title: [Polycystic kidney disease--autopsy review from the period 1987-2007].
abstract: BACKGROUND
  Polycystic kidney disease is an inherited kidney disease that affects both kidneys and it is characterized by diffuse replacement of renal parenchyma by thousands of microcysts. In time, renal insufficiency develops. There are two forms of PKD: ADPKD, which is detected in adults (children are rarely affected), and ARPK, which is detected in neonates (later presentations do occur, but rarely).
  
  
  OBJECTIVE
  The aim of this study was to analyse frequency of polycystic kidney disease, clinical data and morphological characteristics.
  
  
  METHODS
  At the Institute of Pathology, School of Medicine, Belgrade, there were detected 33 cases of ADPKD and 20 cases of ARPKD between 1987 and 2007.
  
  
  RESULTS
  There were no differences between incidence of ADPKD in males and females. Average age of patients with ADPKD was 52 years. In 20 (66.7%) cases of ADPKD there were neither extrarenal cysts nor extrarenal manifestations detected. In other 13 cases, we detected extrarenal cysts: hepatic cysts in 8 cases, pancreatic cysts in 5 cases. In two cases, hepatic cysts were associated with intracranial (arachnoid cysts) and extracranial aneurysms. The most frequent cause of death in patients with ADPKD was end-stage disease. ARPKD affects more often male children compared to female. 70% of children with ARPKD were male. The mean age of patients with ARPKD was 1 month. 5 patients (40%) had hepatic fibrosis. The most frequent cause of death was respiratory insufficiency (75%). In 25% of patients, the cause of death was sepsis and renal insufficiency.
  
  
  CONCLUSIONS
  Morphological and clinical manifestations of the analysed cases of both types of PKD are fairly consistent with literature data. Better knowing of aethiopathogenesis of PKD will facilitate early diagnosis, based on clinical and morphological characteristics and better management of the disease.
authors.forename: Sanja
authors.initials: S
authors.lastname: Radojević-Skodrić
authors.forename: Ljiljana
authors.initials: L
authors.lastname: Bogdanović
authors.forename: Milena
authors.initials: M
authors.lastname: Jovanović
authors.forename: Ivana
authors.initials: I
authors.lastname: Baralić
authors.forename: Marina
authors.initials: M
authors.lastname: Savin
authors.forename: Gordana
authors.initials: G
authors.lastname: Basta-Jovanović
mesh_terms: D000328:Adult; D000368:Aged; D001344:Autopsy; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D007668:Kidney / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications / Q000473:pathology*; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications / Q000473:pathology*