 episode of macroscopic hematuria (MaHu) defines a group of MaHu+ patients; the absence defines a group of MaHu- patients. Hypertension was defined as the use of antihypertensive drugs upon the last investigation. Macroscopic hematuria occurred in 7.2% of children (n = 21). It was the second most frequent presenting symptom of ADPKD (11 out of 51 children). The etiology of macroscopic hematuria included ruptured cyst, urinary tract infection or urolithiasis. The prevalence of hypertension at the last follow-up in MaHu+ children was significantly higher than in MaHu- children (14.3% vs. 3.1%, p = 0.017). The proportion of patients with elevated blood pressure ≥ 75th percentile was significantly higher in patients with hematuria as compared with patients with no hematuria (95% vs. 64%, p = 0.002). Children from the MaHu+ group had a significantly higher number of cysts when compared to the MaHu- group (20 vs. 10 cysts, p = 0.038). There were no significant differences between MaHu+ vs. MaHu- groups in other variables.

conclusionIn children with ADPKD, a history of macroscopic hematuria is associated with an increased risk of developing hypertension and/or elevated blood pressure. WHAT IS KNOWN: • Adults with autosomal dominant polycystic kidney disease have often episodes of macroscopic hematuria that predict development of hypertension. WHAT IS NEW: • In children with autosomal dominant polycystic kidney disease, macroscopic hematuria is rare but is also associated with an increased risk of developing hypertension.