Clinical trial NCT06036992: Study and Management of Cystic Complications in Autosomal Dominant Polycystic Kidney Disease
Official title: Study and Management of Cystic Complications in Autosomal Dominant Polycystic Kidney Disease
Study type: OBSERVATIONAL
Overall status: ACTIVE_NOT_RECRUITING
Conditions: Polycystic Kidney Diseases
Lead sponsor: University Hospital, Brest
Sponsor class: OTHER
First posted: 2023-09-14
Enrollment: 600
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: Patients with autosomal dominant polycystic kidney disease included in Genkyst who present at least one cystic complication.

Brief summary: 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.

Detailed description: 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.

Eligibility criteria: 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.)

Primary outcomes:
measure: To improve knowledge of the epidemiology of cystic complications within the Genkyst network
timeFrame: 12 months
Secondary outcomes:
measure: Creation of a specific multidisciplinary consultation meeting
timeFrame: 12 months
measure: Creation of an image bank
timeFrame: 12 months
measure: Creation of a group of control patients
timeFrame: 12 months
Other outcomes:
Locations:
city: Brest
country: France
facility: Chu Brest
Study officials:
References: