The follow-up period of children and teenagers with polycystic kidney disease

Abstract

THE AIM: To study catamnesis of children and adolescents with polycystic kidney disease (PKD). PATIENTS AND METHODS: we performed a genealogical analysis of 48 families, clinical laboratory, ultrasound (US) 98 members of 48 families in which at least one child (the proband) was observed with a diagnosis of «PKD». To determine age by the detection of cysts based on US , the features of the initial clinical manifestations and course, complications and outcome depending on the type of inheritance conducted follow-up study of 64 children and adolescents (from 48 families) with PKD aged 1 month to 18 years (mean age 13.5±4.5 years). The remoteness from the time of diagnosis of PKD by the time catamnesis in 64 children and adolescents ranged from 1 month to 17 years. RESULTS: Of the 64 children and adolescents autosomal dominant polycystic kidney disease (ADPKD) is diagnosed in 71.9%, autosomal recessive polycystic kidney disease (ARPKD) in 15.6%, unspecified polycystic kidney disease (UPKD) in 12.5% of cases. With age in patients with ADPKD there is a continuous increase of the diameter of cysts in the kidneys. The children with ARPKD revealed a progressive increase in the volume of the kidneys and the number of small cysts. The rate of detection of cysts in other organs (liver, pancreas, ovaries) in children and adolescents with ARPKD, significantly higher than that in patients with ADPKD and UPKD (70%, 6.5% and 0%, respectively). The syndrome of arterial hypertension is development in patients with ARPKD in 100% (40% at birth to 60% in infants), that it is in contrast to patients with ADPKD 32.6% of cases (moving to 100% in adults members of families with ADPKD aged 25 to 50 years). Pyelonephritis is diagnosed in children with ARPKD in 80% of cases, significantly more frequently than in children and adolescents with ADPKD (32.6%) and UPKD (37.5%). ARPKD is characterized by adverse prognosis, the outcome in chronic renal failure (90%) and deaths (30%) in the first year of life. The progression of chronic renal failure in childhood when ADPKD was significantly less (4.3%). CONCLUSION: the identified features of the initial manifestations, course and outcome of PKD in children with different types of inheritance of the disease.