"Autosomal dominant polycystic kidney disease; PKD1 gene; PKD2 gene; Renal function; Ultrasound; Blood pressure; Hypertension; Children; renal-disease; clinical-features; blood-pressure; in-utero; pkd2; prognosis; gene; adolescents; progression; childhood"@en . "P(NR9427), Z(MSM0021620819), Z(MZ0FNM2005)" . . "Pediatric Nephrology" . "000264535700007" . . . . "7"^^ . "Bl\u00E1hov\u00E1, Kv\u011Bta" . . "Genotype-phenotype correlation in children with autosomal dominant polycystic kidney disease" . . . . . . "H\u0159\u00EDbal, Zden\u011Bk" . . . "0931-041X" . . . . "US - Spojen\u00E9 st\u00E1ty americk\u00E9" . . "Genotype-phenotype correlation in children with autosomal dominant polycystic kidney disease"@en . "Fencl, Filip" . . . "4"^^ . . . "Adults with autosomal dominant polycystic kidney disease (ADPKD) and PKD1 mutations have a more severe disease than do patients with PKD2 mutations. The aim of this study was to compare phenotypes between children with mutations in the PKD1/PKD2 genes. Fifty PKD1 children and ten PKD2 children were investigated. Their mean age was similar (8.6 +/- 5.4 years and 8.9 +/- 5.6 years). Renal ultrasound was performed, and office blood pressure (BP), ambulatory BP, creatinine clearance and proteinuria were measured. The PKD1 children had, in comparison with those with PKD2, significantly greater total of renal cysts (13.3 +/- 12.5 vs 3.0 +/- 2.1, P = 0.004), larger kidneys [right/left kidney length 0.89 +/- 1.22 standard deviation score (SDS) vs 0.17 +/- 1.03 SDS, P = 0.045, and 1.19 +/- 1.42 SDS vs 0.12 +/- 1.09 SDS, P = 0.014, successively] and higher ambulatory day-time and night-time systolic BP (day-time/night-time BP index 0.93 +/- 0.10 vs 0.86 +/- 0.05, P = 0.021 and 0.94 +/- 0.07 vs 0.89, P=0.037)" . "[1098634375B6]" . "Genotype-phenotype correlation in children with autosomal dominant polycystic kidney disease"@en . "Janda, Jan" . . "11130" . "RIV/00216208:11130/09:5324" . . "Seeman, Tom\u00E1\u0161" . "5" . "Genotype-phenotype correlation in children with autosomal dominant polycystic kidney disease" . . . "RIV/00216208:11130/09:5324!RIV10-MZ0-11130___" . "Adults with autosomal dominant polycystic kidney disease (ADPKD) and PKD1 mutations have a more severe disease than do patients with PKD2 mutations. The aim of this study was to compare phenotypes between children with mutations in the PKD1/PKD2 genes. Fifty PKD1 children and ten PKD2 children were investigated. Their mean age was similar (8.6 +/- 5.4 years and 8.9 +/- 5.6 years). Renal ultrasound was performed, and office blood pressure (BP), ambulatory BP, creatinine clearance and proteinuria were measured. The PKD1 children had, in comparison with those with PKD2, significantly greater total of renal cysts (13.3 +/- 12.5 vs 3.0 +/- 2.1, P = 0.004), larger kidneys [right/left kidney length 0.89 +/- 1.22 standard deviation score (SDS) vs 0.17 +/- 1.03 SDS, P = 0.045, and 1.19 +/- 1.42 SDS vs 0.12 +/- 1.09 SDS, P = 0.014, successively] and higher ambulatory day-time and night-time systolic BP (day-time/night-time BP index 0.93 +/- 0.10 vs 0.86 +/- 0.05, P = 0.021 and 0.94 +/- 0.07 vs 0.89, P=0.037)"@en . "Stekrova, J." . . . "7"^^ . . . . . . . . . "316217" . . "24" . "Puchmajerov\u00E1, Alena" . .