Affiliation:
1. Saint-Petersburg State Pediatric Medical University
Abstract
THE AIM: to characterize the features of the course of autosomal dominant (ADPKD) and autosomal recessive (ARPKD) polycystic kidney disease detected in the prenatal, neonatal and thoracic periods.PATIENTS AND METHODS: ADP was diagnosed in 28 and ARPP in 12 of 40 children and adolescents. The dynamics of the diameter of renal cysts (mm), total kidney volume (TKV, cm3) by ultrasound were evaluated; Constructed trend lines for average TKV and diameter of renal cysts. The glomerular filtration rate is determined by the Schwartz formula. Liver fibrosis was detected by ultrasound / MRI / CT / biopsy.RESULTS: ADPKD was detected prenatally and during the first year of life in 19.1 %, ARPKD in 70.6 %. Stable arterial hypertension was diagnosed with an ADPKD with “very early detection” in 7 % (among adolescents), with ARPKD in 100 % (under 3 years of age). The diameter of the renal cysts increases with ADPKD. Renal cysts are multiple, bilateral since birth with ARPKD, the diameter of the cysts does not increase. TKV increased at birth in 3.6 % of children with ADPKD, in 100 % with ARPKD. The trend line of average TKV with ADPKD is exponential, with ARPKD – linear. Extrarenal location of cysts was diagnosed with ADPKD in 3.6 % (in the testes), with ARPKD in 67 % (in the liver). Liver fibrosis with portal hypertension syndrome was detected in children with ARPKD in 33.3 %; performed ligation of the veins of the esophagus. Acute kidney damage was found in newborns with ADPKD in 3.6 %, with ARPKD in 33.3 %. Fatal outcome was ascertained in 3 (25 %) children with ARPKD. In the follow-up, the outcome in HBPS3 is in 2 children with ADPKD and 3 children with ARPP, in HBPS4 in 1 child with ARPKD.CONCLUSION: features of the course of ADPKD and ARPKD revealed in the prenatal, neonatal and thoracic periods are shown.
Publisher
Non-profit organization Nephrology
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