KREVNÍ TLAK, FUNKČNÍ A MORFOLOGICKÉVYŠETŘENÍ LEDVIN U DĚTÍ S AUTOZOMÁLNĚDOMINANTNÍM POLYCYSTICKÝMONEMOCNĚNÍM LEDVIN

Blood Pressure, Renal Function and Morphology in Children withAutosomal Dominant Polycystic Kidney Disease

Background.
Autosomal dominant polycystic kidney disease is one of the most common inherited disorders witha prevalence of 1 : 1000 and is responsible for cca 10 % of end-stage renal disease in adult patients. Renal insufficiencyis a rare symptom of ADPKD in children, however, there are some symptoms, which can occur already in childhood.The aim of this study was to detect early signs of renal damage and to reveal the blood pressure profile in childrenwith ADPKD using ABPM (ambulatory blood pressure monitoring).Methods and Results. 32 children (aged 3.4-19.4 years, mean age 12.3) - carriers of PKD - gene with normalGFR, diagnosed on the basis of a positive family history and characteristic ultrasound features, and in 21 cases alsoindirect DNA analysis revealed positive results.11/32 children (34 %) presented arterial hypertension detected by ABPM (values higher than 95th centile of normalindividuals). The mean of ABPM values of all patients was significantly higher than normal children (p < 0.01),76 % patients had values above the 50th centile. Signs of renal damage (proteinuria, microalbuminuria, decreasedrenal concentrating capacity, pathological excretion of tubular markers) were found in 22-64 % of investigatedchildren. Significantly higher renal volume and renal length were found in more than 1/3 of the children, renalvolume and length higher than the mean of healthy children in about 90 % of patients and a significant correlationbetween ABPM parameters and renal length and volume (p < 0.05).Conclusions. The results of this study show, that the signs of renal damage and arterial hypertension occur relativelyoften in children with ADPKD despite still normal GFR. This justifies the early diagnosis of ADPKD especially asit reveals the most important treatable complication of ADPKD - arterial hypertension. These findings emphasisethe importance of early diagnosis in children from families with ADPKD. Probably, early treatment of hypertensioncould even postpone the otherwise common end-stage renal damage in adult patients with ADPKD. Careful followup of all children with ADPKD is strongly recommended, above all the blood pressure should be controlled.

Key words:
autosomal dominant polycystic kidney disease, ABPM, hypertension, early renal damage.