Autosomal dominant polycystic kidney disease
Conditions
Interventions
Group A: No treatment follow-up or (if on tolvaptan at enrollment) administration of tolvaptan
Patients on tolvaptan at the time of enrollment will continue to receive tolvaptan at the same dose as at
Sponsors
Sohara Eisei
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1) Patients diagnosed with autosomal dominant polycystic kidney disease (ADPKD) at the time of consent. (Evidence-based Multiple Cystic Kidney Disease Guidelines 2020) 1. Patients with confirmed familial occurrence of ADPKD (either 1 or 2 below). 1. Ultrasound images show at least 3 cysts in each kidney. 2. CT and MRI images show at least 5 cysts in each of the two kidneys. 2. No known familial occurrence of the disease. 1. 16 years of age or older, CT, MRI or ultrasound tomogram confirms at least 5 cysts in each kidney and the following diseases are ruled out. Multiple simple renal cysts, tubular acidosis, polycystic kidney, polycystic renal cysts, medullary cystic disease (juvenile nephron), polycystic atrophic kidney, Autosomal recessive polycystic kidney disease (ARPKD) (2) Patients who are between 18 and 80 years of age at the time of consent. (3) Patients who have given their written consent to participate in this clinical research.
Exclusion criteria
Exclusion criteria: 1) Patients who have received pemafibrate within 12 weeks prior to obtaining consent 2) Patients with urine estimated proteinuria exceeding 5 g/gCre (albuminuria equivalent: 5,000 mg/gCre) at any time 3) Patients with severe renal dysfunction (eGFR < 15 mL/min/1.73 m2) 4) Patients on maintenance dialysis 5) Patients after renal transplantation 6) Patients with active infection requiring systemic treatment 7) Patients with ascites or pleural effusion of moderate or greater degree (e.g., requiring puncture) 8) Patients with severe mental disorders 9) Patients with malignant tumors undergoing treatment 10) Patients with hypernatremia (sodium levels of 150 mEq/L or higher) 11) Patients with uncontrolled hypertension 12) Patients with poorly controlled diabetes mellitus 13) Patients with severe hepatic disorder or biliary obstruction 14) Patients with gallstones 15) Patients with hypersensitivity to any component of pemafibrate 16) Patients receiving cyclosporine or rifampicin 17) Pregnant women, women who may become pregnant, and women who are breastfeeding 18) Patients who are judged as ineligible for registration by attending doctors
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Percentage of patients with at least a 15% decline in eGFR at 36 months | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Percentage increase in volume of both kidneys at 36 months 2. Percentage of patients with at least 5% increase in both renal volumes at 36 months 3. Percentage of patients with at least a 20% decrease in eGFR at 36 months 4. Relationship between patient background and laboratory findings at enrollment and both renal volumes | — |
Contacts
Public ContactHiroaki Kikuchi
Institute of Science Tokyo Hospital
Outcome results
None listed