Abstract
AIM OF STUDY. To estimate the characteristics of renal and extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD) in children. PATIENTS AND METHODS. 67 children from 60 families with ADPKD were involved in research. Data of follow-up, clinical, laboratory and instrumental examinations were used. Stratification of chronic kidney disease (CKD) according to NKF-K/DOQI classification (2002) and national recommendations (2012) was carried out. RESULTS. The agespecific features of renal cysts diagnostics by ultrasound were detected: 0-15 years in 91%, including 0-18 month (very early onset) in 19,4%; 15-18 years in 9%. Bilateral location of renal cysts dominate: at first detection (mean age 8,24±0,64 years) in 59,7%, at follow-up moment (mean age 13,2±0,54 years) in 95,5%. Regression analysis revealed that in ADPKD children annual increase of maximal diameter of renal cysts by ultrasound (US) scan was 0,21±0,03 cm, annual increase of average renal length by US scan was 0,42±0,05 cm. Arterial hypertension (AH) was diagnosed at first detection of cysts in 4,5%, at follow-up in 21%. Average renal length and maximal diameter of renal cysts in ADPKD children with AH is significantly larger than in ADPKD children without AH. There are no significant differences between average renal length, maximal diameter of renal cysts and AH frequency in children with very early and later detection of cysts. Extrarenal manifestations of ADPKD in children at followup (5,1±0,6 years) are significantly more often (31,3%), than at first renal cysts detection (13,5%). It was demonstrated that computer tomography and magnetic resonance imaging are significantly more informative methods for detection of extrarenal cysts than US scan. In ADPKD children I stage of CKD dominates in 94%. CONCLUSION. Characteristics of renal and extrarenal manifestations, course and outcome of ADPKD in children were revealed.
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