Multicentre, observational registry studying the natural course of polycystic liver disease.

Polycystic Liver Disease Registry (UK)

Polycystic Liver Disease

The Polycystic Liver Disease (PLD) registry (UK) is the UK specific part of an international,
      multicentre, observational registry. The overall international PLD registry is led by Radboud
      University Medical Center, Nijmegan, Netherlands. Note- please direct any queries about the
      international registry to the team at Radboud University Medical Center.

      All patients with Polycystic Kidney Disease (PKD) with PLD or patients with Isolated (without
      PKD) Polycystic Liver Disease are eligible for inclusion (>10 liver cysts).

      Data will be collected prospectively and retrospectively including a specific validated PLD
      questionnaire (PLD-Q).

      This registry provides us insight in patient characteristics, risk factors, symptoms, quality
      of life and treatment strategies in the biggest international PLD cohort so far. Results of
      this registry will be published and shared at national and international congresses.

To establish the demographics, presence and severity of symptoms (quantified using the validated PLD-Q questionnaire), liver cyst characteristics on imaging (assessed using Qian's classification), biochemical marker abnormalities (including gamma-glutamyl transferase and alkaline phosphatase), rate and nature of complications, need for treatment, change in liver volume in patients with PLD. This data will be collected retrospectively and prospectively from clinical records and the PLD-Q questionnaire.

The PLD-Q questionnaire consists of 18 items assessing symptom frequency and severity. It gives a score (0-100) based on the patient's answers with a higher score indicating more severe, symptomatic disease with a poorer quality of life.

For patients that develop a complication of PLD (including jaundice, abdominal hernia, features of portal hypertension, cyst haemorrhage and cyst infection) we will look through their clinical record to establish if there any risk factors which are common to those with a complication (e.g. age, gender, medications, cyst phenotype).

For patients that have a worsening of their PLD (based on increasing symptoms as per the PLD-Q questionnaire, development of complications or need for treatment) we will look through their clinical records to establish if there any elements which are common to those with progressive disease (e.g. age, gender, medications).

For patients who have had or require treatment for PLD we will compare the effectiveness of that treatment based on change in symptoms (based on PLD-Q questionnaire), recurrence of symptoms, development of complications or need for further treatment.

Liver growth rate will be determined using change in liver volume on serial radiology imaging. This will be compared against patients symptoms (measured via PLD-Q questionnaire), development of complications and need for treatment to establish whether there is a correlation in rate of liver growth and disease progression

Polycystic Liver Disease Registry (UK)

About this trial

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Type

Placebo

Accepting

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