Early renal abnormalities in children with postnatally diagnosed autosomal dominant polycystic kidney disease.

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.