Study and Management of Cystic Complications in Autosomal Dominant Polycystic Kidney Disease

Study and Management of Cystic Complications in Autosomal Dominant Polycystic Kidney Disease

Autosomal dominant polycystic kidney disease is characterised by the development of renal and hepatic cysts. While the main complication is chronic end-stage renal failure, specific cyst-related complications are common: intracystic haemorrhage, renal or hepatic cyst infections, cyst-related mechanical complications and lithiasis. To date, there is no reliable epidemiological data on the frequency and clinical impact of these complications. Diagnosis of these complications is often complicated, and their management has not been codified. The latest international recommendations (KDIGO) provide only low-level recommendations. For the most complex cases (recurrent cystic infections, resistant pain, mechanical complications and malnutrition, need for pre-transplant nephrectomy, etc.), practitioners are often at a loss and management varies greatly from one centre to another.

Autosomal dominant polycystic kidney disease is characterised by the development of renal and hepatic cysts. While the main complication is chronic end-stage renal failure, specific cyst-related complications are common: intracystic haemorrhage, renal or hepatic cyst infections, cyst-related mechanical complications and lithiasis. To date, there is no reliable epidemiological data on the frequency and clinical impact of these complications. Diagnosis of these complications is often complicated, and their management has not been codified. The latest international recommendations (KDIGO) provide only low-level recommendations. For the most complex cases (recurrent cystic infections, resistant pain, mechanical complications and malnutrition, need for pre-transplant nephrectomy, etc.), practitioners are often at a loss and management varies greatly from one centre to another.

Inclusion Criteria:

• Patient with autosomal dominant polycystic kidney disease (APKD) participating in the Genkyst study

• Patient with at least one cystic complication. The cystic complications retained are the following:

  • Acute or chronic cyst-related pain requiring analgesic treatments
  • Cyst infection
  • Intracystic haemorrhage
  • Urinary lithiasis
  • Functional complaints related to the cystic mass: digestive disorders with eating disorders, undernutrition, diaphragmatic compression phenomena, portal hypertension, umbilical or linea alba hernias, ventrations
  • Need for a cystic reduction procedure: puncture, marsupialization, open surgery (including preparation for grafting)

Exclusion Criteria:

• Patients who have expressed their opposition to taking part in the study
• Patient under legal protection (guardianship, curatorship, etc.)