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

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Polycystic Kidney Diseases

Participation Criteria
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Eligibility Criteria
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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.)

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| To improve knowledge of the epidemiology of cystic complications within the Genkyst network | number of cases of cystic complications per year per complication | 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Creation of a specific multidisciplinary consultation meeting | Meetings and exchanges between different health professionals: interventional radiologists, surgeons, infectiologists, hepatologists, pain specialists and nephrologists to discuss the best possible management for patients with complex cystic complications. | 12 months | 
| Creation of an image bank | consulting images to recalculate and improve diagnostic scores | 12 months | 
| Creation of a group of control patients | the change from baseline in quality of life scores at 1 year. | 12 months |