Background: Autosomal dominant polycystic kidney disease (ADPKD) in children is often regarded as a benign condition. However, previous studies pointed out renal-related anomalies which may benefit from early appropriate treatments. This study was conducted to evaluate the prevalence and severity of early renal dysfunction in ADPKD children.
Methods: An extensive renal evaluation was performed in 52 consecutive ADPKD patients diagnosed either from prenatal screening or post-natal ultrasound (US) examination (54 % males, mean age 10 ± 4 years [1-17]).
Results: Three patients had both systolic (SBP) and diastolic (DBP) blood pressure above the 95th percentile, one patient had a "high normal" DBP, and one child was treated with an angiotensin-converting enzyme inhibitor (ACEI). The mean ± SD glomerular filtration rate (GFR ml/min per 1.73 m(2), inulin clearance) was 115 ± 26 [47-168] but six children (12 %) had a GFR < 90 and 11 (21 %) experienced hyperfiltration (GFR > 135). Microalbuminuria (2 < Ualb/Ucr ≤ 20 mg/mmol) was found in 25 patients and five had macroalbuminuria (>20 mg/mmol).
Conclusions: Early renal manifestations are frequent in ADPKD children, including hypertension in 6 %, albuminuria in 58 %, and decreased GFR in 12 %. In conclusion, renal function in children with ADPKD should be regularly assessed in order to manage early renal dysfunction and even consider further therapeutic intervention.